Epidermolysis bullosa simplex with transient erythema circinatum

A 4-week-old male infant who developed blisters on the face, hands and buttocks immediately after birth was diagnosed as suffering from generalized epidermolysis bullosa (EB) simplex (Köbner). At 6 months of age, centrifugally expanding erythema circinatum suddenly appeared on the trunk and limbs. Blood tests showed transient elevation of liver enzymes. An intercurrent illness such as a bacterial or viral infection, or an adverse reaction to a drug was suspected, but no supportive evidence was obtained. The circinate or arciform pattern of the eruption, and the ultrastructural findings of basal cell cytolysis and some tonofilament aggregation suggested a possible diagnosis of epidermolysis bullosa herpetiformis (Dowling-Meara) [DM-EB]. However, the predominant feature of this child's dermatosis was erythema circinatum, with a small number of blisters limited to the areas affected by erythema. The erythema resolved spontaneously after 8 months. Round or whisk-like clumping of tonofilaments, a typical finding in DM-EB, was not demonstrated on electron microscopy. These observations were not consistent with classical DM-EB, and we consider that this case is an unusual form of EB simplex showing transient erythema circinatum.