HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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.
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EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
V.a. occurring in adulthood. Occurrence in childhood is very rare and affects about 1% of childhood mastocytosis cases.
LocalizationThis section has been translated automatically.
Preference for the trunk, also extremities, face is left out.
Clinical featuresThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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!
TherapyThis section has been translated automatically.
Symptomatic; cosmetic treatment of individual telangiectasias with laser (argon, pulsed dye laser). Otherwise see below maculopapular cutaneous mastocytosis.
Progression/forecastThis section has been translated automatically.
Chronic course, no regression tendencies
Note(s)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Arber DA et al (2016) The 2016 revision to the World Health Organization classification of myeloidneoplasms
and acute leukemia. Blood 127:2391-405.
- Kalayciyan AK, Kotogyan A (2001) Telangiectasia macularis eruptiva perstans. J Eur Acad Dermatol Venereol 15: 263-264
- Parkes Weber F, Hellenschmied R (1930) Telangiectasia macularis eruptiva perstans. Br J Dermatol 42: 374-382
- 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
- Suzuki K et al (2002) Telangiectasia macularis eruptiva perstans in polycythemia rubra vera. Eur J Dermatol 12: 201-203
Incoming links (6)Cutaneous mastocytosis; Cutaneous mastocytosis of adulthood; Teleangiectasia; Teleangiectasia; Teleangiectasia eruptiva perstans; Unilateral naevoid telangiectasia syndrome;
Outgoing links (4)Darian sign; Laser; Maculopapular cutaneous mastocytosis; Mastocytosis (overview);
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.