Autoimmune Polyendocrine Syndrome Masquerading as Mucocutaneous Candidiasis: A Rare Disorder

Autoimmune polyendocrine syndrome type 1 (APS-1) is a rare autosomal recessive genetic disorder. It is caused by a loss of immune tolerance due to a mutation in the autoimmune regulator gene. Its systemic features include the presence of autoimmunity against multiple endocrine organs, and its cutaneous features include the presence of chronic candidiasis of the mucosa, nails, and skin. We report a case of an 8-year-old male presenting with progressive blackening of the nails, recurrent oral candidiasis, and acute paronychia. Clinical, laboratory, and genetic evaluations confirmed APS-1. The patient was managed with itraconazole, supportive therapy, and an antifungal mouthwash. Early recognition and a multidisciplinary approach are crucial in managing APS-1.