KERATITIS, ICHTHYOSIS, AND DEAFNESS (KID SYNDROME) - REVIEW OF THE LITERATURE AND PROPOSAL OF A NEW TERMINOLOGY

Citation
H. Caceresrios et al., KERATITIS, ICHTHYOSIS, AND DEAFNESS (KID SYNDROME) - REVIEW OF THE LITERATURE AND PROPOSAL OF A NEW TERMINOLOGY, Pediatric dermatology, 13(2), 1996, pp. 105-113
Citations number
68
Categorie Soggetti
Dermatology & Venereal Diseases",Pediatrics
Journal title
ISSN journal
07368046
Volume
13
Issue
2
Year of publication
1996
Pages
105 - 113
Database
ISI
SICI code
0736-8046(1996)13:2<105:KIAD(S>2.0.ZU;2-2
Abstract
The so-called KID (keratitis, ichthyosis, deafness) syndrome is a cong enital disorder of ectoderm that affects not only the epidermis, but a lso other ectodermal tissues such as the corneal epithelium and the in ner ear. Sixty-one patients who fulfill the criteria for this syndrome were identified in a review of the literature through December 1993, All had cutaneous and auditory abnormalities, and 95% also had ophthal mologic defects. The most frequent clinical features were neurosensory deafness 90%, erythrokeratoderma 89%, vascularizing keratitis 79%, al opecia 79%, and reticulated hyperkeratosis of the palms and soles 41%. All of these findings constitute the major criteria for the diagnosis , The KID acronym does not accurately define this entity since the dis order is not an ichthyosis, because scaling is not the main cutaneous feature and not all patients have keratitis early in the course, We su ggest that this syndrome should be included under the general heading of congenital ectodermal defects as a keratodermatous ectodermal dyspl asia (KED).