The so-called KID (keratitis, ichthyosis,
deafness)syndrome is a congenital disorder of ectoderm that affects
not only the epidermis, but also other ectodermal tissues such as the
corneal epithelium and the inner 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 ophthalmologic defects. The most frequent
clinical features were neurosensory deafness 90%, erythrokeratoderma 89%,
vascularizing keratitis 79%, alopecia 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 disorder is not an ichthyosis, because scaling is not the
main cutaneous feature and not all patients have keratitis early in the
course. We suggest that this syndrome should be included under
the general heading of congenital ectodermal defects as a keratodermatous
ectodermal dysplasia (KED).
