Paraneoplastic pemphigus: a case report and review of literature

Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease frequently associated with lymphoproliferative disorders. The rare combination of the disease with other malignancies such as different types of carcinomas, sarcomas, melanoma and skin tumours has also been reported. Most patients develop very severe oral ulceration and conjunctival ulceration with or without genital ulceration resembling the features of Steven's Johnson's syndrome or most severe forms of drug eruptions. The possibility of PNP should be borne in mind when a patient presents with extensive oral ulceration if clinical, histopathological and results of direct immunofluorescence are not pathognomonic for a specific diagnosis. The issue becomes even more important as some patients with PNP have no diagnosed malignancy at the time of presentation. We document a case of PNP in a 29-year-old female who suffers from non-Hodgkin's lymphoma.