Epidermolysis bullosa, dystrofisk

Også kjent som: Dystrofisk epidermolysis b
Engelsk navn: Dystrophic epidermolysis bullosa
Engelske synonym: DEB,Dermolytic epidermolysis bullosa,Epidermolysis bullosa dystrophica


A group of inherited epidermolysis bullosa (EB) characterized by cutaneous and mucosal fragility resulting in blisters and superficial ulcerations that develop below the lamina densa of the cutaneous basement membrane and that heal with significant scarring and milia formation. Dystrophic epidermolysis bullosa (DEB) comprises four major and several rare sub-types with the three most common being intermediate dominant DEB, severe recessive DEB and intermediate recessive DEB.

Fra Orphanet

ORPHA: 303
Klassifiseringsnivå: Group of disorders
ICD-10: Q81.2

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