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目的 报告1例角膜炎·鱼鳞病·耳聋综合征(Keratitis ichthyosis anddeafnes s syndrome,KID syndrome)。 方法 对我院2003年4月发现并确诊治疗的1例患者的临床资料进行分析。 结果 患者为16岁女孩,出生不久皮肤即出现鱼鳞样结痂,头皮明显角化过度,头顶部、鼻尖、双耳、面颊、双下肢自膝部以下呈过度角化的蛎壳样损害。听觉丧失,喉部可以发声,不会讲话。视力较差,秃睫,畏光。躯干部皮肤明显干燥,皮肤角化过度,呈皮革样变化。病理活检为鱼鳞病,耳科检查为先天感音神经性耳聋,眼科检查为角膜血管翳。 结论 根据患者临床表现、皮损特征及各项检查结果,诊断为KID综合征,并给予阿维A治疗,皮肤科症状明显改善。
Objective To report a case of keratitis ichthyosis and deafness s syndrome (KID syndrome). Methods The clinical data of 1 patient who was diagnosed and diagnosed in our hospital in April 2003 were analyzed. Results The patient was a 16-year-old girl. Scales were scaly on the skin shortly after birth. The scalp was hyperkeratotic. The top of the head, nose, ears, cheeks and both lower extremities were hyperkeratotic oyster shell-like lesions below the knee. Hearing loss, throat can sound, can not speak. Poor vision, bald eyelashes, photophobia. Trunk skin was dry, hyperkeratosis, leather-like changes. Pathological biopsy for ichthyosis, otological examination for congenital sensorineural deafness, ophthalmic examination of corneal blood vessels. Conclusion According to the clinical manifestations of patients, the characteristics of lesions and the results of various tests, diagnosis of KID syndrome, and given Avia A treatment, dermatology symptoms improved significantly.