Epidermolysis bullosa acquisita in a 6-year-old Japanese boy

A 6-year-old Japanese boy started to develop blisters around the mouth and eyes in May 1996. With provisional diagnoses of herpes simplex or Kaposi's varicelliform eruption, he was treated with acyclovir and immune globulin. Blister formation increased, however, and he was referred to the Dermatology Clinic of Kobe University School of Medicine in August 1996. On physical examination, tense bullae and vesicles were observed on the trunk and limbs and also on the palms and soles (Fig. 1). Neither mucosal involvement nor cutaneous fragility was observed, and no nail deformity was seen. The blistering on both erythematous bases and normal skin showed a 'string-of-pearls' arrangement. Abnormal laboratory results were as follows: white blood cell count, 12.4x10³/mm³ (N = 4-8.5) (eosinophils, 25%); platelets, 514x10³/mm³ (N = 130-300); total protein, 4.9 g/dL.