Background: We describe a new variant of inherited epidermolysis bullo
sa and elucidate the clinical, histologic, and ultrastructural feature
s of this condition. Observations: This form of epidermolysis bullosa
displays an autosomal dominant inheritance pattern, is characterized b
y acral bullae, and histologically demonstrates suprabasal clefting wi
th acantholysis. Ultrastructural findings are nonspecific but reminisc
ent of those observed in benign familial pemphigus. Conclusion: Acanth
olytic epidermolysis bullosa is a rare but distinct clinicopathologic
entity that warrants inclusion in the nosologic classification of epid
ermolysis bullosa.