Notalgia Paresthetica for Physicians

The Skin Center

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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.

10. Savk E, Savk O.On brachioradial pruritus and notalgia paresthetica. J Am Acad Dermatol. 2003; 48: 521-524.

11.Goodless DR

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

12. Talt CP, Grigg E, Quirck CJ.Brachioradial pruritus and cervical spine manipulation. Australas J Dermatol.1998;39:168-170.

13. Weinfeld P. Successful Treatment of Notalgia Paresthetica With Botulinum Toxin Type A. Arch Dermatol. 2007; 143(8):980-982.

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.
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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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