Teleangiektasie macularis eruptiva perstans
Last Updated: 2019-08-26
Weber and Hellenschmied (1930)
- Urticaria pigmentosa adultorum
- Teleangiectasia eruptiva perstans
It is a form of cutaneous mastocytosis that occurs due to a congenital stem cell disease with mutation in the KIT gene. The multiple telangiectasias mainly affect the stem, which, along with other effluorescences, are caused by mast cell enrichment.
- Very rare
- Almost exclusively adults affected
Many patients have an activating mutation of the KIT gene to D186V. However, neither the expression of KIT (CD117) on the cell surface nor the mutation itself is specific for mastocytosis. The disease shows an increased number of mastocytes in the skin due to clonal stem cell disease. A systemic manifestation is discussed.
- Multiple, asymptomatic, flat, brownish and often teleangiectatic maculae
- Primarily the trunk is affected
- Usually itching (can also be the sole symptom) and urticarial dermographism near the skin change itself
- Often there is an aspirin or codeine intolerance in these patients, but this is an ideosyncratic reaction, which is not mediated by IgE and can therefore rarely cause a shock reaction.
Usually, a chronic course is observed, spontaneous healing is rare.
- often discreet occurrence of the disease in the clinic
- older skin changes can be yellow-brownish in appearance
- a possible systemic manifestation has not yet been conclusively clarified
- In the case of affected children, think of a familial form of illness, since the Onset already occurs in childhood.
- Darier character positive
Predominantly the trunk is affected
- Mention of aspirin or coidein intolerance
- ask about the duration of the effluences, as they are often ignored by the patient at first, since they are considered harmless
The disease is usually chronic, spontaneous healing is rarely observed.
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