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Bullous diffuse cutaneous mastocytosis

ORPHA:280785· ICD-10 Q82.2

Definition

Bullous diffuse cutaneous mastocytosis (BDCM) is a form of diffuse cutaneous mastocytosis (DCM) characterized by generalized erythroderma and severe blistering associated with the accumulation of mast cells in the skin.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
Infancy, Neonatal