Pemphigus vulgaris with circulating anti-desmoglein 3 and anti-BP180 antibodies: a case report and brief review of cases with coexistence of pemphigus vulgaris and bullous pemphigoid.

ABSTRACT

Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are two autoimmune blistering diseases involving the skin and the mucous membranes characterized by circulating autoantibodies directed against desmosomal cadherins or antigens expressed in the basement membrane zone, respectively. The simultaneous presence of clinical and/or immunopathological features of PV and BP in the same patient has been reported in very few cases in the literature to date. Most of these cases had exclusive cutaneous involvement, while a minority showed concomitant oral lesions. We describe the case of a 59-year-old female patient with a 10-year history of refractory PV lesions limited to mucous membranes (conjunctiva, oral cavity and genital mucosa), which were controlled by the addition of mycophenolate sodium to oral prednisone. Immunofluorescence studies revealed findings consistent with PV, whereas enzyme-linked immunosorbent assay revealed circulating anti-BP180 antibodies in association with anti-desmoglein 3 antibodies. The significance and relevance of this finding are briefly discussed, in light of the literature data.