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August 1991

Retinal and Optic Nerve Manifestations of Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy

Author Affiliations

Chicago, Ill

Arch Ophthalmol. 1991;109(8):1065. doi:10.1001/archopht.1991.01080080023012

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive disease characterized by a variable failure of the parathyroid glands, adrenal cortex, gonad glands, pancreatic beta cells, gastric parietal cells, and thyroid gland. Mucocutaneous candidiasis, viral hepatitis, and dystrophy of dental enamel and nails may result from APECED. Alopecia, vitiligo, and keratopathy may also occur.1

We have followed up a 39-year-old man with APECED who demonstrates optic nerve and retinal changes that we believe are autoimmune.

Report of a Case.  —At age 19 years, the patient experienced decreased visual acuity in his right eye. Recently, he noted progressive loss of vision in his left eye over a 6-month period. His best corrected visual acuity was 20/400 OD and 20/40 OS. Color vision was absent in the right eye and normal in the left eye. An afferent pupillary defect was present in the right eye. Results of slit lamp examination and

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