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Дифузний шкірний мастоцитоз

ORPHA:79456· ICD-10 Q82.2· Diffuse cutaneous mastocytosis

Визначення(English summary)

Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM; see this term) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM; see this term) and one with infiltrations (Pseudoxanthomatous DCM; see this term).

Поширеність
<1 / 1 000 000
Успадкування
Not applicable
Вік початку
Infancy, Neonatal