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