Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome presenting as unexplained chronic interstitial keratitis

Autoimmune polyglandular syndrome is a rare autosomal recessive polyendocrinopathy with variable combinations of mucocutaneous candidiasis, autoimmune destruction of endocrineglands, and ectodermal dystrophy. specificendocrine dysfunction can include hypoparathyroidism, Addison's disease, hypothyroidism, and diabetes. this syndrome is also known as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy [APECED]. Ocular manifestations can be part of the disease and are often disabling. Here we describe the characteristics of APECED syndrome and its association with chronic interstitial keratitis, a rarely seen ocular manifestation with an early onset presentation