A CASE OF AUTOIMMUNE BULLOUS DERMATOSIS WITH FEATURES OF PEMPHIGUS-VULGARIS AND BULLOUS PEMPHIGOID

Pleomorphic blisters, including tense bullae and annularly arranged ve sicles around the erythema as well as erosive eruptions in the oral ca vity, appeared on a 61-year-old woman 5 years after surgery for cholan giocellular carcinoma. A biopsy specimen from the oral cavity showed i ntraepidermal blisters, and those from skin lesions showed subepiderma l blisters with infiltrates of eosinophils and neutrophils. The early- stage vesicles showed infiltrates along the epidermal-dermal junction, where electron microscopy disclosed disruption of the lamina densa, b asal cells remaining on the dermis, and acantholytic keratinocytes amo ng the infiltrates, but there was no cleavage of the epidermal-dermal junction at the lamina lucida. Direct immunofluorescence studies showe d immune deposition at the intercellular space (ICS) and along the bas ement membrane zone (BMZ). Indirect immunofluorescence studies confirm ed coexistence of IgG class anti-ICS and anti-BMZ antibodies. Although this case showed immunohistochemical features of bullous pemphigoid, the presence of suprabasal cleavage in the oral mucosa, acantholytic c ells in the blister cavity, the deposition of IgG at the ICS of the pe rilesional epidermis, and circulating anti-ICS antibodies strongly sug gested that this case was primarily pemphigus. The strong inflammation along the epidermal-dermal junction due to unknown factors may have m odified the clinical appearance and the histopathology.