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.
- Teleangiectasias can be treated with lasers (argon or pulsed dye lasers)
cooling lotions if necessary with Polidocanol 5% R200
- Alternative: Antihistaminic gels (Fenestil Gel, Tavegil Gel, Solventol Gel)
- Glucocorticoid creams e.g. 0.5% hydrocortisone cream R120 cannot be used in the long term and should therefore not be used.
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
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.