Barts syndrome




Subtype of dominant dystrophic epidermol-ysis bullosa with congenital localized absence of skin, nail abnormalities, and blistering


Mutation of the COLA7A1 gene, resulting in the production of poorly formed anchoring fibrils at the skin's basement membrane zone

Clinical manifestation

Congenital erosions of the lower extremities, which heal with hairless scars; trauma-induced blistering; absent or dystrophic nails; mucous membrane erosions only in early life

Differential diagnosis

Aplasia cutis congenita; epidermolysis bul-losa simplex; junctional epidermolysis bul-losa; child abuse


Hydrocolloid dressings to erosions; petrolatum between toes to minimize scarring


Amichai B, Metzker A (1994) Bart's syndrome. International Journal of Dermatology 33(3)1161-163

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