Teleangiectasia macularis eruptiva perstans
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
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 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.>
- Very rare
- Almost exclusively adults affected
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.
- multiple, asymptomatic, flat, brownish and often teleangiectatic macules
- primarily the trunk is affected
- mostly pruritus (may also be the sole symptom) and urticarial dermographism near the skin lesion itself
- often there is aspirin or codeine intolerance in these patients, but this is an ideosyncratic reaction which is not mediated by IgE and thus rarely causes a shock reaction
- mostly shows a chronic course, spontaneous healing is rare
- often discrete appearance of the disease in the clinic
- older skin lesions may have a yellow-brownish appearance
- a possible systemic manifestation has not yet been conclusively clarified
- think of a familial form of the disease in affected children, since in such cases the onset already occurs in childhood.
- Darier's sign positive
Mainly the trunk is affected
- Mention of aspirin or coidein intolerance
- ask about duration of effluorescences, as they are often ignored by the patient initially as they are considered harmless
The disease is usually chronic, and spontaneous healing is rarely observed.
Watkins CE1, Bokor WB1, Leicht S2, Youngberg G3, Krishnaswamy G4 Dermatol Reports. 2011 Jul 29;3(1):e12. doi: 10.4081/dr.2011.e12. eCollection 2011. Telangiectasia macularis eruptiva perstans: more than skin deep.
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.