Teleangiectasia macularis eruptiva perstans
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
ICD11: 2A21.1Y
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
ICD11: 2A21.1Y
Weber and Hellenschmied (1930)
It is a form of cutaneous mastocytosis that occurs due to a congenital stem cell disease with a mutation in the KIT gene. It is characterised by multiple telangiectasias predominantly on the trunk, which are caused by mast cell accumulation along with other effluorescences.
>Many patients have an activating mutation of the KIT gene on 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 the clonal stem cell disease. A systemic manifestation is being discussed.
Mainly the trunk is affected
The disease is usually chronic, and spontaneous healing is rarely observed.
external:
Radiation: certain patients improve with medium to high doses of UVA1 irradiation or PUVA therapy
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Chang A1, Tung RC, Schlesinger T, Bergfeld WF, Dijkstra J, Kahn TA. Pediatr Dermatol. 2001 Jul-Aug;18(4):271-6. Familial cutaneous mastocytosis.
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Severino M1, Chandesris MO2, Barete S3, Tournier E4, Sans B5, Laurent C4, Apoil PA6, Lamant L4, Mailhol C7, Laroche M8, Fraitag S9, Hanssens K10, Dubreuil P10, Hermine O2, Paul C11, Bulai Livideanu C12.J Am Acad Dermatol. 2016 May;74(5):885-91.e1. doi: 10.1016/j.jaad.2015.10.050. Epub 2016 Feb 19.Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement.
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