Congenital Epidermolysis Bullosa Acquisita

A first description of mother-to-infant transfer of this autoimmune disorder

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disorder in which autoantibodies against the 145-kDa NC1 domain of type VII collagen result in a decrease in the anchoring fibrils of the lamina densa. Typically presenting in adulthood, EBA is characterized by chronic skin fragility, with blistering of the skin and mucosa, scarring, milia, and dyspigmentation. EBA has rarely been reported in children; authors now present the first report of congenital EBA.

A 32-year-old woman (gravida 4, para 3) with EBA delivered at 36 weeks' gestation after labor was induced because of a deficiency of amniotic fluid and intrauterine growth restriction. The newborn's Apgar scores were 9 at both 1 and 5 minutes; however, tense blisters an…

Reviewing Author

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre