H number H00585
Name Epidermolysis bullosa, hemidesmosomal, including:
Epidermolysis bullosa simplex with pyloric atresia (EBS-PA);
Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD);
Epidermolysis bullosa simplex, Ogna type (EBS-Ogna)
Description Inherited epidermolysis bullosa is a diverse group of disorders characterized by mechanically fragile skin that readily blister. The conditions in which the blister formation occurs at the level of hemidesmosome are classified as hemidesmosomal variants, that is subtype of epidermolysis bullosa simplex.
Category Skin and connective tissue disease