Urticaria pigmentosa

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Classification according to ICD-10
Q82.2 Mastocytosis (congenital) - urticaria pigmentosa
ICD-10 online (WHO version 2019)

The Urticaria pigmentosa is a very rare congenital disease with the main feature of urticaria due to mastocytosis skin.

Synonyms are: Nettleship's disease; Nettleship Syndrome; Mastocytosis syndrome; cutaneous mastocytosis; Brown man; Mast cell reticulosis, Rywlin's disease, generalized mastocytosis; English Maculopapular cutaneous mastocytosis

Back of a child with urticaria pigmentosa

The name refers to the author of the first description from 1869 by Edward Nettleship .

The disease should not be confused with Nettleship Falls Syndrome , an ocular albinism.

distribution

Frequency and mode of inheritance are unknown.

root cause

The disease are mutations in KIT - gene on chromosome 4 locus q12 based.

Classification

The following subdivisions are common:

Systematic:

  • Nodular shape
  • Plaque shape
  • Typical shape

Clinical:

  • Hemorrhagic form (Urticaria pigmentosa haemorrhagica)
  • Exanthematic form (urticaria pigmentosa, exanthematic)
  • Bullous form (Urticaria pigmentosa bullosa)
  • Xanthelasmoid form (Urticaria pigmentosa xanthelasmoidea)

Clinical manifestations

Clinical criteria are:

  • First manifestation often already in the first 2 years of life, then more frequently between the 4th and 6th decades of life
  • Localization mainly on the trunk
  • Flat gray-brown spots with Darier's signs , often elevatus dermographism

Differential diagnosis

A systemic form of mastocytosis must be distinguished; also a malignant lymphoma or a dermatofibroma .

therapy

In addition to avoiding triggers such as u. a. Non-steroidal anti-inflammatory drugs , acetylsalicylic acid , codeine , procaine , polymyxin B , mechanical irritation or abrupt temperature changes, cooling gels or antihistamine-containing gels as well as PUVA are available.

Prospect of healing

The prognosis is favorable and most of those affected have a normal life expectancy. If manifest in childhood, spontaneous remission occurs in a good half .

literature

Web links

Individual evidence

  1. ↑ a b Maculopapular cutaneous mastocytosis. In: Orphanet (Rare Disease Database).
  2. ↑ a b c d Encyclopedia Dermatology
  3. ^ E. Nettleship: Rare forms of urticaria. In: British Medical Journal , London 1869, Volume 2, pp. 323-324.
  4. ^ E. Nettleship: Chronic urticaria leaving brown stains: nearly two years' duration. In: British Medical Journal , London 1869, Volume 2, A 435
  5. ↑ Mast cell disease.  In: Online Mendelian Inheritance in Man . (English)
  6. ^ Urticaria pigmentosa, nodular. In: Orphanet (Rare Disease Database).
  7. ↑ Urticaria pigmentosa, plaque-shaped. In: Orphanet (Rare Disease Database).
  8. ↑ Urticaria pgimentosa, typical. In: Orphanet (Rare Disease Database).
  9. ↑ A. Heinze, TJ Kuemmet, Ye Chiu, SS Galbraith: Longitudinal Study of Pediatric urticaria pigmentosa. In: Pediatric dermatology. [Electronic publication before going to press] January 2017, doi: 10.1111 / pde.13066 , PMID 28133781 .