Teleangiektasie macularis eruptiva perstans
Last Updated: 2019-08-26
ICD11: -
Last Updated: 2019-08-26
Author(s): Anzengruber F., Navarini A.
ICD11: -
Weber and Hellenschmied (1930)
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.
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.
Usually, a chronic course is observed, spontaneous healing is rare.
Predominantly the trunk is affected
The disease is usually chronic, spontaneous healing is rarely observed.
external:
cooling lotions if necessary with Polidocanol 5% R200
Irradiation: in certain patients an improvement occurs under medium to high dose UVA1 irradiation or under PUVA therapy.
internal: combination of non-sedating H1 antagonists e.g. levocetirizine Xyzal®) once/day 5mg p.o. or of a sedating H1 antagonist such as diemetinden (fenistil) 3 times/day 1-2mg p.o. with an H2 antagonist such as cimetidine (e.g. tagamet)
Mast cell stabilizers such as ketotifen (e.g. Zaditen Kps./Sirus) 2 times/day 1-2mg p.o.
In individual cases improvement under disodium chromoglyceric acid e.g. Colimune) 4 times/day 100-200 mg achieved
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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.
Unterstell N1, Lavorato FG, Nery NS, Mann D, Alves Mde F, Barcauí C. An Bras Dermatol. 2013 Jul-Aug;88(4):643-5. doi: 10.1590/abd1806-4841.20132053.Dermatoscopic findings in telangiectasia macularis eruptiva perstans.
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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