Teleangiectasia macularis eruptiva perstans Q82.2

Clonal disease of the hematopoietic stem cell with the development of clinically heterogeneous clinical pictures characterized by mast cell accumulations in the skin and in internal organs.

In urticaria pigmentosa (cutaneous mastocytosis), the proliferation of mast cells is limited to the skin.

In systemic mastocytosis, at least 1 extracutaneous organ is affected.

Teleangiectasia eruptiva perstans is clinically characterized by the appearance of multiple telangiectasias rather than the characteristic pigmented patches/papules of maculopapular cutaneous mastocytosis.

Most patients have an activating point mutation of the KIT gene (KIT D816V). However, both the expression of KIT (CD117) on the cell surface and the mutation are not specific for mastocytosis.

V.a. occurring in adulthood. Occurrence in childhood is very rare and affects about 1% of childhood mastocytosis cases.

Preference for the trunk, but extremities also possible Face remains unaffected. The face can also be affected (see pictures).

Frequently discreet, less frequently massive clinical picture in which itching is the main symptom (see below Urticaria pigmentosa adultorum). In a symmetrical arrangement in disseminated sowing, 0.2-2.0 cm large, roundish, red or reddish-brown patches interspersed with telangiectasia, which acquire a urticarial character when rubbed vigorously(Darian's sign).

Older efflorescences can get a yellow-brown coloration.

One also sees clinical pictures in which the teleangiectatic aspect completely dominates.

In contrast to the juvenile form of Urticaria pigmentosa, the mast cell infiltrates in the dermis of the telangiectatic form are often only discrete. They can be completely overlooked in the HE section, so that the diagnosis is missed. Only in special staining (e.g. Giemsa; CD25) the mast cells can be visualized.

Cave! The biopsy specimen must not be squashed during collection, otherwise the mast cells lose their granules, which are important for staining!

Symptomatic; cosmetic treatment of individual telangiectasias with laser (argon, pulsed dye laser). Otherwise see below maculopapular cutaneous mastocytosis.

Chronic course, no regression tendencies

In the current classification of mastocytoses (see Arber DA et al. 2016) this variant of adult Urticaria pigmentosa is not mentioned. Therefore, its entity is disputed. From a dermatological-diagnostic point of view, however, knowledge of this, often only discrete cutaneous mastocytosis is necessary to be able to initiate the necessary diagnostic measures in a targeted manner.

1. Arber DA et al (2016) The 2016 revision to the World Health Organization classification of myeloidneoplasms
   and acute leukemia. Blood 127:2391-405.
2. Kalayciyan AK, Kotogyan A (2001) Telangiectasia macularis eruptiva perstans. J Eur Acad Dermatol Venereol 15: 263-264
3. Parkes Weber F, Hellenschmied R (1930) Telangiectasia macularis eruptiva perstans. Br J Dermatol 42: 374-382
4. Pascual JC et al (2003) Presentation of telangiectasia macularis eruptiva perstans as a long-standing solitary plaque associated with renal carcinoma. J Cutan Med Surg 7: 399-402
5. Suzuki K et al (2002) Telangiectasia macularis eruptiva perstans in polycythemia rubra vera. Eur J Dermatol 12: 201-203