Childhood epidermolysis bullosa acquisita

ABSTRACT

We describe a case if a 4 year-old girl who consulted our institution because of multiple tense vesicles and bullae on the perioral area, trunk, elbows, knees and dorsum of hands and feet. Biopsy of the lesion revealed subepidermal cleft with few ill-defined acantholytic cells and patchy areas of vacuolar degeneration showing infiltration with numerous neutophils and occasional eosinorphils. There was linear deposition of C3, IgG and IgA on basement membrane zone. The diagnosis was epidemolysis bullosa acquisita, IgA bullous dermatosis-like presentation. The patient was treated with topical corticosteroid for 1 1/2 months with complete resolution of lesions and appearance of milia on the perioral area and dorsum of hands and feet. Patient remained in remission.