016 Brunsting-Perry pemphigoid after remission of previous bullous pemphigoid related to epitope spreading from BP180 to BP230

Abstract

Brunsting–Perry pemphigoid is a localized form of cicatricial pemphigoid. Here, we report a case of Brunsting–Perry pemphigoid after remission of previous bullous pemphigoid. A 63-year-old Chinese male presented to our department with a three-month history of pruritic lesions in August 2013. Tense vesicles and bullae appeared on his hands, upper limbs and trunk. The skin biopsy showed subepidermal bulla with eosinophil infiltrations in upper dermis. The enzyme-linked immunosorbent assay (ELISA) for IgG antibodies against the BP180 NC16A domain was 90.44 and for the BP230 was 33.75. The patient was diagnosed as BP and treated with prednisone 30mg per day. The lesions improved quickly. Then the dose of prednisone was tapered down and kept at 5mg daily. Four years later, he revisited our department with a seven-month history of head lesions. Clinical examination revealed crusts, and trophic scars on the parietal scalp region. IIF using 1 mol L-1 NaCl-split healthy human skin sections revealed that the anti-basement membrane zone (BMZ) antibodies bound to the roof of the split. The ELISA index for the BP180 NC16A was 25.45 and for the BP230 was 110.82. From these findings, we diagnosed this patient as Brunsting–Perry type pemphigoid. He received oral prednisone (10 mg/d) but the improvement was limited. He later had the crusts debrided and 0.1mg mL-1 dexamethasone wet packing. The lesions healed gradually. The patient continuously had oral prednisone 10 mg daily in the following 9 months and was in stable condition. In this case, we observed a rare transition to Brunsting–Perry type pemphigoid from previous BP. As far as we know, it is firstly reported. And the dominant IgG anti-BMZ antibody switched from anti-BP180 NC16A to anti-BP230. But the mechanism still need further study.