Barts syndrome

Synonym(s)

None

Definition

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

Pathogenesis

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

Therapy

Hydrocolloid dressings to erosions; petrolatum between toes to minimize scarring

References

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

How To Reduce Acne Scarring

How To Reduce Acne Scarring

Acne is a name that is famous in its own right, but for all of the wrong reasons. Most teenagers know, and dread, the very word, as it so prevalently wrecks havoc on their faces throughout their adolescent years.

Get My Free Ebook


Post a comment