Catalog

Recessive dystrophic epidermolysis bullosa, generalised severe

No MMS code

Link to WHO Definition

Recessive dystrophic epidermolysis bullosa, generalised severe – http://id.who.int/icd/entity/365362994

Severe generalised recessive dystrophic epidermolysis bullosa is the most severe subtype of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens type. It is characterised by generalised cutaneous and mucosal blistering and scarring associated with severe deformities and major extracutaneous involvement. Affected individuals are at high risk of aggressive squamous cell carcinomas in areas of chronic ulceration.