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Definition

A group of autosomal dominant blistering skin diseases characterized by tissue separation which occurs below the dermal-epidermal basement membrane at the level of the anchoring fibrils. Various clinical types with different severity are recognized, ranging from severe mutilating forms to mild forms with limited and localized scarring, and less frequent extracutaneous manifestations.

Acronym

DDEB

Synonyms

Albopapuloid dominant dystrophic epidermolysis bullosa
Autosomal dominant dystrophic epidermolysis bullosa
EBDCT
EBDD
Epidermolysis bullosa dystrophica, Cockayne-Touraine type
Epidermolysis bullosa dystrophica, Pasini type

Keywords

Epidermolysis bullosa

 
Disclaimer

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