Common characteristics of both types of this
syndrome include hyperkeratotic skin lesions, congenital sensorineural
hearing deficits, and corneal opacity often requiring corneal transplant.
The skin lesions start as erythematous, scaly patches on the face, ears,
extensor surfaces of the limbs, palms, and soles, which later become
brownish-yellow plaques. Ectodermal dysplasia is another major manifestation
of KID syndrome. Most patients have partial alopecia, fragile and malformed
nails, and small, malformed teeth. The recessive form also presents with
hepatic cirrhosis, which may progress to the point of requiring liver
transplantation by middle age, short stature, and mental retardation.
Epidermal glycogen deposits were present in some patients with the recessive