Multiple oral squamous cell carcinomas associated with autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy

Abstract

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder characterized by chronic mucocutaneous candidiasis, hypoparathyroidism, and adrenal insufficiency. Chronic oral candidiasis is frequently the first manifestation of the condition. There is an increased incidence of oral squamous cell carcinomas at an early age in this population, and it is possible that chronic oral candidal infection has a role in oral carcinogenesis in patients with APECED. We present a case of multiple oral squamous cell carcinomas in a 35-year-old woman with chronic mucocutaneous candidiasis as a component of APECED. Our patient has had 3 confirmed oral squamous cell carcinomas to date, which have been managed with laser resection and ablation. She remains on systemic antifungal therapy and is under regular surveillance in a multidisciplinary head and neck clinic.