Paraneoplastic pemphigus: Diagnosing with a modified criteria – A case report

Abstract

Paraneoplastic pemphigus is a rare autoimmune blistering disorder which presents with polymorphous cutaneous lesions and recalcitrant stomatitis. The disorder has a 100% association with an underlying neoplasia, primarily lymphoproliferative disorders. We report a patient who presented with oral mucosal lesions resistant to treatment with bullous cutaneous lesion and an underlying NHL-B cell lymphoma. The condition since its inception has been diagnosed using the criteria by Anhalt et al, which still is the most standard one. The criterion has been revised and updated every now and then. A current update has been proposed by Svoboda SA et al, which is a more relaxed one making diagnosis easier in a patient of Paraneoplastic pemphigus. Our patient was confidently diagnosed with Paraneoplastic pemphigus using this criterion. A quick diagnosis with fewer investigations allowed early and aggressive institution of treatment, to which patient responded well.