Document Type : Case Report


Cutaneous Leishmaniasis Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran


Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant hereditary syndrome caused by mutations of the MEN1 tumor suppressor gene. We describe a patient with a prior confirmed diagnosis of pemphigus vulgaris (PV), who presented with new cutaneous manifestations that led to the diagnosis of MEN1. A man in his early forties with a history of PV from 11 years ago presented with some cutaneous lesions six months ago, diagnosed as angiofibroma and collagenoma. Moreover, he suffered from recurrent renal stones and gout for several years. Laboratory analysis showed hypercalcemia, which led us to confirm the diagnosis of a parathyroid adenoma by sestamibi scintigraphy. Contrast-enhanced computed tomography (CT) of the abdomen revealed hyper-enhancing pancreatic lesions while the patient had no related symptoms. Thus, the clinical diagnosis of MEN1 syndrome was settled, and the patient underwent surgical and medical management. A hitherto unreported cooccurrence between MEN-1, as a hereditary syndrome, and PV, as an autoimmune bullous cutaneous dermatosis, opens a hazy challenging issue: whether MEN-1 has any association with autoimmune bullous cutaneous diseases like PV or increases the incidence of such conditions.