ABSTRACT

Observation:

Paraneoplastic pemphigus is a rare immunobullous disease which is associated with an underlying neoplasm. Herein, a case of paraneoplastic pemphigus induced by nerve sheath sarcoma as a rare underlying neoplasm is presented. Our patient had an unusual clinical course and his neoplasm developed 26 months after mucocutaneous disease. In addition to vesiculobullous, erosive and lichenoid lesions, the patient showed erythroderma, which is a very rare cutaneous manifestation of paraneoplastic pemphigus.