A Case of Severe Refractory Pemphigus Vulgaris in a Patient With Stable Esophageal Malignancy

Pemphigus is a broad term that is used to describe a group of bullous autoimmune diseases affecting the skin and mucous membranes; the pathogenesis involves autoantibodies directed against various cell junction desmosomal proteins. In patients with a history of malignancy who present with bullous lesions, the differential diagnosis may include, but is not limited to, paraneoplastic pemphigus (PNP) and pemphigus vulgaris (PV) secondary to a primary autoimmune process, or induced by chemotherapy or radiation therapy. In this report, we present a case of refractory PV in a patient with stable esophageal cancer, five years after undergoing radiation therapy. He was poorly responsive to corticosteroids and intravenous immunoglobulin (IVIG). PNP or PV in a patient with stable esophageal malignancy has not been previously reported. PNP and PV can have overlapping autoantigens [desmoglein types 1 and 3 (DSG1 and DSG3)] as well as similar presentations. Thus, distinguishing between the two may be challenging in a patient with a history of cancer. More research must be done to determine if PNP can be seen in a patient with stable esophageal malignancy and, similarly, if PV can be precipitated by stable esophageal malignancy. Such research would aid in determining whether or not similar presentations are more severe or refractory to standard treatment regimens, thereby contributing to improve treatment strategies.