Keratitis associated with the multiple endocrine deficiency, autoimmune disease, and candidiasis syndrome

Optimal management of the ocular and systemic components of the multiple endocrine deficiency, autoimmune disease, candidiasis syndrome requires early identification of affected individuals. This autosomal recessive syndrome is characterized by hypoparathyroidism, Addison's disease, and chronic mucocutaneous candidiasis among other endocrinopathies and immune disorders. We retrospectively reviewed 16 patients, 14 with the full syndrome and two with the syndrome partially manifested. Four of these patients manifested a self-limited, bilateral keratitis in which the age of onset ranged from 2 to 9 years. Keratitis preceded the onset of any endocrinopathy in two of four patients and was among the first signs of the syndrome. The keratitis was not caused by hypoparathyroidism or candidiasis. The anterior stromal vascularization and scarring resulted in a visual acuity of 20/50 or worse in four of eight affected eyes. We recommend medical management of the corneal disease without surgical intervention.