Two Cases of Epidermolysis Bullosa Acquisita with Atypical Distribution of Eruptions / 대한피부과학회지

ABSTRACT

We herein report two patients with epidermolysis bullosa acquisita(EBA), who had showed the atypical clinical features. A 25-year-old male presented with multiple pruritic vesicles, erosion and crusts which occurred more severely on the face than any other sites of the skin and healed with atrophic scar formation. Histopathologically, there was a subepidermal neutrophilic blister with moderate perivascular lymphohistiocytic infiltrates in the dermis, and the diagnosis of EBA was confirmed by means of direct immunofluorescence and salt-split direct immunofluorescence test performed on the perilesional skin. The other patient was a 24-year-old female who had had multiple painful ulcerative lesions on the oral mucosa for 4 months. After then, vesicles and bullae developed to progressively generalize to the anterior neck, chest and lower leg. Histolopathologic examination of peribullous skin showed a subepidermal bulla with neutrophils and eosinophils. The salt-split skin direct immunofluence test showed IgG and IgM binding to the dermal side only. We diagnosed this patient as nonscarring inflammatory EBA. Both patients were treated with prednisolone, colchicine, and dapsone resulting in clinical improvement, but their skin lesions recurred several months after discontinuing medication.