171 Paraneoplastic pemphigus: Clinical, pathologic, and immunopathologic features in a series of twelve Bulgarian patients

Paraneoplastic pemphigus (PNP) also called Autoimmune Multiorgan Syndrome (PAMS), is a rare but severe clinical variant of with high mortality rate. It clinically characterized by polymorphic muco-cutaneous and polyorgan involvement in association malignancies mostly lymphoproliferative origin. The diagnosis PNP/PAMS requires specific clinical, histological immunological criteria. In Bulgaria the most frequent autoimmune blistering disease, showing incidence comparable to reported studies worldwide. However, no data are available on characteristics our country. With this case series we try determine its presentations among Bulgarian population. Patients diagnosed referral center Sofia, from 2002 2022 were retrospectively included. histologic, immunopathologic results analyzed. Twelve patients (4 men, 8 women) an average age 60 years identified. Associated included chronic lympholeucosis (CLL), thymoma, or myeloleucosis (CML). Severe mucositis cutaneous lesions, including erythema multiforme-like palmo-plantar changes observed. Indirect immunofluorescence rat urinary bladder was positive 10 patients, envoplakin ELISA immunoblot tested 5 4 respectively. Despite intensive therapy, expired. highly associated CLL thymoma. Immunosuppressive agents often required decrease treating underlying malignancy chemotherapy may control autoantibody production. prognosis remains poor disease fatal.