KID syndrome

Keratitis-ichthyosis-deafness (KID) syndrome is a rare disorder that causes skin abnormalities, eye problems, and hearing loss. Skin abnormalities include thick, hard skin on the underside of the hands and feet (palmoplantar keratoderma); thick, red patches of skin (erythrokeratoderma); and dry, scaly skin (ichthyosis). Eye problems are caused by keratitis (inflammation of the cornea) which can lead to pain; sensitivity to light; extra blood vessel growth; scarring; and eventual vision loss or blindness. KID syndrome is caused by mutations in the GJB2 gene. Most cases are sporadic but familial cases with autosomal dominant inheritance have been reported. Treatment focuses on the specific symptoms present.

In general, the skin manifestations are progressive. The skin lesions are also prone to infection and rare fatal cases of severe recurrent infections have been reported. The cutaneous fungal infections that occur in those with KID syndrome are generally not contagious. However, exposure to a high concentration of the fungi would be more likely to cause infection in other staff members or students. If the child has a cutaneous fungal infection, it would be appropriate to treat topically, both to control the signs and symptoms and to reduce exposure to others.

You might also consider contacting the following support organization as they may be able to provide additional information:

Foundation for Ichthyosis and Related Skin Types (FIRST)
2616 North Broad Street
Colmar, PA18915
Toll free: 1-800-545-3286
Telephone: 215-997-9400
E-mail: info@firstskinfoundation.org
Web site: http://www.firstskinfoundation.org/

We were unable to find specifiic information about intelligence being affected in those with this condition. However, the combined vision and hearing loss may lead to developmental delay. Cerebellar and neuromuscular defects have also been reported in a few cases.

The long-term outlook for people with KID syndrome varies, although fatal complications are rare. The symptoms and severity may vary greatly between patients and may change over time. The skin features are generally persistent and are prone to infections, which can be life- threatening (especially in newborns). While the skin symptoms and hearing loss are typically present from birth, eye problems usually begin during childhood or adolescence and worsen with time.

Even though the general prognosis seems good, life-long follow-up is necessary because the condition can be associated with malignant tumors (cancer), particularly squamous cell carcinoma (SCC). SCC of the skin and oral mucosa is a rare but serious complication that can shorten life expectancy.