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Keratitis, ichthyosis, and deafness (KID syndrome): review of the literature and proposal of a new terminology.

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).

Adult↗
PubMed1989

Jaccoud arthropathy and acroosteolysis in KID syndrome.

KID (keratitis, ichthyosis, deafness) syndrome is a congenital ectodermal defect of unknown etiology. Although achilles tendon retraction has been seen on several occasions, no other evidence of skeletal, articular or enthesopathic processes has been described. We report a patient with KID syndrome and acroosteolytic changes on radiographs, along with clinical and radiographic evidence of Jaccoud arthropathy.

Adult↗
PubMed1987

Keratitis, ichthyosis, and deafness (KID) syndrome. Vertical transmission and death from multiple squamous cell carcinomas.

A father and daughter had typical clinical features of the keratitis, ichthyosis, and deafness (KID) syndrome, as described by Skinner et al in 1981. To our knowledge, ours is the first observation of a vertical transmission of this syndrome. The mechanism of inheritance is uncertain. These two patients as well as the 26 previously described exhibited a typical hyperkeratotic eruption, which should not be confused with ichthyosis. The characteristic features are diffuse hyperkeratosis, keratotic plaques, reticulated hyperkeratosis on the face, peribuccal grooves, and heavy-grain leatherlike keratoderma. The occurrence of multiple squamous cell carcinomas underlines the seriousness of this congenital ectodermal defect. Etretinate can relieve the lesions without actually improving the chances of survival.

Adult↗
PubMed1986

Congenital alopecia in a Bichon Frise.

A Bichon Frise pup had congenital alopecia. Histologic evaluation revealed the absence of hair follicles, arector pili muscles, sebaceous glands, and sweat glands. Unlike previously described cases of congenital ectodermal defect, this alopecia was not associated with any color pattern; the pup was white until it was 4 months old, at which time normal black and brown pigmentation developed independently of the alopecic pattern.

Alopecia↗