recessive epidermolysis bullosa dystrophica

recessive epidermolysis bullosa dystrophica
recessive dystrophic epidermolysis bullosa the autosomal recessive forms of epidermolysis bullosa dystrophica; these tend to be more severe than the dominant forms. Most are characterized by extensive denuded hemorrhagic erosions and blisters on all body surfaces (including mucous membranes) at birth or in early infancy. Lesions in the esophagus heal to leave esophageal strictures that can impair feeding; other healed lesions can restrict mobility by causing syndactyly and sometimes flexion contractures. Called also Hallopeau-Siemens syndrome.

Medical dictionary. 2011.

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