Keratitis-ichthyosis-deafness (KID) syndrome is a genetic disorder characterized by:

• Skin findings: Keratosis, ichthyosis, palmoplantar keratoderma, erythrokeratoderma, abnormal teeth / hair / nails, skin infections, delayed wound healing. Infants present at birth or soon after with mild diffuse erythema and fine scale (often diagnosed as ichthyosiform erythroderma). This resolves, but within the first year and generally within 3 months, typical scaly plaques appear on the face and limbs, along with hyperkeratosis of palms and soles. Bacterial, fungal, viral, and mite infections of the skin are troublesome in these patients.
• Ocular findings: KID syndrome is characterized by progressive inflammation and thickening of the cornea (vascularizing keratitis), which may not present until late childhood. Cataracts, scarring, and vison loss may also occur.
• Hearing loss: Sensorineural deafness.

There are two modes of inheritance for KID syndrome:

• The autosomal dominant form of KID (KIDAD) is caused by a mutation in GJB2 (connexin 26) on chromosome 13.
• Autosomal recessive keratitis-ichthyosis-deafness syndrome (KIDAR) is caused by a mutation in the AP1B1 gene on chromosome 22.

Additionally, mutation in the AP1S1 gene can cause a disorder with overlapping features of KID.