Lichenoid skin lesions & mucosal erosions as a paraneoplastic syndrome

132 Fig. 1. Violaceous, mildly scaly, discrete to confluent papules and plaque over the back. Direct immunofluorescence (IF) of perilesional skin showed cell surface and basement membrane zone deposits of IgG (Fig. 2a). Intercellular IgG deposits were also demonstrated by indirect IF using normal human skin section (1:40; not in Fig. 2b) and rat bladder transitional epithelium (1:10) (Fig. 2b). On immunoblotting, patient’s serum reacted with the 210 kDa envoplakin and 190 kDa periplakin. A diagnosis of paraneoplastic pemphigus (PNP) was made and the patient was treated with oral corticosteroids and intravenous rituximab but had poor response to therapy. The patient died of uncontrolled disease three months after the initial presentation.