Wagman R D, Kazdan J J, Kooh S W, Fraser D
Am J Ophthalmol. 1987 Apr 15;103(4):569-75. doi: 10.1016/s0002-9394(14)74281-3.
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.
多内分泌腺功能减退、自身免疫性疾病、念珠菌病综合征眼部和全身症状的最佳管理需要早期识别受影响个体。这种常染色体隐性综合征的特征是甲状旁腺功能减退、艾迪生病以及慢性黏膜皮肤念珠菌病，还伴有其他内分泌病和免疫紊乱。我们回顾性分析了16例患者，其中14例患有完全型综合征，2例患有部分表现型综合征。这些患者中有4例出现了自限性双侧角膜炎，发病年龄在2至9岁之间。4例患者中有2例角膜炎先于任何内分泌病出现，且是该综合征的首发症状之一。角膜炎并非由甲状旁腺功能减退或念珠菌病引起。前部基质血管化和瘢痕形成导致8只患眼中有4只视力降至20/50或更差。我们建议对角膜疾病进行药物治疗，无需手术干预。
