Keratitis, Ichthyosis and Deafness Syndrome: The First Case Report in China
- VernacularTitle:角膜炎、鱼鳞病、耳聋综合征一例国内首报
- Author:
Xibao ZHANG
;
Kang ZENG
;
Ju WEN
;
Zhengguang WU
;
Quan LUO
;
Liping LIU
;
Yanfang WANG
;
Xiao XU
;
Zhenping ZHANG
;
Yuanxing LIAO
- Publication Type:Journal Article
- Keywords:
Keartitis, ichthyosis and deafness syndrome
- From:
Chinese Journal of Dermatology
2003;0(07):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To report one case with congenital ichthyosiform eruption, neurosensory deafness and vascularizing keratitis. Methods The overall clinical and laboratory examinations were conducted to confirm the diagnosis of keratitis, ichthyosis and deafness (KID) syndrome. Results The case presented with the typical hypotrichosis features of the eye lashes and eyebrows, alopecia of the scalp, and ophtalmological lesions. The keratotic plaques over the face, nose, ears, and the extremities were characterstic, and the skin of the trunk was leather-like, dry and hyperkeratotic. Dysplasia of cerebellum, and cystic enlargement of the fourth ventricle of cerebrum, and Dandy Walker syndrome were observed on MRI scanning. Treatment with oral acitretin for 3 weeks cleared the hyperkeratotic ichthyotic lesions on her posterior scalp and also improved other lesions on the extremities and the trunk. Conclusion Acitretin seems to be promising in the treatment of keratotic skin lesions in KID syndrome.
