KID SYNDROME

By Gary M. White, MD


Keratitis-Ichthyosis-Deafness Syndrome (KID syndrome) is a rare genodermatosis.

Clinical

The skin is red, dry, and thickened at birth and later develops fine, dry scale. Nail dystrophy and patchy alopecia of the scalp, eyelashes, and eyebrows are other characteristic cutaneous signs. Sensorineural deafness develops usually from 1-7 years of age. Vascularization and inflammation of the cornea usually develops in early adolescence. Visual acuity may be decreased and blindness may occur. Skin infections, dental defects, follicular hyperkeratosis, a reticulated pattern of hyperkeratosis of the palms, brown, rippled leather-like plaques on the knees, and squamous cell carcinoma (SCC) may also occur. Porokeratotic eccrine ostial and dermal duct nevus has been associated.

It is suggested that chronic inflammation, secondary bacterial infection, and the genetic mutation may all predispose to the SCCs.

Treatment

Multiple disciplines should be involved. Hearing and visual function should be closely monitored. Genetic counseling may be indicated. Monitoring for infection and skin cancer is key.

For the skin, topical moisturizers and keratolytic agents may be tried. Oral retinoids have been used with varying success.

References

SCC arising in KID syndrome. Acta Dermato-Venereologica

Generalized erythrokeratoderma since birth. Dermatology Online Journal

Scalp cysts, alopecia, and reticulated hyperkeratosis. JAAD July 2013;Volume 69, Issue 1, Pages 127–134.e3

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