Notalgia Paresthetica for Physicians

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Notalgia Paresthetica and Brachioradial Pruritus are a continuum of a single disease associated with cervical pathology: A prospective large scale study  

Abstract

Although Notalgia Paresthetica (NP) and Brachioradial Pruritus ( BRP)  have been historically described as two different diseases, we propose that these are more likely than not in fact a continuum of the same pruritic and paresthetic or dysesthetic disease process. While Notalgia is a common, refractory sensory neuropathic syndrome with the hallmark symptom of localized pruritus and paresthesia of the unilateral infrascapula, Brachioradial Pruritus is classified as a localized pruritus syndrome, but of the upper extremities, typically of one or both forearms. NP and BRP are both generally chronic, non-lethal, incurable conditions with periodic remissions and exacerbations. Often described as dermatologic syndromes, both diseases are typically considered to be multi-factorial in etiology. However, recent literature suggests that it is highly probable that NP and BRP in many, if not nearly all cases, have an association with underlying cervical disease, including musculoskeletal pathology and cervical disc disease. This described relationship has resulted in a paradigm shift in evaluation and treatment of NP and BRP as often cervical diseases with referred skin manifestations to the mid back and forearms. BRP and NP may and do occur concurrently and/ or sequentially in the same individuals.  In order to determine the possible underlying cervical disease, it is vital to conduct a physical exam of the neck and consider radiographic and magnetic resonance imaging studies of the spine more than they have been previously for these conditions. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, orthopedics, acupuncture, massage therapy, and physical therapy is often indicated as primary management of these 2 conditions. For cases of NP and BRP that are associated with cervical disease, the first line therapy may include non-dermatologic cervical treatments such as transcutaneous electronic nerve stimulation (TENS), electric muscles stimulations (EMS), acupuncture, cervical spinal manipulation, physical therapy, massage, cervical traction, cervical muscle strengthening, posture strengthening and retraining, and oral antihistamines, non-steroidal anti-inflammatory medications, and muscle relaxants. Typically, NP and BRP are most likely dermatologic signs of underlying degenerative, traumatic, or musculoskeletal cervical spinal disease. Here we present the what we believe to be the largest prospective case series report of NP and BRP.

Study

A prospective study identifying individuals that presented to an outpatient dermatology clinic with localized pruritus syndromes was undertaken from 2010 to 2015. A total of 128 individuals were diagnosed by a single practitioner specializing in pruritus and in particular localized pruritus syndromes. The diagnosis of NP and /or BRP was established based on physical exam findings and patient histories.  A significant percentage of patients were seen in self referral or by professional consultation, and had been seen by multiple prior dermatologists without definitive diagnoses or effective therapies.

Results

A total of 128 subjects were examined and diagnosed with Notalgia Paresthetica and/or Brachioradial pruritus from 2010 to 2015 in an outpatient dermatology practice. The age range was from 30-89 years. The median age of subjects was 65 years.  There was a female to male predominance of 3:1.Of the 128 subjects, 25% had concurrent NP and BRP.  NP was the more common diagnosis, accounting for more than 80% of the study subjects. Most patients with NP were right handed and had left side mid back NP. 30% of the subjects had BRP.  Only 3 of the 128 study subjects had presented with symptoms of only BRP without precedent NP back symptoms sometime prior to the onset of their BRP.  Laterality of NP was 2:1, left side of the back greater than right.

There were a number of comorbidities in the subjects with NP and BRP. In no order of prevalence, these were atopic dermatitis, hepatitis C, prior cervical spine disc surgery including spinal fusion, chronic renal insufficiency, cervical spinal disease on MRI, scoliosis, kyphosis, rheumatoid arthritis , unspecified eczema, eosinophilia, and elevated immunoglobulin E (IgE).

Patients self referred to the clinic had refractory and debilitating disease which reportedly had failed extensive therapies including topical anti-pruritics, topical anti-histamines, topical capsacin, intralesional triamcinolone, and  topical steroids including clobetasol, fluocinonide, and triamcinolone. Topical treatments were essentially minimally to not effective.  A number of  individuals had  reported they had exhausted all medical evaluation through care with innumerable physicians  and  multiple dermatologists including  university based dermatology departments prior to self=referral to our center.

Initial dermatologic examination of nearly all subjects revealed palpable and tender cervical muscle spasm multiple, and anterior cervical and supraclavicular muscle spasm. Most subjects had minimal skin findings on initial exam, and a small number had visible skin changes including mild hyperpigmentation on the infrascapular back. A small number of subjects presented with significant secondarily excoriated, hemorrhagic, hyperpigmented plaques and papules on the mid upper back (Figure1) and forearms. Additional exam findings included arm and forearm erosions , prurigo nodules, and lichen simplex chronicus (Figure 2). Affected NP areas were sometimes associated with slightly decreased sensory alternations to light touch, and pin prick.  A large number of subjects had physical exams consistent with significant palpable cervical muscle spasm with a slight to minimal decreased range of motion in the neck.

Recommended studies include laboratory testing for skin cultures to exclude Staphylococcus aureus skin secondary infection in individuals with skin erosions. Most subjects were advised to empirically cleanse neck to toe with chlorhexidine body wash as part of standard best practices. Consideration of general pruritus serology and laboratory workup was given to individuals with atypical patterns and generalized pruritus, Cervical imaging workup was considered for appropriate candidates with a contributory history of cervical trauma, motor vehicle accidents, and those with numbness or weakness in the upper extremities.  In the small minority of subjects who did elect to undergo MRI of the cervical spine, imaging showed varying degrees of pathology in more than 80% of subjects ranging from mild to severe cervical canal stenosis or disc disease at typically C4- C6 as seen in the example of  (Figure 3).

Our subjects typically underwent initial therapy with transcutaneus electronic nerve stimulation (TENS) and/or electrical myostimulation (EMS), as well with acupuncture of the cervicothoracic spine (Figure 4). We began standard regimens of chlorhexidine cleansing bid from neck to toes, oral hydroxyzine 10-40 mg every 6-8 hours, and mupirocin or bacitracin intranasally twice a day for 14 days for empiric treatment of staphylococcal colonization.  Nearly all subjects reported decreased pruritus, improved sleep, and enhanced quality of life within a few days and up to a few weeks of initial assessment and therapy. A standard therapy of at- home or in office TENS/EMS use 3 times daily, for 5-10 minutes intervals was highly effective for most subjects.

Discussion

Notalgia paresthetica (NP) is a sensory neuropathic syndrome of the back, classically of the unilateral infrascapular (Figure 5). NP is largely associated with severe localized pruritus. In 1934 NP was first named and described as a periodic itching or pain on a small patch of skin located along the mid back, usually just past easy reach.

The dermatologic conditions may consist of other symptoms which include, but not limited to localized burning, pain, tenderness, hyperalgesia, or dysesthesias. NP is typically associated with a poorly confined tan or hyperpigmented patch in the symptomatic area while BRP is often described as a small patch or a dermatomal distribution of burning and an “under the skin” itch on 1 or both forearms. NP tends to be a more chronic condition than BRP with periodic remissions and exacerbations. NP is and BRP may occur more frequently in association with other co-morbidities such as those conditions predisposing individuals to pruritus and/ or spinal disease. NP and BRP while not life threatening, however do frequently decrease quality of life and cause much discomfort and annoyance to the affected patients.

Topical treatments for NP and BRP have generally failed and are considered difficult because of the out-of-reach affected location in NP and the refractory symptoms in BRP. The reports of topical therapies like capsacin having some effect can perhaps be attributed to part placebo as well as part occlusive of the underlying pathology. It is paramount to treat the underlying systemic conditions in BRP and NP as opposed to masking the cutaneous signs of the underlying pathology. To date, there has been no uniformly effective treatment for NP or BRP in the literature, and none in large scale reports. The etiology for NP and BRP continue to be unclear and controversial. While the etiology of these paresthetic processes remains uncertain, there are multiple possible mechanisms that have been previously proposed for NP including 1) localized increased sensory innervations of the affected skin areas and 2) neuropathy from degenerative cervico-thoracic disc disease or direct nerve impingement. Mechanisms of photo induced symptoms with seasonal exacerbations have been described with BRP.

The differential diagnosis in NP and BRP may include allergic or irritant contact dermatitis, fixed drug eruption, dermatophytosis, neoplasm, lichen amyloid, arthropod reaction, lichen simplex chronicus, neurodermatitis, secondary excoriations, varicella zoster, skin infection, and other hypersensitivity reactions.

It is important that during the initial assessment of patients with NP and BRP to obtain a thorough past history of osteoarthritis, prior neck trauma, motor vehicle accident, vertebral fracture, cervical neoplasm or malignancy, or cervical disc disease. Radiographs or MRI of the cervical spine may aid in diagnosis and treatment if there is an absence of positive medical history. Studies may be additionally advantageous if there is a positive family history of osteoarthritis or vertebral disc disease.

A full laboratory workup including complete blood count, chemistry panel including renal and liver functions, and other labs may be warranted if pruritus is generalized and persistent in order to exclude other causes. Proper management of NP and BRP may involve a multi-specialty effort of dermatology with radiology, orthopedic surgery, neurology, and adjunctive fields including acupuncture, chiropractic, massage therapy, and physical therapy.

Topical therapies aimed at the back may be in fact be ineffectual or partially effective as basic emollients or placebo. Due to NP and BRP’s periodic spontaneous remissions and exacerbations, it may be difficult to accurately measure response to various therapies. Some therapies may be considered comparable to a placebo response in some affected individuals.

Previously accepted therapeutic options for NP and BRP were inconsistent and unsatisfactory and included capsaicin cream, eutectic mixture of local anesthetic (EMLA) cream, topical steroids, pramoxine cream, topical cooling, oral steroids, Tiger balm, menthol creams, flurandrenolide tape, intralesional corticosteroid injections, botulinum toxin injections (12), oral antihistamines, hydroxyzine, doxepin, topamax, anticonvulsant medications, carbamazepine (Tegretol) antidepression medications, gabapentin ( Neurontin), oxcarbazepine, topiramate, thalidomide (11), paravertebral local anaesthetic block, cervical epidural injection, surgical resection of the rib, and many others. Some of the current systemic therapies exert their effect through the spinal nerves and central nervous system thereby supporting the neuropathic etiology of NP and BRP.

A case publication by Alai et al in 2016 reported a 74 year old male patient with documented NP on the right mid back, bilateral upper shoulders, and BRP on his bilateral arms and forearms with MRI findings of severe disc-disease at C4-C7 and moderate nerve impingement, which strongly suggested the association of C-spine disc-disease and NP and BRP. (1)An earlier case publication by Alai et al in 2010 reported a 37 year old patient with documented NP on the right back with MRI findings of disc-disease at C5-C6 and mild nerve impingement, which strongly suggest the association of C-spine disc-disease and NP. (2) Both patients responded significantly and definitely to TENS/EMS and cervical therapy including physical therapy, massage, and acupuncture of the neck and trapezius trigger point regions.

A study by Savk et al in 2000 showed 7 of 10 patients with NP demonstrated normal neurological examination and standard electrodiagnostic results in all study patients. All had skin histopathology compatible with postinflammatory hyperpigmentation. There were no amyloid deposits or other described pathology on pathologic exam of the skin.  7 of the 10 cases confirmed radiographic changes in the vertebrae corresponding to the dermatome of the cutaneous lesion. (8)

A study published by Savk  in 2004 discussed the relationship between  brachioradial pruritus and notalgia paresthetica. (11 )

An earlier study by Springer et al in 1990 evaluating the mechanism of NP studied whether the cutaneous symptoms were caused by alternations on the cutaneous innervation of the involved infrascapular area. They postulated that the histology findings with increased dermal innervation to the areas however no measurable change in the distribution of neuropeptide-immunoreactive axons was found. There was an increase in the number of intradermal PGP 9.5-immunoreactive nerve fibers and epidermal dendritic cells compared with unaffected areas from the same patients and normal controls. It was concluded that the symptoms of NP may in part be related to an increase in the sensory epidermal innervation in the affected skin areas. (4)

Histologic studies have shown cutaneous changes in a few cases including lichen amyloid which may be secondary to the localized chronic scratching and rubbing. (13) Clinical observations in orthopaedics have established a clear relationship between the upper thoracic/interscapular region and the lower cervical spine. (1,2) Frequently, cervical disc disease presents as referred pain in the upper thoracic and interscapular area.  Similarly, some tumors of the cervical medulla have also presented as interscapular pain. (3)

Some have speculated direct involvement and actual entrapment of the posterior rami of T2 to T6 spinal nerves. However, there are referred symptoms from the cervical area directly to the interscapular back. Degenerative vertebral and disc changes corresponding to the affected dermatome may be observed in some cases.  Recent literature supports a role for radiographic imaging of cervical and thoracic spine to exclude disc disease and possible nerve compromise.

Early recognition of cervical disease in NP and BRP patients may promote timely intervention and treatment to mitigate cervical spine disease progression. In addition to degenerative cervical discs, osteoarthritis, and cervical spine strain and muscle spasm, there may be neoplasms or other pathology of the neck, particularly at the level of C4-C6 contributing to NP and BRP.

There are to date a number of studies from Alai et al and Savk et al demonstrating the relationship between NP and BRP. The recently described association of many cases of BRP and cervical musculoskeletal disease (6) and description of the disease as a possible neuropathic/ neurogenic condition also support a probable neuropathic association of NP. In contrast, NP is classically described as unilateral disease while BRP may involve unilateral or bilateral upper extremities. There are however, a number of atypical presentations of NP and BRP, including extension to the scalp and axilla, such that there is a need for ongoing investigation into the association and possible reclassification of all of these local pruritus syndromes.

First line therapy for NP and BRP with associated cervical disease typically includes non-dermatologic, non-invasive treatments such as spinal manipulation, physical therapy, cervical soft collars, massage, pillow adjustment, cervical traction, cervical muscle strengthening and increased range on motion, acupuncture, therapeutic massage including Thai massage, cervical discectomy with fusion, oral non-steroidal anti- inflammatory medications (ibuprofen, celecoxib,  ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other measures for degenerative disc disease as introduced can also be considered.

Conclusion:

We propose a paradigm shift in the classification of Notalgia Paresthetica (NP) and Brachioradial pruritus (BRP) as related and often contemporaneously or serially occurring diseases, which are in essence both cutaneous signs of underlying cervical musculoskeletal disease. (20) The striking association of NP with BRP in this large case series, and the described degenerative and/or or traumatic cervico-thoracic spine disease suggests that early cervical pathology particularly at the C5-C6 level may contribute to the pathogenesis of these skin symptoms. Additional studies are needed to further assess the relationship of NP and BRP with each other, as well as that of each disease entity independently with cervical spine disease and the other described local pruritus syndromes especially posterior scalp pruritus. Moreover, we propose that much of the nomenclature for these paresthesias warrants revision to reflect a general category of “spinal paresthesias” which would embody all of the various local pruritus syndromes including NP, BRP, burning scalp syndrome, burning scrotum, meralgia paresthetica, and others. (21-22) Whether these observations are causal or coincidental findings remains to be determined in larger studies. While topical therapies may in some cases seemingly help decrease the localized symptoms in NP and BRP, this effect may be considered as potential placebo or as basic emollients. In most cases, systemic assessment and broader scope spinal evaluation may be warranted to fully evaluate refractory cases of NP and BRP. Cervical exam and treatment may be appropriate primary therapy in many cases of NP and BRP. The proposed paradigm shift in thinking is these two diseases may be to treat the cervical muscles and spine, and to expect that the skin will follow. Although notalgia paresthetica (NP) and brachioradial pruritus (BRP)  have been historically described as two distinct diseases, we propose that these are in fact a continuum of the same pruritic and paresthetic disease processes. Therefore, it may be time to consider a reclassification and update of the nomenclature for these processes to reflect this incestuous relationship.

 References 

  1. Alai NN, Skinner HB  Concurrent Notalgia Paresthetica and Brachioradial Pruritus Associated With Cervical Spinal Stenosis and Cervicothoracic Disk Disease at C3 Through C6. Cutis. 2016; accepted for publication
  2. Alai NN, Skinner HB, Nabili S, Jeffes E, Shahrokni S, Saemi A. Notalgia Paresthetica Associated With Cervical Spinal Stenosis and Cervicothoracic Disk Disease at C4 Through C7. Cutis. 2010; 85:77-81.
  3. Eisenberg E, Barmeir E, Bergman R. Notalgia paresthetica associated with nerve root impingement. J Am AcadDermatol. 1997;37:998-1000.
  4. Misery L. What is notalgia paresthetica? Dermatology.2002;204:86-87.
  5. Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: cervical spine disease and neurogenic/neuropathic pruritus. J Am Acad Dermatol. 2003;48:521-524.
  6. Pleet AB, Massey EW. Notalgia paresthetica. Neurology.1978;28:1310-1312.
  7. Springall DR, Karanth SS, Kirkham N, et al. Symptoms of notalgia paresthetica may be explained by increased dermal innervation. J Invest Dermatol. 1991;97:555-561.
  8. Weber PJ, Poulos EG. Notalgia paresthetica. case reports and histologic appraisal J Am Acad Dermatol. 1988; 18(1, pt 1):25-30.
  9. Savk O, Savk E. Investigation of spinal pathology in notalgia paresthetica. J Am Acad Dermatol. 2005;52: 1085-1087.
  10. Savk E, Savk O, Bolukbasi O, et al. Notalgia paresthetica: a study on pathogenesis. Int J Dermatol. 2000;39:754-759.
  11. Savk E, Savk SO. On brachioradial pruritus and notalgia paresthetica. J Am Acad Dermatol. 2004;50:800-801.
  12. Goodless DR, Eaglstein WH. Brachioradial pruritus treatment with topical capsaicin. J Am Acad Dermatol. 1993;29(5, pt 1):783-784.
  13. Tait CP, Grigg E, Quirk CJ. Brachioradial pruritus and cervical spine manipulation. Australas J Dermatol. 1998;39:168-170.
  14. Weinfeld PK. Successful treatment of notalgia paresthetica with botulinum toxin type A. Arch Dermatol. 2007;143:980-982.
  15. Savk E, Bolukbasi O, Akyol A, et al. Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica. J Am Acad Dermatol. 2001;45:630-632.
  16. Pereira J. Brachioradial pruritus treated with thalidomide [in Spanish]. An Bras Dermatol. 2005;80: 295-296.
  17. Savk E, Savk O, Sendur F. Transcutaneous electrical nerve stimulation offers partial relief in notalgia paresthetica patients with a relevant spinal pathology. J Dermatol. 2007;34:315-319.
  18. Goulden V, Toomey PJ, Highet AS. Successful treatment of notalgia paresthetica with a paravertebral local anesthetic block. J Am Acad Dermatol. 1998;38:114-116.
  19. Hruza GJ. The cutting edge [letter]. Arch Dermatol. 2007;143:1062
  20. Alai NN, Dehghanian PM.  DERM DX: What caused this refractory pruritic eruption? Refractory Concurrent Atypical Notalgia Paresthetica and Brachioradial pruritus associated with Hepatitis C and Rheumatoid Arthritis. The Dermatologist. XX 2016; 24(xx);46-50.
  21. Thornsberry LA, English JC 3rd. Scalp dysesthesia related to cervical spine disease. JAMA Dermatol. 2013 Feb. 149(2):200-3. [Medline].
  22. Shumway NK1Cole E2Fernandez KH3. Neurocutaneous disease: Neurocutaneous dysesthesias. J Am Acad Dermatol. 2016 Feb;74(2):215-28. doi: 10.1016/j.jaad.2015.04.059.

Figure 1: Back close up showing atypical NP upper back and classic area lower back at level of T10

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Figure 2: BRP left arm with excoriations and lichenifcaction and prurigo nodules

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Figure 3: MRI  of  C-spine demonstrating a 4 mm disc bulge at C5-C6 in patient with concurrent BRP and NP.

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Figure 4: Left Low Back with atypical NP and moderate lichenification in a pink plaque.

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Figure 5: Back with transcutaneous electronic nerve stimulation (TENS) treatment

 

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Case Report and Review of the Literature

Successful Treatment of Atypical Notalgia Paresthetica with Acupuncture, Cervical

Physical Therapy, and Cervical Nerve Block Steroid Injection at C3-C5

 Abstract

Notalgia paresthetica (NP) is an under-recognized and under diagnosed, extremely common cause of localized pruritus of the back, most commonly of the infrascapula. Although long considered a dermatologic condition, NP is more completely described as a neuropathic dysesthesia. Dysesthesia is a fundamental term describing cutaneous symptom such as crawling sensations, pruritus, burning, tingling, stinging, anesthesia, hypoesthesia, tickling, and cold or hot sensations.   NP’s known association with underlying cervical disease in a large subset of patients warrants categorization of this disorder as a primary neuropathy with cutaneous manifestations. A paradigm shift in the dermatologist’s approach to treatment of NP may be warranted, with a focus on eliciting relevant cervical spinal history including trauma, arthritis, degenerative disc disease, and other spinal neoplasms. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, pain management, chiropractic, physical therapy, acupuncture, and orthopedics may be indicated in primary management of this condition. Proposed first line therapy for NP with associated cervical disease includes non-dermatologic spinal treatments such as cervical traction, spinal manipulation, cervical steroid injection, physical therapy, massage, cervical muscle strengthening, and oral non-steroidal anti-inflammatory medications and muscle relaxants. NP is in fact another dermatologic sign of an underlying systemic disease. We present a case report of a 65 year old with atypical NP successfully treated with cervical acupuncture, physical therapy, and spinal nerve block at C3-C5.

 

Case Report

65 year old right handed Caucasian female presented with a 6-12 month history of intermittent bouts of recurrent itching on the right infrascapular skin of the back in the T5-T6 dermatome. She additionally presented with an acute onset of superficial, almost zosteriform, erosions on her upper back and shoulder. Figures 1 and 2 depict the acute exacerbation of her symptoms. Figure 3 demonstrates the classic clinical infrascapular presentation of her baseline Notalgia Paresthetica (NP).  Dermatologic examination revealed a slightly hyperpigmented, non-indurated, very ill defined patch of the right mid back with mild decrease in associated sensory alternations to light touch and pin prick. Neurologic examination confirmed a marked decreased in the cervical range of motion with associated marked bilateral cervical muscle spasm. Further, the patient had marked tenderness and decrease range of motion in the cervical and lumbar spine, with scoliosis and kyphosis.

Past medical history of the patient was significant for three motor vehicle accidents (MVA) and sustained whiplash injuries over 10 years. The most recent MVA occurred less than 1 year prior to the onset of NP and the patient had been undergoing active therapy for 10 years. Current treatments included weekly physical therapy, weekly traction, home ice and heat, and multiple pain releasing modalities.  Additional history included Hepatitis C acquired 17 years prior through blood transfusions, mild generalized pruritus, xerosis, and allergies.  Pertinent history included 15-20 years status post multiple mild automobile related whiplash injuries with subsequent intermittent interscapular, low back, and neck pain. Past treatments for neck and back pain included intermittent chiropractic spinal adjustments, physical therapy, acupuncture, cervical and trigger point injections with triamcinolone 40mg/cc and 0.5% bupivacaine, and various other medical treatments.

The patient’s dermatologic chart documented a direct correlation of exacerbation of the NP skin symptoms with reported subjective exacerbations of the patient’s cervical pain at her regular biweekly to monthly office visits. Spontaneous remissions were observed in the skin discoloration and itching corresponding with a temporary decrease in neck and interscapular pain. The symptoms were minimally affected by the use of oral hydroxyzine, loratidine, and multiple topical agents including clobetasol cream, doxepin cream (Zonalon), intralesional botuminum toxin, and oral doxepin.

Magnetic Resonance Imaging (MRI) of the cervical, thoracic, and lumbar spine revealed cervical spondylosis, including degenerative disc disease, osteophytosis and ligamentum flavum hypertrophy at C3-C4, C5-C6, and C6-C7. Figure 4 shows the MRI cervical findings. Laboratory workup revealed mild anemia, stable thrombocytopenia, minimally elevated liver function tests, IGE levels, negative H. pylori IgG and IGM, HIV and hepatitis panel except for positive Hepatitis C.  Patient did exceptionally well at 1 year follow up. Skin lesions cleared using a newly standardized multimodal therapy program at The Skin Center’s Notalgia Clinic.  This specialty Notalgia program utilizes a customized clearing and maintenance phase schedule directed at cervical spinal and disc disease at C4-C6. The main components of this program utilize transcutaneous electrical nerve stimulation (TENS) and electrical muscle stimulator (EMS), cervical acupuncture, phototherapy with narrow band ultraviolet B (NB UVB) for generalized pruritus control, and physical therapy referral.  Three years after the successful treatment and resolution of her severe NP and additional brachioradial pruritis (BRP) symptoms, the patient developed lymphoma and began chemotherapy. Histopathologic examination of involved back and arm skin biopsies demonstrated nonspecific findings of superficial and perivascular dermal inflammation with mild, focal secondary impetiginization

Discussion

NP was first described in 1934 as an episodic itching or pain on a small patch of the mid back, usually an area of skin just past easy reach. NP is a common, but frequently under diagnosed, chronic dermatologic condition with periodic remissions and exacerbations. Notalgia is classically described as intermittent, intense itching of the unilateral infrascapula. Patients frequently present with a non-distinct area of itching in the mid back, often just under the brassiere hook area in women.

Additional NP symptoms may include localized pain, burning, hyperalgesia, dysesthesias, or tenderness. Patients describe the itch as an unusual one with an “under the skin sensation”. The skin in NP may appear completely normal, or may reveal a single or multiple poorly circumscribed, tan or hyperpigmented patches, or dry or moderately lichenified areas in the classis mid back location. In this patient, Figure 1 depicts atypical zosteriform erosions on the right deltoid and lateral scapular region. Figure 3 depicts a more lichenified plaque in the mid to low back dermatome.  NP waxes and wanes, and is frequently quite symptomatic in some patients, causing much discomfort and nuisance, and overall decrease in the quality of life.

Multiple possible neuropathic mechanisms have been proposed for NP. Two of the more common potential etiologies include 1) neuropathy from degenerative cervico-thoracic disc disease, and 2) direct nerve impingement localized increased sensory innervation of the affected skin areas.

Savk et al in 2000 published a study of 10 NP patients with uniformly normal neurological examination and standard electrodiagnostic results. Seven of the 10 cases confirmed radiographic changes in the vertebrae corresponding to the dermatome of the cutaneous lesion. All 10 subjects demonstrated skin histopathology compatible with post inflammatory hyperpigmentation without any amyloid deposits or other described pathology. 9  An earlier study by Springer et al in 1990 evaluated the mechanism of NP by studying the histology of the skin and cutaneous innervations of the affected areas and concluded that the symptoms of NP may in part be related to an increase in the sensory epidermal innervation in the affected skin areas. 3  While not typical in NP, histological changes including cutaneous lichen amyloid have been described on biopsy of affected skin. These lichenoid changes may be secondary to localized chronic scratching and rubbing. 12

What has been elusive in the clinical understanding of NP is the referred location in the mid back. Because of the involved thoracic area, others have speculated possible actual entrapment and direct involvement of the posterior rami of the T2 to T6 spinal nerves. However, the cervical area frequently has symptom referral directly to the infrascapular back. Although cervical degenerative disease typically presents as corresponding localized tenderness or pain, cervical symptoms may also commonly present as referred pain in the upper thoracic and interscapular area without any associated neck symptoms. Clinical observations in orthopedic surgery have established a definite causal relationship between the lower cervical spine upper and the thoracic/interscapular region. Similarly, tumors of the cervical medulla may present as interscapular pain. (2)

There is a significant relationship between NP and brachioradial pruritus (BRP), such that Alai et al have recently proposed these may be a continuum of the same disease. (6,7) Figures 1, 2, and 3 depict the atypical pattern of pruritus and erosions in this patient at different presentations. There are areas of NP and some of BRP. The described association of many cases of BRP and cervical spine disease and description of the disease as a probable neuropathic condition also support a neuropathic association of NP. (5)

Topical therapies aimed at the back may be in fact be ineffectual or partially effective as basic emollients. Since the disease does have periodic spontaneous remissions and exacerbations, it may be difficult to accurately measure response to various therapies versus a placebo response. Traditionally, treatment of NP with topical medications has also been difficult because of the hard to reach location.

The differential diagnosis in NP may include allergic or irritant contact dermatitis, fixed drug eruption, dermatophytosis, neoplasm, lichen amyloid, arthropod reaction, lichen simplex chronicus, neurodermatitis, cutaneous infection, zoster, paraneoplatic disorder, and other hypersensitivity reactions. Figure 1 depicts a zosteriform distribution of this patient’s polymorphic and cyclical eruptions.

During the initial assessment of patients with NP, it is important to examine the neck for muscle spasm, tenderness, and range of motion. Secondly, it is useful to obtain a thorough past history of osteoarthritis, prior neck trauma, motor vehicle accident, vertebral fracture, cervical neoplasm or malignancy, or cervical disc disease. In the absence of positive medical history, radiographs or MRI of the cervical spine may aid in diagnosis and treatment. Further, a positive family history of osteoarthritis or vertebral disc disease may be contributory.

Comprehensive laboratory workup is warranted for refractory generalized pruritus including but not limited to complete blood count, chemistry panel including renal and liver functions, sedimentation rate, hepatitis screening panel, anti-thyroid antibodies, and other studies like chest radiography, and computerized tomography of the chest, abdomen, and pelvis as required to exclude other underlying causes.

Proper management of NP involves a multi-specialty cooperative effort of dermatology with radiology, orthopedic surgery, neurology, and adjunctive fields including acupuncture, massage, chiropractic, and physical therapy.

While to date there has been no uniformly effective and satisfactory treatment for NP, our new paradigm shift in treatment approach of the underlying cervical region with therapeutic modalities like TENS/EMS, cervical acupuncture, and physical therapy and massage are very promising and have been hugely successful in large scale studies. (8)

Historically, therapeutic options for NP, albeit largely unsatisfactory and inconsistent in results included capsaicin cream, eutectic mixture of local anesthetic (EMLA) cream, topical steroids, pramoxine cream, topical cooling, oral steroids, menthol creams, flurandrenolide tape (Cordran), intralesional corticosteroid injections, botulinum toxin injections, 11 oral antihistamines, hydroxyzine, doxepin, topamax, anticonvulsant medications, carbamazepine (Tegretol), antidepressant medications, gabapentin (Neurontin), oxcarbazepine, 14 topiramate, thalidomide ,10 paravertebral local anesthetic block, 15 cervical epidural injection, surgical resection of the rib, and many others. Some of the utilized systemic therapies may in fact exert their effect through the cervical spinal nerves and central nervous system thereby supporting the neuropathic etiology of NP.

Proposed new first line therapy for NP include non-dermatologic, non-invasive treatments such as physical therapy, acupuncture, massage, spinal manipulation, cervical soft collars, cervical traction, cervical muscle strengthening and increased range of motion, cervical discectomy with fusion, oral non-steroidal anti-inflammatory medications (ibuprofen, celecoxib, ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other measures for degenerative disc disease and arthropathy as introduced may also be considered.

Conclusions

NP is most likely not a true dermatologic disorder, but rather a cutaneous sign of underlying systemic disease.  NP is predominantly associated with varying degrees of cervical musculoskeletal disease ranging from asymptomatic and extremely mild cervical muscle spasm, to severe spinal stenosis and cord impingement at C4-C6 with corresponding upper extremity radiculopathy.  The striking association of NP with degenerative or traumatic cervico-thoracic spine disease suggests that cervical arthropathy or maylagia often contributes to the pathogenesis of this skin symptoms of the disease. Additional studies are needed to further assess the relationship of NP with cervical spine disease and comorbidities predisposing certain individuals to developing NP. The most successful treatments for NP center around treating the cervical pathology first. The new rule in NP is to treat the neck, and the skin will follow. While topical therapies have historically been utilized as first line therapy in NP, we propose a paradigm shift in the global assessment and approach to NP with therapies aimed at the cervical region. Broader scope spinal evaluation and systemic laboratory and imaging evaluation for underlying causes of pruritus may be warranted to fully evaluate refractory cases of NP. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of NP.

References 

  1. Hruza GJ, The Cutting Edge, Arch Dermatol. 2007;143(8):1062.
  1. Bernard PA, Wayne ME. Notalgia paresthetica. Neurology (1978). 28,1310-.
  1. Springall DR, Karanth SS, Kirkham N, Darley CR, Polak JM. Symptoms of Notalgia Paresthetica May Be Explained by Increased Dermal Innervation Journal of Investigative Dermatology (1991) 97, 555–561.
  1. Alai NN, Skinner HB, Nabili ST, Jeffes E,  Shahrokhni S, Saemi AM.  Notalgia paresthetica associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C7. Cutis 85(2):77-81 (2010) PMID 20349681
  1. Alai NN, Skinner HB Concurrent Notalgia paresthetica and Brachioradial pruritus (BRP) associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C6. Accepted for publication Cutis2016
  1. Alai NN, Dehghanian PM.  DERM DX: What caused this refractory pruritic eruption? Refractory Concurrent Atypical Notalgia Paresthetica and Brachioradial pruritus associated with Hepatitis C and Rheumatoid Arthritis. The Dermatologist. XX 2016; 24(xx);46-50.
  1. Alai NN, Skinner HB Notalgia Paresthetica and Brachioradial Pruritus: a continuum of a single disease process associated with cervical C4-C7 pathology: A prospective large scale study   Pending publication 2016
  1. Perez LC. General features and treatment of notalgia paresthetica. Skinmed 9(6):353-8; quiz 359 (2011) PMID 22256623
  1. Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: cervical spine disease and neurogenic/neuropathic pruritus. J Am Acad Dermatol. 2004 May;50(5):800-1.
  1. Savk E, Savk SO.On brachioradial pruritus and notalgia paresthetica. J Am Acad Dermatol. 2003 Apr;48(4):521-4.
  1. Misery L. What is notalgia paresthetica? Dermatology. 2002; 204 (2):86-7.
  1. Pleet AB, Massey EW. Notalgia paresthetica. Neurology 1978.Dec; 28(12):1310-12.
  1. Şavk, Ekin; Şavk, Ş. Öner; Bolukbasi, Okan; Ccedilulhaci, Nil1; Dikicioğlu, Emel; Karaman, Göksun; Şendur, Neslihan. Notalgia paresthetica: a study on pathogenesis. International Journal of Dermatology, Volume 39, Number 10, October 2000 , pp. 754-760(7).
  1. Pereira J. Brachioradial pruritus treated with thalidomide. An Bras Dermatol. 2005;80 (3):295-6.
  1. Weinfeld P. Successful Treatment of Notalgia Paresthetica With Botulinum Toxin Type A. Arch Dermatol. 2007;143(8):980-982.
  1. Eisenberg E et al. Notalgia paresthetica associated with nerve root impingement
    J Am Acad Dermatol. December 1997 (Vol. 37, Issue 6, Pages 998-1000).
  1. Savk , E . Savk O. Investigation of spinal pathology in notalgia paresthetica.  J Am Acad Dermatol., Volume 52 , Issue 6 , Pages 1085 – 1087.
  1. Şavka E, Bolukbasib O, Akyolb A, Karamana G. Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica  J Am Acad Dermatol. October 2001 (Vol. 45, Issue 4, Pages 630-632).
  1. Goulden V, Toomey, Highet AS. Successful treatment of notalgia paresthetica with a paravertebral local anesthetic block . J Am Acad Dermatol.
    January 1998 (Vol. 38, Issue 1, Pages 114-116).
  1. Savk O, Savk E. Investigation of spinal pathology in notalgia paresthetica
    Journal of the American Academy of Dermatology June 2005 (Vol. 52, Issue 6, Pages 1085-1087).
  1. Weber PJ, Poulos EG. Notalgia paresthetica. Case reports and histologic appraisal
    J Am Acad Dermatol.. January 1988 (Vol. 18, Issue 1, Pages 25-30).
  1. Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: Cervical spine disease and neurogenic/neurogenic pruritus. J Am Acad Dermatol.
    April 2003 (Vol. 48, Issue 4, Pages 521-524.

23.  Nora K. Shumway, Emily Cole, Kristen Heins Fernandez, Neurocutaneous disease: Neurocutaneous dysesthesias, J Am Acad Dermatol., Vol. 74, Issue 2, p215–228: February 2016

24.  Miguel Chuquilin, Yazan Alghalith, Kristen Heins Fernandez Neurocutaneous disease: Cutaneous neuroanatomy and mechanisms of itch and pain, J Am Acad Dermatol., Vol. 74, Issue 2, p197–212: February 2016

 

 

 

Patient Presentation

A 67 year-old female presented with greater than 3 years of refractory and debilitating intermittent bouts of recurrent pruritus on the left infrascapular skin in the T5-T6 dermatome and new onset of right arm and shoulder, and low back tingling and small superficial erosions. Skin examination of the right deltoid, lateral upper back, and low back revealed multiple superficial erosions in a very atypical zosteriform pattern. Orthopedic examination confirmed moderate spinal scoliosis, marked decreased range of motion in the neck with associated marked bilateral cervical muscle spasm and tenderness to palpation, particularly on the left side. Past medical history of the patient was significant for rheumatoid arthritis (RA), post transfusion acquired hepatitis C, borderline anemia of chronic disease, and iron deficiency. Pertinent history included 2 years status post multiple mild-moderate automobile related whiplash injuries with subsequent intermittent interscapular and neck pain. Past treatments for neck and back pain included frequent spinal adjustments, physical therapy, trigger point injections, paravertebral ganglion blocks with a pain specialist, and various other failed medical treatments including oral gabapentin and muscle relaxants.

What is your diagnosisDiagnosis: Refractory Concurrent Atypical Notalgia Paresthetica (NP) and Brachioradial pruritus (BRP) Associated with Hepatitis C and Rheumatoid Arthritis.

Notalgia paresthetica (NP) and Brachioradial Pruritus (BRP) are heavily under-recognized and under-diagnosed common causes of episodic and chronic, localized, dysesthesias and itching of the mid back and upper extremities respectively (1, 2). While NP and BRP are often described as independent conditions, these two conditions do often occur concurrently or serially in the same individual (2).

Clinical and Histologic Features

Typically, NP presents with a hyperpigmented patch or plaque in the infralateral scapula while BRP presents as symptomatic periodic dysesthesias accompanied by mild erythema or excoriations on the affected forearm(s). An atypical dermatomal distribution of superficial skin erosions can mimic Herpes Zoster. Negative viral laboratory testing, infectious disease consultation, and failed treatment with oral valacyclovir excluded a viral infectious etiology for our patient. Histopathologic examination of the involved back and arm skin biopsies demonstrated nonspecific findings of superficial and perivascular dermal inflammation with mild, focal secondary impetiginization.

  

Differential Diagnosis

The differential diagnosis for NP includes irritant dermatitis, contact dermatitis, lichen simplex, neurodermatitis, lichen amyloid, bra strap dermatitis, and others. Although BRP and NP have long been considered dermatologic conditions, both are more accurately described as sensory neuropathies (1-4). NP and BRP may in fact represent the same spectrum of one pathophysiologic neuropathic disorder originating from the cervical spinal region (2). Both NP and BRP’s well described associations with underlying cervical musculoskeletal disease in many patients warrants categorization of these disorders as a primary neuropathy with cutaneous manifestations (1,2).

Treatment

Similar to prognostication of standard cervical musculoskeletal diseases, NP and BRP are generally chronic, controllable conditions with periodic remissions and exacerbations. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, pain management, acupuncture, physical therapy and orthopedics surgery may be indicated in best practices of management of these 2 conditions (1,2).  First line therapy for NP and BRP with associated cervical disease should include consideration of potential non-dermatologic therapies aimed at the spine. Such cervical spinal treatments include transcutaneous electrical stimulation (TENS), acupuncture, electrical muscle stimulation (EMS), cervical traction, spinal manipulation, physical therapy, massage, cervical muscle strengthening and range of motion enhancing routines, neck passive continuous motion (PCM), oral non-steroidal anti-inflammatory medications, and oral and topical muscle relaxants. NP and BRP may both in fact dermatologic signs of underlying systemic disease (1-4).

A paradigm shift in the dermatologist’s approach to treatment of NP and BRP is warranted, with a focus on eliciting relevant spinal history including cervical muscle spasm, neck trauma, arthritis, degenerative disk disease, and other spinal neoplasms (1,2). Topical therapies for NP and BRP have been highly inconsistent and unsatisfactory in a majority of patients (1-4). Further, appropriate patient education for both BRP and NP requires discussion of potential cervical conditions and clear awareness of the probable association of the localized itching with underlying cervical disease (2). Radiographic studies of the cervical spine including plain film and MRI may be more considered in primary evaluation of both NP and BRP than current standards.

Our patient

We describe a concurrent case of refractory NP and BRP in a 67 year-old female successfully controlled with cervical musculoskeletal disease management. Skin lesions in this patient cleared using our newly standardized multimodal therapy program for NP and BRP utilizing a customized clearing and maintenance phase schedule directed at cervical spinal and disc disease at C4-C6. The main components of this program utilized in office and at-home TENS and EMS, cervical acupuncture, phototherapy with Narrow band ultraviolet B (NB UVB) for generalized pruritus control, physical therapy referral, and consultation with Rheumatology and Pain Management. Patient was doing very well dermatologically at a 3 year follow up with complete resolution of symptoms and remission of skin findings. She recently notified us of a new diagnosis of systemic lymphoma.

Conclusion

Notalgia paresthetica (NP) and Brachioradial Pruritus (BR) are not true dermatologic disorders, but rather cutaneous signs of underlying systemic disease (1-5). NP and BRP are predominantly associated with varying degrees of cervical muscoskeletal disease ranging from asymptomatic and extremely mild cervical muscle spasm, to severe spinal stenosis and cord impingement at C4-C6 with corresponding upper extremity radiculopathy.  The striking association of NP and BRP with degenerative or traumatic cervico-thoracic spine disease suggests that cervical arthropathy or maylagia often contribute to the pathogenesis of these skin symptoms of both diseases (1-5). Additional studies are needed to further assess the relationship of NP and BRP with cervical spine disease and comorbidities predisposing certain individuals to developing these dermatologic manifestations. The most successful treatments for these neurocutaneous diseases center around treating the cervical pathology first (2).

We propose that the new rule in NP and BRP is to treat the neck, and the skin will follow. While topical therapies have historically been utilized as first line therapy in NP and BRP, we also propose a best practices paradigm shift in the global assessment and approach to these diseases with therapies aimed at the cervical region. Broader scope spinal evaluation and systemic laboratory and imaging evaluation for underlying causes of pruritus may be warranted to fully evaluate refractory the underlying causes. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of refractory NP and BRP.

References

  1. Alai NN, Skinner HB.  Concurrent Notalgia paresthetica and Brachioradial pruritus (BRP) associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C6. Accepted for publication Cutis 2016
  2. Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: cervical spine disease and neurogenic/neuropathic pruritus. J Am Acad Dermatol. 2004 May; 50(5):800-1.
  3. Savk E, Savk SO.On brachioradial pruritus and notalgia paresthetica. J Am Acad Dermatol. 2003 Apr;48(4):521-4.
  4. Savk E, Savk O, Bolukbasi O, Culhaci N, Dikicioğlu E, Karaman G, Sendur N. Notalgia paresthetica: a study on pathogenesis. Int J Dermatol. 2000 Oct; 39(10):754-9.

 

 

 

 

 

 

 

 

Refractory Concurrent Atypical Notalgia Parethetica (NP) and Brachioradial pruritus (BRP) Associated with Hepatitis C and Rheumatoid Arthritis

Notalgia paresthetica (NP)and Brachioradial Pruritus (BRP) are heavily under-recognized and under-diagnosed common causes of  episodic and chronic, localized, dysesthesias  and itching of the mid back and upper extremities respectively. 1, 2 While NP and BRP are often described as independent conditions, these 2 conditions do often occur concurrently or serially in the same individual. 2 Figure 1 demonstrates atypical NP/BRP with zosteriform erosions on the right deltoid and lateral scapular regions. Figure 2 depicts zosteriform erosions on the left low back, but noted to cross midline. Figure 3 demonstrates Notalgia Paresthetica with a hyperpigmented, lichenified pink plaque of the left low back with small excoriations.

NP and BRP may in fact represent the same spectrum of one pathophysiologic neuropathic disorder originating from the cervical spine. 2 Although BRP and NP have long been considered dermatologic conditions, both are more accurately described as sensory neuropathies. 1-4 Both NP and BRP’s well described associations with underlying cervical musculoskeletal disease in many patients warrants categorization of these disorders as a primary neuropathy with cutaneous manifestations. 1,2 A paradigm shift in the dermatologist’s approach to treatment of NP and BRP is warranted, with a focus on eliciting relevant spinal history including cervical muscle spasm, neck trauma, arthritis, degenerative disk disease, and other spinal neoplasms. 1,2 Topical therapies for NP and BRP have been highly inconsistent and unsatisfactory in a majority of patients. 1-4 Further, appropriate patient education for both BRP and NP requires discussion of potential cervical conditions and clear awareness of the probable association of the localized itching with underlying cervical disease. 2

Radiographic studies of the spine including plain film and MRI may be more considered in primary evaluation of both NP and BRP than current standards. Figure 4 shows an MRI of this patient’s Spine which demonstrated osteophytes, arthropathy, and cervical disc disease at C4-C6.

Similar to prognostication of standard cervical musculoskeletal diseases, NP and BRP are generally chronic, controllable conditions with periodic remissions and exacerbations. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, pain management, acupuncture, physical therapy and orthopedics surgery may be indicated in best practices of management of these 2 conditions. 1,2 First line therapy for NP and BRP with associated cervical disease should include consideration of potential non-dermatologic therapies aimed at the spine. Such cervical spinal treatments include transcutaneous electrical stimulation (TENS), acupuncture, electrical muscle stimulation (EMS), cervical traction, spinal manipulation, physical therapy, massage, cervical muscle strengthening and range of motion enhancing routines, neck passive continuous motion (PCM), oral non-steroidal anti-inflammatory medications, and oral and topical muscle relaxants. NP and BRP are both in fact dermatologic signs of underlying systemic disease. 1-4 We describe a concurrent case of refractory NP and BRP in a 67 year old female successfully controlled with cervical musculoskeletal disease management.

Case Report

We report a unique case of presentation of a concurrent atypical NP and BRP in a Hepatitis C patient with rheumatoid arthritis. A 67 year old female presented with greater than 3 years of refractory and debilitating intermittent bouts of recurrent itching on the left infrascapular skin in the T5-T6 dermatome and 2 month onset of right arm and shoulder tingling and small erosions. Figure 1 shows the clinical picture of BRP mimicking dermatomal Varicella Zoster. Skin examination of the mid back revealed multiple fresh superficial excoriations on a slightly dusky, tan to pink non-indurated patch with mild associated sensory alternations to light touch and pin prick. Orthopedic examination confirmed moderate spinal scoliosis, marked decreased range of motion in the neck with associated marked bilateral cervical muscle spasm and tenderness to palpation, particularly on the left side. Past medical history of the patient was significant for rheumatoid arthritis (RA), post transfusion acquired Hepatitis C, borderline anemia of chronic disease, and iron deficiency. Pertinent history included 1-2 years status post multiple mild-moderate automobile related whiplash injuries with subsequent intermittent interscapular and neck pain. Past treatments for neck and back pain included intermittent spinal adjustments, physical therapy, trigger point injections, paravertebral ganglion blocks with a pain specialist, and various other failed medical treatments including oral gabapentin and muscle relaxants. An atypical dermatomal distribution of BRP and skin dysesthesias mimicked Herpes Zoster on her shoulder. Negative viral laboratory testing,  infectious disease consultation, and failed treatment with oral valacyclovir excluded a viral infectious etiology. Histopathologic examination of the involved back and arm skin biopsies demonstrated nonspecific findings of superficial and perivascular dermal inflammation with mild, focal secondary impetiginization.

 

Skin lesions cleared using a newly standardized multimodal therapy program for notalgia utilizing a customized clearing and maintenance phase schedule directed at cervical spinal and disc disease at C4-C6. The main components of this program utilized in office and at-home TENS and EMS, cervical acupuncture, phototherapy with Narrow band ultraviolet B (NB UVB) for generalized pruritus control, physical therapy referral, and consultation with Rheumatology and Pain Management. Patient was doing very well dermatologically at a 3 year follow up with complete resolution of symptoms and remission of skin findings. She recently notified us of a new diagnosis of systemic lymphoma.

Conclusion

Notalgia paresthetica (NP) and Brachioradial Pruritus (BR) are not true dermatologic disorders, but rather cutaneous signs of underlying systemic disease. 1-5 NP and BRP are predominantly associated with varying degrees of cervical muscoskeletal disease ranging from asymptomatic and extremely mild cervical muscle spasm, to severe spinal stenosis and cord impingement at C4-C6 with corresponding upper extremity radiculopathy.  The striking association of NP and BRP with degenerative or traumatic cervico-thoracic spine disease suggests that cervical arthropathy or maylagia often contribute to the pathogenesis of this skin symptoms of both diseases. 1-5 Additional studies are needed to further assess the relationship of NP and BRP with cervical spine disease and comorbidities predisposing certain individuals to developing these dermatologic manifestations. The most successful treatments for these neurocutaneous diseases center around treating the cervical pathology first. 2 We propose that the new rule in NP and BRP is to treat the neck, and the skin will follow. While topical therapies have historically been utilized as first line therapy in NP and BRP, we propose a paradigm shift in the global assessment and approach to these diseases with therapies aimed at the cervical region. Broader scope spinal evaluation and systemic laboratory and imaging evaluation for underlying causes of pruritus may be warranted to fully evaluate refractory the underlying causes. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of refractory NP and BRP.

Reference

1. Alai NN, Skinner HB, Nabili ST, Jeffes E,  Shahrokhni S, Saemi AM.  Notalgia paresthetica associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C7. Cutis 85(2):77-81 (2010) PMID 20349681

2. Alai NN, Skinner HB Concurrent Notalgia paresthetica and Brachioradial pruritus (BRP) associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C6. Accepted for publication Cutis 2016

3.  Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: cervical spine disease and neurogenic/neuropathic pruritus. J Am Acad Dermatol. 2004 May;50

(5):800-1.

4. Savk E, Savk SO.On brachioradial pruritus and notalgia paresthetica.

J Am Acad Dermatol. 2003 Apr;48(4):521-4.

5. Şavk, Ekin; Şavk, Ş. Öner; Bolukbasi, Okan; Ccedilulhaci, Nil1; Dikicioğlu, Emel; Karaman, Göksun; Şendur, Neslihan. Notalgia paresthetica: a study on pathogenesis. International Journal of Dermatology, Volume 39, Number 10, October 2000 , pp. 754-760(7).

 

Figure 1 Atypical NP/ BRP with zosteriform erosions on the right deltoid and lateral scapular region.

 

 

 

 

 

 

 

 

 

 

 

 

 

 


NOTALGIA PARESTHETICA  – Successful Treatment

with TENS


INTRODUCTION

Background

Notalgia paresthetica (NP) is a sensory neuropathic syndrome of the back skin, classically of the unilateral infrascapula. It is primarily a localized pruritus syndrome. Notalgia paresthetica was first named in 1934 and described as episodic itching or pain on a small patch of the mid back, usually an area of skin just past easy reach.

Additional features of Notalgia paresthetica may include localized burning, pain, tenderness, hyperalgesia, or dysesthesias. Notalgia paresthetica may be associated with a poorly circumscribed tan or hyperpigmented patch in the symptomatic area. Notalgia paresthetica  tends to be a chronic condition with periodic remissions and exacerbations. While not life threatening and not generally associated with other co-morbidities, it does frequently decrease quality of life causing much discomfort and nuisance to the affected patients.



  

                       Photos of Notalgia Paresthetica : One sided itch on mid back

 


NOTALGIA PARESTHETICA( NP)


INTRODUCTION

Background

Notalgia paresthetica (NP) is a sensory neuropathic syndrome of the back skin, classically of the unilateral infrascapula. It is primarily a localized pruritus syndrome. Notalgia paresthetica was first named in 1934 and described as episodic itching or pain on a small patch of the mid back, usually an area of skin just past easy reach.

Additional features of Notalgia paresthetica may include localized burning, pain, tenderness, hyperalgesia, or dysesthesias. Notalgia paresthetica may be associated with a poorly circumscribed tan or hyperpigmented patch in the symptomatic area. Notalgia paresthetica  tends to be a chronic condition with periodic remissions and exacerbations. While not life threatening and not generally associated with other co-morbidities, it does frequently decrease quality of life causing much discomfort and nuisance to the affected patients.

 

Pathophysiology

The exact pathophysiology of the cutaneous findings of notalgia paresthetica remain unknown.

Although the etiology of notalgia paresthetica is unclear, two of the multiple proposed possible mechanisms include 1) localized increased sensory innervation of the affected skin areas and 2) neuropathy from degenerative cervico-thoracic disc disease or direct nerve impingement. 5,6,7

Savk et al in 2000 showed more than half of their patients had significant radiographic changes in the vertebrae corresponding to the dermatome of the cutaneous lesion. Further, all study patients demonstrated normal neurological examination and standard electrodiagnostic results. All had skin histopathology compatible with post inflammatory hyperpigmentation.7 There were no amyloid deposits or other described pathology on pathologic exam of the skin.6,7                                        

Springer et al in 1990 concluded that the symptoms of notalgia paresthetica may in part be related to an increase in the sensory epidermal innervation in the affected skin areas. 5 Histological studies have shown cutaneous changes in a few cases including lichen amyloid which may be secondary to the localized chronic scratching and rubbing. 1,6

 

Frequency

United States

Notalgia paresthetica (NP) is a relatively common disorder which remains largely underdiagnosed. Therefore, the true frequency may not be accurately reportable. It is described worldwide in all races.

International

Mortality/Morbidity

While not life threatening and not generally associated with other non-spine co-morbidities, the cutaneous symtpoms of notalgia paresthetica frequently decrease quality of life causing much discomfort and nuisance to the affected patients.

There may be some increased morbidity because of the possible underlying cervical and thoracic spine and disc disease. Notalgia paresthetica tends to be a chronic condition with periodic remissions and exacerbations. There is no described increased mortality with this disorder.

Race

Notalgia paresthetica may be seen in all races without any described racial predilection.

Sex

Notalgia paresthetica may be seen in both males and females, although there seems to be an increase in females.

Age

Notalgia paresthetica is more common in adulthood, typically in ages 40-80.


CLINICAL

History

Notalgia paresthetica (NP) patients often present with the hallmark symptom of localized pruritus of the unilateral infrascapula. 

Physical

Notalgia paresthetica classically presents with skin findings of a unilateral, ill defined, tan, pink, or hyperpigmented non-indurated patch of the infrascapular back (mid back). The affected skin area usually ranges in size from 3-10cm.

Secondary skin changes such as lichenification, lichen amyloid, excoriations, eczema, xerosis, and secondary infection may be noted. There may be associated mild sensory alternations to light touch, vibration, and pin prick.

Examination of the spine may be normal or reveal tenderness, decreased range of motion in the neck, and possible associated cervical muscle spasm.

Causes

The exact cause of the cutaneous findings of notalgia paresthetica remain unknown. Notalgia paresthetica may in fact be a dermatologic sign of an underlying systemic disease

Notalgia paresthetica may not be solely a skin disease per se but a cutaneous sign of an underlying degenerative cervical spine disease. The striking association of notalgia paresthetica with degenerative or traumatic cervico-thoracic spine disease suggests that early spinal nerve impingement may contribute to the pathogenesis of the skin symptoms of the disease.

Additional studies are needed to further assess the relationship of notalgia paresthetica with cervical spine disease. Whether this is a causal or coincidental finding remains to be determined in larger studies. While topical therapies may in some cases seemingly help decrease the localized symptoms in notalgia paresthetica , systemic or broader scope spinal evaluation may be warranted to fully evaluate refractory cases. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of notalgia paresthetica.


DIFFERENTIALS

Arthropod Bite Reaction
Atopic Dermatitis
Brachioradial Pruritus
Contact Dermatitis
Delusions of Parasitosis
Drug Eruptions
Fixed Drug Eruption
Herpes Zoster
Impetigo
Lichen Simplex Chronicus
Neurodermatitis
Postinflammatory Hyperpigmentation
Prurigo Nodularis
Pruritus and Systemic Disease
Tinea Corporis
Tinea Versicolor
Xerosis

 


Other Problems to Be Considered

Other problems to be considered include cervical and thoracic spine disease. Additional radiographic studies may be warranted  to further assess possible underlying cervical spine disease.


WORKUP

Lab Studies

Although laboratory tests are generally not required in the workup of notalgia paresthetica, a basic pruritus workup may be helpful in select cases based on history and contributory symptoms.

Imaging Studies

Although imaging tests have traditionally not been a part of the workup of notalgia paresthetica, basic cervical and possibly thoracic x-rays or MRI may be warranted in the initial management of the disorder. Imaging studies may be  particularly helpful in patients with contributory spine symptoms of pain, tenderness, spasm or decreased range of motion and any history of spinal trauma or injury.

Histologic Findings

Skin biopsy and tissue histology are usually not indicated for the diagnosis of notalgia paresthetica. Biopsies may be done to exclude other diagnosis and neoplasms. There are no described criteria for tissue diagnosis of notalgia paresthetica. Prior studies have shown various histologic findings including postinflammatory hyperpigmentation and lichen amyloid.


TREATMENT

Medical Care

Treatments of notalgia paresthetica with topical modalities have generally failed and are refractory because of the difficult to reach location. To date, there has been no clearly described etiology and no uniformly effective treatment for notalgia paresthetica.

Topical therapies aimed at the back skin may be in fact be ineffectual or partially effective as basic emollients. Since notalgia paresthetica does have periodic spontaneous remissions and exacerbations, it may be difficult to accurately measure response to various therapies. A placebo response may be considered with some therapies.

During the initial assessment of patients with notalgia paresthetica, it is important to obtain a thorough past history of osteoarthritis, prior neck trauma, motor vehicle accident, vertebral fracture, cervical neoplasm or malignancy, or cervical disc disease. Even in the absence of positive medical history, radiographs or MRI of the cervical spine may aid in early diagnosis and treatment of degnerative spine disease.

The striking association of notalgia paresthetica with degenerative or traumatic cervico-thoracic spine disease suggests that early spinal nerve impingement may contribute to the pathogenesis of the skin symptoms of the disease. Additional studies are needed to further assess the relationship of notalgia paresthetica with cervical spine disease. Whether this is a causal or coincidental finding remains to be determined in larger studies. While topical therapies may in some cases seemingly help decrease the localized symptoms in notalgia paresthetica, systemic or broader scope spinal evaluation may be warranted to fully evaluate refractory cases. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of notalgia paresthetica.

In the future, first line therapy for notalgia paresthetica with associated cervical disease may include non-dermatologic, non-invasive treatments such as spinal manipulation, physical therapy, cervical soft collars, massage, cervical traction, cervical muscle strengthening and increased range on motion, transcutaneous electrical nerve stimulation *TENS), cervical discectomy with fusion, oral non-steroidal anti-inflammatory medications (ibuprofen, celecoxib,  ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other medical and surgical measures for degenerative disc cervical disease and nerve impingement as introduced may also be considered.

For more generalized and chronic pruritus, full laboratory workup including complete blood count, chemistry panel including renal and liver functions, chest x-ray, and other studies may be warranted to exclude underlying physiologic causes of pruritus. Alternatively, proper management of NP may involve a multi-specialty cooperative effort of dermatology with radiology, orthopedic surgery, neurology, and possibly adjunctive fields including acupuncture, chiropractic, and physical therapy.

Surgical Care

Surgical therapy for notalgia paresthetica with associated cervical disease may include discectomy with fusion, disc replacement surgery, minimally invasive injectable disc repair techniques, and other surgical measures for degenerative cervical disease and nerve impingement.

Consultations

Proper evaluation and management of notalgia paresthetica may involve a multi-specialty cooperative effort of dermatology with radiology, orthopedic surgery, neurology, pain management, and possibly adjunctive fields including acupuncture, massage, chiropractic, and physical therapy.

Consultations with other specialists may be warranted based on radiologic findings and individual patient history and physical exam.

Diet

There are no dietary treatments or associated factors decribed.

Activity

Certain physical activities may potentially worsen notalgia paresthetica via exacerbation of the underlying cervicothoracic spine disease.


MEDICATION

While to date there has been no uniformly effective treatment for the cutaneous symptoms of notalgia paresthetica, common first line medications include potent topical steroid creams.

Currently available therapeutic options for the localized itch syndromes include capsaicin cream11, eutectic mixture of local anesthetic (EMLA) cream, topical steroids, pramoxine cream, topical cooling or ice pack applications, oral steroids, Tiger balm, menthol creams, flurandrenolide tape (Cordran Tape), intralesional corticosteroid injections, botulinum toxin injections,12 oral antihistamines, hydroxyzine, doxepin, topamax, anticonvulsant medications, carbamazepine (Tegretol) antidepressant medications, gabapentin (Neurontin), oxcarbazepine,13 topiramate, thalidomide,14 and many others.

It is possible that some of the current systemic therapies may in fact exert their effect through the spinal nerves and central nervous system thereby supporting the neuropathic etiology of notalgia paresthetica.3,5,16,17

First line therapy for notalgia paresthetica with associated cervical or cervicothoracic disease may include non-dermatologic medications such as oral non-steroidal anti-inflammatory medications (ibuprofen, celecoxib,  ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other medical and surgical measures for degenerative cervical disc disease and nerve impingement as introduced may also be considered

Drug Category: Corticosteroid, Topical (very High Potency)

Drug Name Clobetasol Propionate
Description Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction. Decreases inflammation by stabilizing lysosomal membranes, inhibiting PMN and mast cell degranulation.
Adult Dose Apply bid for up to 2 wk; not to exceed 50 g/wk
Pediatric Dose <12 years: Not recommended

>12 years: Administer as in adultsContraindicationsDocumented hypersensitivity; viral or fungal skin infectionsInteractionsNone reportedPrecautionsMay suppress adrenal function in prolonged therapyPregnancyC – Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Drug Category: Corticosteroid, Topical (high Potency)

Drug Name Fluocinonide
Description High-potency steroid, inhibits cell proliferation, is immunosuppressive, antiproliferative, and anti-inflammatory. Also has antipruritic, and vasoconstrictive properties.
Adult Dose Apply sparingly bid/qid as severity warrants
Pediatric Dose Administer as in adults
Contraindications Documented hypersensitivity; herpes simplex infection; fungal, viral, or tubercular skin lesions
Interactions None reported
Precautions May cause adverse systemic effects if used over large areas, denuded areas, on occlusive dressings, or during prolonged treatment periods
Pregnancy C – Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Drug Category: anti-pruritus

Drug Name Hydroxyzine hydrochloride
Description Antagonizes H1 receptors in periphery. May suppress histamine activity in subcortical region of CNS.
Adult Dose 25-100 mg PO qd/qid
Pediatric Dose 0.6 mg/kg/dose PO q6h
Contraindications Documented hypersensitivity
Interactions CNS depression may increase with alcohol or other CNS depressants
Precautions Associated with clinical exacerbations of porphyria (may not be safe for porphyric patients); ECG abnormalities (alterations in T-waves) may occur; may cause drowsiness
Pregnancy C – Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Drug Category: Analgesic, Topical

Drug Name Capsaicin
Description Natural chemical derived from plants of Solanaceae family. Penetrates deep for temporary relief of minor aches and pains of muscles and joints associated inflammatory reactions. Derived from plants of Solanaceae family. May render skin and joints insensitive to pain by depleting substance P in peripheral sensory neurons. Has demonstrated effectiveness in several studies of diabetic neuropathic pain and in other types of neuropathic pain.
Adult Dose Apply to affected area tid/qid for 3-4 consecutive wk and evaluate efficacy; not to exceed 4 applications/d; wash hands with soap and water after applying
Pediatric Dose Not established
Contraindications Documented hypersensitivity; broken or irritated skin
Interactions None reported
Precautions For external use only; avoid contact with eyes; do not use tight bandage; discontinue use if condition worsens or symptoms persist for 14-28 d
Pregnancy C – Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Drug Category: Corticosteroid, Topical (medium Potency)

Drug Name Triamcinolone
Description For inflammatory dermatosis responsive to steroids; decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Available in ointment (0.1%) and cream (0.025%, 0.1%, 0.5%).
Adult Dose Apply thin film bid/tid to response
Pediatric Dose Apply as in adults
Contraindications Documented hypersensitivity; fungal, viral, and bacterial skin-infections
Interactions None reported
Precautions Do not use in decreased skin circulation; prolonged use, applications over large areas, and use of potent steroids and occlusive dressings may result in systemic absorption; systemic absorption may cause Cushing’s syndrome, reversible HPA axis suppression, hyperglycemia and glycosuria
Pregnancy C – Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Prognosis

Notalgia paresthetica tends to be a chronic disease with periodic remissions and exacerbations. The prognosis for control of the symptoms is good. It is not curable.

Patient Education

Patient education involves discussion of possible underlying causes and associations with cervico-thoracic spinal disease. Patients need to be advised of potential disease flare with exacerbations of their spinal disease.

At a Glance

  • Sensory  syndrome of the back skin
  • May include localized burning, pain, tenderness, hyperalgesia, or dysesthesias on a small patch of the mid back just under the shoulder blade (scapula)
  • Associated with a poorly circumscribed tan or hyperpigmented patch
  • Chronic condition
  • Usually gets worse in the evening and just before bedtime
  • Causes discomfort and nuisance to affected patients
  • Not life threatening
  • Common disorder which remains largely underdiagnosed
  • Effective treatments are available including TENS unit applied to the neck base, neck and upper back massage, neck range of motion exercises, and expert physical therapy
  • Key words for Notalgia include Notalgia paresthetica, NP, Notalgia, sensory disturbance of the back, one sided back itch, unilateral itching of back, interscapular itching, infrascapular itch, PPP, PPPP, pigmented pruritic posterior patch, pigmented posterior patch, skin dysesthesia of back, localized pruritus syndrome, sensory neuropathic syndrome, hyperalgesia, unilateral back itching, nostalgia, nostalgia paresthtica, notalgia paresthetic, what is notalgia, nostalgia paresthetica, photos of notalgia, pictures of notalgia parsthetica, best treatment for notalgia, Specialist for notalgia, back itching, bra allergy, back rash, TENS, transcutaneous electrical nerve stimulator, neck muscle spasm, paresthetica-notalgia, pareaesthetica, bra strap allergy, detergent allergy back, neck itching, shoulder itching, arm itch, itchy back, itchy scalp, itchy underarms, scalp itching, rash mid back, back rash, itchy back from bra, itchy bra,

REFERENCES

  • Notalgia paresthetica associated with nerve root impingement.
  • Eisenberg E, Barmeir E, Bergman R. Notalgia paresthetica associated with nerve root impingement. J Am Acad Dermatol. Dec 1997;37(6):998-1000. [Medline].
  • Misery L. What is notalgia paresthetica?. Dermatology. 2002;204(2):86-7. [Medline].
  • Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: cervical spine disease and neurogenic/neuropathic [corrected] pruritus. J Am Acad Dermatol. Apr 2003;48(4):521-4. [Medline].
  • Bernard PA, Wayne ME. Notalgia paresthetica. Neurology. 1978;28:1310-.
  • Springall DR, Karanth SS, Kirkham N, Darley CR, Polak JM. Symptoms of notalgia paresthetica may be explained by increased dermal innervation. J Invest Dermatol. Sep 1991;97(3):555-61. [Medline].
  • Weber PJ, Poulos EG. Notalgia paresthetica. Case reports and histologic appraisal. J Am Acad Dermatol. Jan 1988;18(1 Pt 1):25-30. [Medline].
  • Savk O, Savk E. Investigation of spinal pathology in notalgia paresthetica. J Am Acad Dermatol. Jun 2005;52(6):1085-7. [Medline].
  • Savk E, Savk O, Sendur F. Transcutaneous electrical nerve stimulation offers partial relief in notalgia paresthetica patients with a relevant spinal pathology. J Dermatol. May 2007;34(5):315-9. [Medline].
  • Savk E, Savk O, Sendur F. Transcutaneous electrical nerve stimulation offers partial relief in notalgia paresthetica patients with a relevant spinal pathology. J Dermatol. May 2007;34(5):315-9. [Medline].
  • Savk E, Savk O. On brachioradial pruritus and notalgia paresthetica. J Am Acad Dermatol. 2003;48:521-524.
  • Goodless DR,Eagelstein WH. Brachioradial pruritus treatment with capsacin. J Am Acad Dermatolog. 1993;29:783-784.
  • Tait CP, Grigg E, Quirk CJ. Brachioradial pruritus and cervical spine manipulation. Australas J Dermatol. Aug 1998;39(3):168-70. [Medline].
  • Weinfeld PK. Successful treatment of notalgia paresthetica with botulinum toxin type A. Arch Dermatol. Aug 2007;143(8):980-2. [Medline].
  • Savk E, Bolukbasi O, Akyol A, Karaman G. Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica. J Am Acad Dermatol. Oct 2001;45(4):630-2. [Medline].
  • Pereira J. Brachioradial pruritus treated with thalidomide. An Bras Dermatol. 2005;80:295-6.


Author: Dr. Nili N. Alai, M.D. , FAAD 

Dr. Alai is an expert on notalgia paresthetica diagnosis and effective treatment. Her newest scientific publications on this topic have recently been accepted for print in Cutis (Dermatology Journal) and Emedicine (online medical Dermatology textbook).

 

 


CASE REPORT

 

Author: Dr. Nili N. Alai, M.D, FAAD

U.S. Board Certified Dermatologist

Dr. Alai

Notalgia paresthetica associated with cervical spinal stenosis and disc disease at C5-C7  


Abstract

Notalgia paresthetica (NP) is a common, refractory sensory neuropathic syndrome with the hallmark symptom of localized pruritus of the unilateral infrascapula. It is generally a chronic, non-curable condition with periodic remissions and exacerbations. While the dermatologic syndrome may be multi-factorial in etiology, its possible association with underlying cervical spine disease needs to be evaluated for proper treatment. Radiographic studies of the spine may be more considered than they are currently. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, and orthopedics may be indicated in primary management of this condition. First line therapy for notalgia paresthetica with associated cervical disease may include non-dermatologic spinal treatments such as spinal manipulation, physical therapy, massage, cervical traction, cervical muscle strengthening, and oral non-steroidal anti-inflammatory medications and muscle relaxants. Notalgia paresthetica may in fact be a dermatologic sign of an underlying systemic disease.

Case Report

37 year old patient presented with 3 years of intermittent bouts of recurrent itching on the right infrascapular skin of the back in T5-T6 dermatome. Failed past Notalgia Paresthetica skin treatments had included topical clobetasol cream, fluocinonide cream, oral hydroxyzine, oral diphenhydramine, chlortrimeton, intralesional triamcinolone 2.5mg/cc, and Tiger balm.

MRI of the cervical and thoracic spine revealed C5- C6, and C6-C7 disc protrusions and multiple osteophytes at these levels.

Discussion

 

Notalgia paresthetica (NP) is a sensory neuropathic syndrome of the back, classically of the unilateral infrascapula. It is primarily associated with intense localized pruritus. NP was first named in 1934 and described as episodic itching or pain on a small patch of the mid back, usually an area of skin just past easy reach.

 

Additional features of the dermatologic condition may include localized burning, pain, tenderness, hyperalgesia, or dysesthesias.  Notalgia paresthetica may be associated with a poorly circumscribed tan or hyperpigmented patch in the symptomatic area. Notalgia paresthetica tends to be a chronic condition with periodic remissions and exacerbations. While not life threatening and not generally associated with other co-morbidities, it does frequently decrease quality of life causing much discomfort and nuisance to the affected patients.

 

Treatment with topical modalities have generally failed and are difficult because of the difficult to reach location. To date, there has been no clearly described etiology and no uniformly effective treatment for notalgia paresthetica.

 

Although the etiology of notalgia paresthetica is unclear, two of the multiple proposed possible mechanisms include 1) localized increased sensory innervation of the affected skin areas and 2) neuropathy from degenerative cervico-thoracic disc disease or direct nerve impingement.

A study by Savk et al in 2000 studying 10 patients with NP demonstrated normal neurological examination and standard electrodiagnostic results in all study patients. All had skin histopathology compatible with post inflammatory hyperpigmentation. There were no amyloid deposits or other described pathology on pathologic exam of the skin. Seven of the 10 cases confirmed radiographic changes in the vertebrae corresponding to the dermatome of the cutaneous lesion. 9

An earlier study by Springer et al in 1990 evaluating the mechanism of notalgia paresthetica studied whether the cutaneous symptoms were caused by alternations on the cutaneous innervation of the involved infrascapular area. They postulated that the histology findings with increased dermal innervation to the areas however no measurable change in the distribution of neuropeptide-immunoreactive axons was found. There was an increase in the number of intradermal PGP 9.5-immunoreactive nerve fibers and epidermal dendritic cells compared with unaffected areas from the same patients and normal controls. It was concluded that the symptoms of NP may in part be related to an increase in the sensory epidermal innervation in the affected skin areas. 3

A study by Wallengren et al from Sweden published in the Archives of Dermatology in 2001 demosntrated the effectiveness  of cutaneous field stimulation in NP and BRP patients. Their study showed a reduction in itching accompanied by degeneration of the epidermal nerve fibers, as evidenced by the loss of protein gene product 9.5 immunoreactivity.23

 

Histologic studies have shown cutaneous changes in a few cases including lichen amyloid which may be secondary to the localized chronic scratching and rubbing. 12

Clinical observations in orthopedics has established a clear relationship between the upper thoracic/interscapular region and the lower cervical spine. Frequently, cervical disc disease presents as referred pain in the upper thoracic and interscapular area. Similarly, some tumors of the cervical medulla have also presented as interscapular pain. 2

Some have speculated direct involvement and actual entrapment of the posterior rami of T2 to T6 spinal nerves. However, there is referred symptoms from the cervical area directly to the infrascapular back. Degenerative vertebral and disc changes corresponding to the affected dermatome may be observed in some cases. Recent literature supports a role for radiographic imagine of cervical and thoracic spine to exclude disc disease and possible nerve compromise.


With recent advances in radiography and availability of magnetic resonance imaging (MRI), earlier detection and intervention of cervical disc disease may be possible. Early recognition may promote timely intervention and treatment to prevent cervical spine disease progression. In addition to degenerative cervical discs, osteoarthritis, and cervical spine strain and muscle spasm, there may be a neoplasm or other pathology of the cervical spine contributing to notalgia paresthetica.


There is some thought that there may be a relation between notalgia paresthetica and brachioradial pruritus. The recently described association of many cases of brachioradial pruritus (BRP) and cervical spine disease and description of the disease as a possible neuropathic/ neurogenic condition also support a probable neuropathic association of nostalgia paresthetica. 5 In contrast, notalgia paresthetica is unilateral while BRP may be involving unilateral or bilateral upper extremities.


Topical therapies aimed at the back may be in fact be ineffectual or partially effective as basic emollients. Since the disease does have periodic spontaneous remissions and exacerbations, it may be difficult to accurately measure response to various therapies. A placebo response may be considered with some therapies.

The differential diagnosis in notalgia paresthetica may include allergic or irritant contact dermatitis, fixed drug eruption, dermatophytosis, neoplasm, lichen amyloid, arthropod reaction, lichen simplex chronicus, neurodermatitis, infection, other hypersensitivity reaction.

During the initial assessment of patients with notalgia paresthetica, it is important to obtain a thorough past history of osteoarthritis, prior neck trauma, motor vehicle accident, vertebral fracture, cervical neoplasm or malignancy, or cervical disc disease. In the absence of positive medical history, radiographs or MRI of the cervical spine may aid in diagnosis and treatment. Further, a positive family history of osteoarthritis or vertebral disc disease may be contributory.

When pruritus is generalized and persistent, a full laboratory workup including complete blood count, chemistry panel including renal and liver functions, chest x-ray, and other studies may be warranted to exclude other causes.

Proper management of notalgia paresthetica may involve a multi-specialty cooperative effort of dermatology with radiology, orthopedic surgery, neurology, and adjunctive fields including acupuncture, chiropractic, and physical therapy.

While to date there has been no uniformly effective treatment, current therapeutic options for notalgia paresthetica include capsaicin cream, eutectic mixture of local anesthetic (EMLA) cream, topical steroids, pramoxine cream, topical cooling, oral steroids, Tiger balm, menthol creams, Cordran tape, intralesional corticosteroid injections, botulinum toxin injections, 11 oral antihistamines, hydroxyzine, doxepin, topamax, anticonvulsant medications, carbamazepine (Tegretol) antidepressant medications, gabapentin (Neurontin), oxcarbazepine, 14 topiramate, thalidomide ,10 paravertebral local anesthetic block, 15 cervical epidural injection, surgical resection of the rib, and many others. Some of the current systemic therapies may in fact exert their effect through the spinal nerves and central nervous system thereby supporting the neuropathic etiology of NP.

In the future, first line therapy for notalgia paresthetica with associated cervical disease may include non-dermatologic, TENS or transcutaneous electrical nerve stimulation, non-invasive treatments such as spinal manipulation, physical therapy, cervical soft collars, massage, cervical traction, cervical muscle strengthening and increased range on motion, cervical discectomy with fusion, oral non-steroidal anti-inflammatory medications (ibuprofen, celecoxib, ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other measures for degenerative disc disease as introduced may also be considered.

Conclusions

Notalgia paresthetica may not be solely a skin disease per se but a cutaneous sign of an underlying degenerative cervical spine disease. The striking association of notalgia paresthetica with degenerative or traumatic cervico-thoracic spine disease suggests that early spinal nerve impingement may contribute to the pathogenesis of this skin symptoms of the disease. Additional studies are needed to further assess the relationship of notalgia paresthetica with cervical spine disease. Whether this is a causal or coincidental finding remains to be determined in larger studies. While topical therapies may in some cases seemingly help decrease the localized symptoms in notalgia paresthetica, systemic or broader scope spinal evaluation may be warranted to fully evaluate refractory cases. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of notalgia paresthetica.

  






 

Case Report and Review of the Literature ( as seen in Cutis 2010: CME Albert Einstein Medical College)

Notalgia paresthetica associated with cervical spinal stenosis and disc disease at C4-C7

Author : Dr. Nili Alai

Abstract

Notalgia paresthetica (NP) is a common, refractory sensory neuropathic syndrome with the hallmark symptom of localized pruritus of the unilateral infrascapula.  It is generally a chronic, non-curable condition with periodic remissions and exacerbations. While the dermatologic syndrome may be multi-factorial in etiology, its possible association with underlying cervical spine disease needs to be evaluated for proper treatment. Radiographic studies of the spine may be considered more than they are currently. Collaborative multi-specialty evaluation by dermatology, radiology, neurology, and orthopedics may be indicated in primary management of this condition. First line therapy for notalgia paresthetica with associated cervical disease may include non-dermatologic spinal treatments such as spinal manipulation, physical therapy, massage, cervical traction, cervical muscle strengthening, and oral non-steroidal anti-inflammatory medications and muscle relaxants. NP may in fact be a dermatologic sign of an underlying systemic disease. We describe the case of a patient with NP in whom cervical nerve root impingement directly corresponded with the clinical findings.

Case  Report

37 year old right handed white female presented with 2 years of intermittent bouts of recurrent itching on the right infrascapular skin of the back in T5-T6 dermatome. Figure 1 shows the clinical picture of NP. Skin examination revealed a slightly dusky, tan to hyperpigmented non-indurated patch of the right mid back without associated sensory alternations to light touch, vibration, or pin prick. Orthopedic examination confirmed decreased range of motion in the neck with associated marked bilateral cervical muscle spasm, particularly on the right side.

MRI of the cervical and thoracic spine revealed C4- C5, and C5-C6 disc bulges and multiple osteophytes at these levels. Figure 2 shows the MRI findings. General laboratory workup revealed normal complete blood count, full chemistry and hepatic panel, normal IGE levels, negative H. pylori IgG and IGM, HIV and hepatitis panel except for positive Hepatitis B surface antibody from prior vaccination. Chest x-ray and computerized tomography (CT) scan of chest, abdomen, and pelvis were negative.

Past medical history of the patient was significant for atopy, allergies, asthma, and allergy to sulfonamide. Pertinent history included 15-20 years status post multiple mild automobile related whiplash injuries with subsequent intermittent interscapular and neck pain. Prior treatments for neck and back pain had included intermittent chiropractic spinal adjustments, physical therapy, acupuncture, trigger point injection with triamcinolone, and botulinum toxin intramuscular neck injections. Failed past NP skin treatments had included potent topical steroids including clobetasol cream, oral antihistamines including hydroxyzine,diphenhydramine, and chlortrimeton, as well as intralesional triamcinolone 2.5mg/cc.

Three months following the initial presentation, the patient presented with an acute onset of a markedly pruritic raised 2-3 cm dusky plaque arising just below her typical NP patch. The patient requested injectable treatment for the refractory lesion which had failed applications of topical clobetasol twice daily for 5 days. Intralesional triamcinolone 2.5 mg /cc (2 cc) was performed which quickly relieved the symptoms but subsequently resulted in longstanding dermal atrophy and hyperpigmentation in the treated area.

During the last 2 years, the patient had logged a direct correlation of exacerbation of the NP skin symptoms with onset of cervical pain. Spontaneous remissions were observed in the skin discoloration and itching corresponding with a temporary decrease in neck and interscapular pain.

Discussion

Notalgia paresthetica (NP) is a sensory neuropathic syndrome of the back skin, classically of the unilateral infrascapula. It is primarily a localized pruritus syndrome. NP was first named in 1934 and described as episodic itching or pain on a small patch of the mid back, usually an area of skin just past easy reach.1,2,3,4,5 Additional features of the dermatologic condition may include localized burning, pain, tenderness, hyperalgesia, or dysesthesias. NP may be associated with a poorly circumscribed tan or hyperpigmented patch in the symptomatic area. NP tends to be a chronic condition with periodic remissions and exacerbations. While not life threatening and not generally associated with other co-morbidities, it does frequently decrease quality of life causing much discomfort and nuisance to the affected patients.

Treatments of NP with topical modalities have generally failed and are refractory because of the difficult to reach location. To date, there has been no clearly described etiology and no uniformly effective treatment for NP.  Although the etiology of NP is unclear, two of the multiple proposed possible mechanisms include 1) localized increased sensory innervation of the affected skin areas and 2) neuropathy from degenerative cervico-thoracic disc disease or direct nerve impingement. 5,6,7

A study by Savk et al in 2000 showed 7 out of 10 patients with NP had significant  radiographic changes in the vertebrae corresponding to the dermatome of the cutaneous lesion.  Further, all study patients demonstrated normal neurological examination and standard electrodiagnostic results. All had skin histopathology compatible with post inflammatory hyperpigmentation.7 There were no amyloid deposits or other described pathology on pathologic exam of the skin.6,7

An earlier study by Springer et al in 1990 evaluating the mechanism of NP studied whether the cutaneous symptoms were caused by alternations on the cutaneous innervation of the involved infrascapular area. They postulated that the histology findings with increased dermal innervation to the areas however no measurable change in the distribution of neuropeptide-immunoreactive axons was found. There was an increase in the number of intradermal PGP 9.5-immunoreactive nerve fibers and epidermal dendritic cells compared with unaffected areas from the same patients and normal controls. It was concluded that the symptoms of NP may in part be related to an increase in the sensory epidermal innervation in the affected skin areas. 5 Histological studies have shown cutaneous changes in a few cases including lichen amyloid which may be secondary to the localized chronic scratching and rubbing. 1,6

Clinical observations in orthopedics have established a clear relationship between the upper thoracic/interscapular region and the lower cervical spine. Frequently, cervical disc disease presents as referred pain in the upper thoracic and interscapular area.  Similarly, some tumors of the cervical medulla have also presented as interscapular pain.4 Some have speculated direct involvement and actual entrapment of the posterior rami of T2 to T6 spinal nerves. However, symptoms from the cervical area are referred directly to the infrascapular back. Degenerative vertebral and disc changes corresponding to the affected dermatome may be observed in some cases. 

Recent literature supports a role for radiographic imagine of cervical and thoracic spine to exclude disc disease and possible nerve compromise. With recent advances in radiography and availability of magnetic resonance imaging (MRI), earlier detection and intervention of cervical disc disease may be possible. Early recognition may promote timely intervention and treatment to prevent cervical spine disease progression. In addition to degenerative cervical discs, osteoarthritis, and cervical spine strain and muscle spasm, there may be a neoplasm or other pathology of the cervical spine contributing to notalgia paresthetica.

There is some thought that there may be a relation between NP and brachioradial pruritus as two types of localized pruritus syndromes. The recently described association of many cases of brachioradial pruritus( BRP) and cervical spine disease and description of the disease as a possible neuropathic/ neurogenic condition also support a probable neuropathic association of notalgia paresthetica.3,5,8,9,10 In contrast, NP is generally unilateral while BRP may involve the unilateral or less commonly bilateral upper extremities.

Topical therapies aimed at the back skin may be in fact be ineffectual or partially effective as basic emollients. Since NP does have periodic spontaneous remissions and exacerbations, it may be difficult to accurately measure response to various therapies. A placebo response may be considered with some therapies. The differential diagnosis in NP may include allergic or irritant contact dermatitis, fixed drug eruption, infection, neurodermatitis, dermatophytosis, neoplasm, lichen amyloid, arthropod reaction, lichen simplex chronicus, and other hypersensitivity reactions.

During the initial assessment of patients with NP, it is important to obtain a thorough past history of osteoarthritis, prior neck trauma, motor vehicle accident, vertebral fracture, cervical neoplasm or malignancy, or cervical disc disease. In the absence of positive medical history, radiographs or MRI of the cervical spine may aid in diagnosis and treatment.

For more generalized and chronic pruritus, full laboratory workup including complete blood count, chemistry panel including renal and liver functions, chest x-ray, and other studies may be warranted to exclude underlying physiologic causes of pruritus. Alternatively, proper management of NP may involve a multi-specialty cooperative effort of dermatology with radiology, orthopedic surgery, neurology, and possibly adjunctive fields including acupuncture, chiropractic, and physical therapy.

While to date there has been no uniformly effective treatment for NP, current therapeutic options for the localized itch syndromes include capsaicin cream11, eutectic mixture of local anesthetic (EMLA) cream, topical steroids, pramoxine cream, topical cooling or ice pack applications, oral steroids, Tiger balm, menthol creams, Cordran tape, intralesional corticosteroid injections, botulinum toxin injections,12 oral antihistamines, hydroxyzine, doxepin, topamax, anticonvulsant medications, carbamazepine (Tegretol) antidepressant medications, gabapentin (Neurontin), oxcarbazepine,13 topiramate, thalidomide,14 paravertebral local anesthetic block,cervical epidural injection, surgical resection of the rib,15 and many others. Some of the current systemic therapies may in fact exert their effect through the spinal nerves and central nervous system thereby supporting the neuropathic etiology of NP.3,5,16,17

In the future, first line therapy for notalgia paresthetica with associated cervical disease may include non-dermatologic, non-invasive treatments such as spinal manipulation, TENS Trancutaneous electrical nerve stimulation, physical therapy, cervical soft collars, massage, cervical traction, cervical muscle strengthening and increased range on motion, cervical discectomy with fusion, oral non-steroidal anti-inflammatory medications (ibuprofen, celecoxib,  ketoralac) and oral muscle relaxants (carisoprodal, cyclobenzapril, methocarbamol, metaxalone). Other medical measures for degenerative disc cervical disease and nerve impingement as introduced may also be considered.

Conclusions

Notalgia paresthetica may not be solely a skin disease per se but a cutaneous sign of an underlying degenerative cervical spine disease. The striking association of notalgia paresthetica with degenerative or traumatic cervico-thoracic spine disease suggests that early spinal nerve impingement may contribute to the pathogenesis of this skin symptoms of the disease. Additional studies are needed to further assess the relationship of NP with cervical spine disease. Whether this is a causal or coincidental finding remains to be determined in larger studies. While topical therapies may in some cases seemingly help decrease the localized symptoms in NP, systemic or broader scope spinal evaluation may be warranted to fully evaluate refractory cases. Cervical spinal imaging and treatment may be appropriate as primary or first line therapy in many cases of NP.

References 

1. Eisenberg E et al. Notalgia paresthetica associated with nerve root impingement
J Am Acad Dermatol. 1997; 37: 998-1000.

2. Misery L. What is notalgia paresthetica? Dermatology. 2002; 204:86-87.

3. Goodkin R, Wingard E, Bernhard JD. Brachioradial pruritus: Cervical spine disease and neurogenic/neurogenic pruritus. J Am Acad of Dermatol. 2003; 48: 521-524.

4. Bernard PA, Wayne ME. Notalgia paresthetica. Neurology.1978 ; 28: 1310-.

5. Springall DR, Karanth SS, Kirkham N, Darley CR, Polak JM. Symptoms of notalgia paresthetica may be explained by increased dermal innervation. J Invest Dermatol.1991 ;97: 555–561.

6. Weber PJ, Poulos EG. Notalgia paresthetica. Case reports and histologic appraisal
J Am Acad of Dermatol.1988;

7. Savk E, Savk O. Investigation of spinal pathology in notalgia paresthetica.  J Am Acad Dermatol, ;52:1085–1087.

8. Savk E, Savk O, Bolukbasi Culhaci N, et al. Notalgia paresthetica: a study on pathogenesis. Int J Dermatol.2000; 39: 754-759.

9. Pleet AB, Massey EW. Notalgia paresthetica. Neurology.1978; 28:1310-1312.

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,Eagelstein WH.Brachioradial pruritus treatment with capsacin. J Am Acad Dermatolog.193:29:783-784.

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14.  Savk E, Bolukbasib O, Akyolb A, Karamana G. Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica. J Am Acad Dermatol; 2001; 45: 630-632.

15. Pereira J. Brachioradial pruritus treated with thalidomide. An Bras Dermatol. 2005;80 (3):295-6.

16. Goulden V, Toomey PJ, Highet AS. Successful treatment of notalgia paresthetica with a paravertebral local anesthetic block. J Am Acad of Dermatol. 1998;38:114-116.

17. Hruza GJ, The Cutting Edge, Arch Dermatol. 2007;143(8):1062.

 

Figures

Figure 1: photograph of patient’s back showing mild hyperpigmentation of the right infrascapular back skin.

Figure 2: MRI of cervical spine demonstrating osteophytes and mild disc protrusions at C4-C5-C6.

   

Picture of atypical Notalgia and Brachioradial pruritus

MRI showing disc bulges and osteophytes in neck of Notalgia


Author : Dr. Nili Alai

Call to Schedule an appointment at (949) 582-SKIN 

Dr. Gary Coleand Dr. Nili Alai are Board-Certified Dermatologists.
For more information, please call (949) 582-7699 or visit the practice website at
www.lagunaskincenter.com.

 

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Synonyms, Key Words, and Related Terms Notalgia paresthetica

Notalgia paresthetica, NP, Notalgia, sensory disturbance of the back, unilateral itching of back, interscapular itching, skin dysesthesia of back, localized pruritus syndrome, sensory neuropathic syndrome, hyperalgesia, unilateral back itching, nostalgia, nostalgia paresthtica, notalgia paresthetic, what is notalgia, nostalgia paresthetica, photos of notalgia, pictures of notalgia parsthetica, best treatment for notlagia, Specialist for notalgia, back itching, bra allergy, back rash, TENS, Transcutaneous electrical nerve stimulator, neck muscle spasm, paresthetica-notalgia, pareaesthetica,


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