Notalgia paraesthetica G58.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

hereditary localized pruritus; Peculiar spotty pigmentation; Puzzling posterior pigmented pruritic Patches

History
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Branch Vazaturov 1934

Definition
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Rare isolated mononeuropathy (neuropathic pruritus form) of unexplained etiology.

Etiopathogenesis
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Damage to the primary branches of rami posteriores of unknown cause. Discussed are mechanical irritations and damage due to traumatic or degenerative changes of the affected spinal segments. The pigmentation of the area is understood as a stimulus response to constant rubbing at this site.

Manifestation
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About 70% of those affected are women. The average age of the disease is 57 years (25-80 years).

Localization
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Located above or between the shoulder blades (segments C7-Th6).

Clinical features
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In studies with larger collectives, the symptoms of "localized pruritus of varying intensity" and a circumscribed, blurred, brown-grey patch of skin are described (probably caused reactively by rubbing and chafing of the affected areas). Furthermore, (less frequently) intermittent pain (30%), paraesthesia (28%) and hyperaesthesia (11%) are reported.

Radiologically, degenerative changes and/or nucleus pulposus hernias are found in the spinal column sections of the affected segments (most frequently Th4 and C7) in about 80% of patients.

Histology
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In a larger study histologically 71.4% showed single cell necroses in the epidermis, 74.3% showed epidermal hyperpigmentation and 80% showed dermal hyperpigmentation.

Amyloid deposits in the papillary body were found in 11.4% of patients. 5.7% of the patients had none of the histological features mentioned above.

The number of peripheral nerves is significantly reduced in lesional skin.

Differential diagnosis
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Therapy
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The best results are achieved with 0.025-0.05% capsaicin ointment (e.g. dolenone, capsamol) 3-5x/day over a treatment period of several months. Application of capsaicin is unpleasant due to burning, itching etc. in the first days (inform the patient!).

Improvement can also be achieved with local anaesthetic creams (e.g. EMLA cream).

Treatments with antipruriginous substances such as camphor or menthol (Pruricalm®) can be tried.

In other collectives, success could be achieved with anticonvulsants (e.g. gabapentin).

Progression/forecast
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Eminently chronic course.

Note(s)
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The term is derived from the Greek (noton=back - algie=pain).

Literature
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  1. Astvazaturov M (1934) On paresthetic neuralgia and a special form of it - Notalgia parästhetica. Dt Z Nervenheilkd 133: 188-196
  2. Cerroni L et al (1993) Notalgia paresthetica, posterior pigmented pruritic patch and macular amyloidosis. Three stages of a disease. dermatologist 44: 777-780
  3. Huesmann T et al (2012) Notalgia paraesthetica: a descriptive two-cohort study of 65 patients from Brazil and Germany. Acta Derm Venereol 92:535-540
  4. Inaloz HS et al (2002) Notalgia paresthetica with a significant increase in the number of intradermal nerves. J Dermatol 29: 739-743
  5. Layton AM et al (1991) Notalgia paraesthetica - report of three cases and their treatment. Clin Exp Dermatol 16: 197-198
  6. Poet E (1992) Treatment of notalgia paresthetica with capsaicin. Cutis 49: 335-336
  7. Metz M et al (2011) Treatment of notalgia paraesthetica with an 8% capsaicin patch. Br J Dermatol 165:1359-1361
  8. Savk O et al (2005) Investigation of spinal pathology in notalgia paresthetica. J Am Acad Dermatol 52:1085-1087
  9. Savk E et al (2002) Immunohistochemical findings in notalgia paresthetica. Dermatology 204: 88-93
  10. Savk E et al (2000) Notalgia paresthetica: a study on pathogenesis. Int J Dermatol 39: 754-759

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020