Urticaria pigmentosa
Classification according to ICD-10 | |
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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
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
- RJ Vasani, SV Medhekar: Urticaria pigmentosa. In: Indian dermatology online journal , Volume 6, No. 6, 2015 Nov-Dec, pp. 464-465, doi: 10.4103 / 2229-5178.169727 , PMID 26752589 , PMC 4693376 (free full text).
Web links
Individual evidence
- ↑ a b Maculopapular cutaneous mastocytosis. In: Orphanet (Rare Disease Database).
- ↑ a b c d Encyclopedia Dermatology
- ^ E. Nettleship: Rare forms of urticaria. In: British Medical Journal , London 1869, Volume 2, pp. 323-324.
- ^ E. Nettleship: Chronic urticaria leaving brown stains: nearly two years' duration. In: British Medical Journal , London 1869, Volume 2, A 435
- ↑ Mast cell disease. In: Online Mendelian Inheritance in Man . (English)
- ^ Urticaria pigmentosa, nodular. In: Orphanet (Rare Disease Database).
- ↑ Urticaria pigmentosa, plaque-shaped. In: Orphanet (Rare Disease Database).
- ↑ Urticaria pgimentosa, typical. In: Orphanet (Rare Disease Database).
- ↑ 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 .