THE SKIN CENTER

NOTALGIA PARESTHETICA

At a Glance

• sensory neuropathic syndrome of the back skin
• may include localized burning, pain, tenderness, hyperalgesia, or dysesthesiason a small patch of the mid back
• associated with a poorly circumscribed tan or hyperpigmented patch
• chronic condition
• causes discomfort and nuisance to affected patients
• not life threatening
• common disorder which remains largely underdiagnosed
• no uniformly effective treatment available

Synonyms, Key Words, and Related Terms

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, notalgia paresthetic, what is notalgia, nostalgia paresthetica

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

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 Category: Corticosteroid, Topical (high Potency)

Drug Category: anti-pruritus

Drug Category: Analgesic, Topical

Drug Category: Corticosteroid, Topical (medium Potency)

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.

CASE REPORT

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

U.S. Board Certified Dermatologist

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.