角膜炎、鱼鳞病、耳聋综合征

Keratitis-ichthyosis-deafness syndrome

报告1例并发头皮严重感染的散发性典型角膜炎、鱼鳞病、耳聋综合征。患儿男,4岁。出生时呈火棉胶样婴儿,随年龄增长皮肤角化过度加重,并发严重的头皮感染,并具有典型的角膜炎、鱼鳞病及耳聋症状。皮损组织病理检查示严重的角化过度,眼科检查表现为血管性角膜炎,听力检查表现为感音神经性耳聋。诊断为KID综合征。该例患儿经口服阿维A,外用维生素E、尿囊素等润肤,口服抗生素、外用莫匹罗星软膏抗感染及外用环孢素眼药水和人工泪液等治疗后,症状明显缓解。

A case of sporadic keratitis-ichthyosis-deafness（KID）syndrome accompanied with severe scalp infection is reported.A 4-year old boy displayed collodion baby-appearance at birth.As he grew,skin hyperkeratosis exacerbated and severe scalp infection developed,showing typical symptoms of keratitis,ichthyosis and deafness.Histological examination revealed severe hyperkeratosis.Ophthalmological examination showed vascular keratitis while hearing tests showed sensorineural deafness.The diagnosis of KID syndrome was made.The patient＇s symptoms were alleviated after the treatment with oral acitretin A,antibiotics,topical vitamin E and allantoin lotion,topical mupirocin,cyclosporin eye drops and artificial tears eye drops.