Preferred Label : Recessive dystrophic epidermolysis bullosa, generalised severe;
ICD-11 definition : Severe generalized recessive dystrophic epidermolysis bullosa is the most severe subtype
of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens
type. It is characterized by generalized 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.;
ICD-11 synonym : RDEB-gen sev; RDEB-GS - [recessive dystrophic epidermolysis bullosa, generalised severe]; RDEB-gen sev - [recessive dystrophic epidermolysis bullosa, generalised severe]; RDEB Hallopeau-Siemens;
ICD-11 acronym : RDEB-GS;
Origin ID : 365362994;
Currated CISMeF NLP mapping
Severe generalized recessive dystrophic epidermolysis bullosa is the most severe subtype
of dystrophic epidermolysis bullosa. It was formerly known as the Hallopeau-Siemens
type. It is characterized by generalized 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.
