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Diseases

Genetic and Rare Diseases Information Center (GARD)

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KID syndrome


Other Names for this Disease
  • Keratitis, Ichthyosis, and Deafness (KID) Syndrome
  • Keratitis-ichthyosis-deafness syndrome, autosomal dominant
  • KID syndrome, autosomal dominant
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Your Question

I am a special education teacher. My new student is 5-years-old and is very impacted by this condition. Is his intelligence presumed to be typical? Also are his fungal infections contagious to other students and staff?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is KID syndrome?

Keratitis ichthyosis deafness (KID) syndrome is a very rare, inherited, multi-system disorder. Less than 100 cases have been reported in the medical literature. It is characterized by defects of the surface of the corneas (keratitis); red, rough, thickened plaques of skin (erythrokeratoderma); and sensorineural deafness or severe hearing impairment. The skin on the palms of the hands and soles of the feet as well as the nails may be affected. KID syndrome belongs to a group of skin disorders marked by dry, scaly skin known as the ichthyoses. KID syndrome is inherited as an autosomal dominant trait and is caused by specific mutations in the GJB2 gene.[1][2]
Last updated: 9/9/2011

What are the signs and symptoms of KID syndrome?

KID syndrome is present at birth. Nearly all cases have skin involvement, which includes red, rough, thickened plaques that are sometimes scaling, as well as sensorineural deafness or severe hearing impairment. Most affected individuals develop eye problems, usually keratitis (cornea defects), which can result in the eyes being very sensitive to light, small blood vessels growing from the iris over the cornea (neovascularization), and progressive vision loss. A small percentage of people may have recurrent or chronic inflammation of the mucous membrane of the eye (conjunctivitis).[1]

Sparse hair or areas of baldness (alopecia) is relatively common, while a complete lack of hair is rare. The palms of the hands and soles of the feet typically have thickened, hardened skin. Less common is absent or abnormal nails. Other associated symptoms may include recurrent infections, abnormal teeth, reduced sweating,. Some of the rare features include an increased risk for developing squamous cell carcinoma of the skin or mucous membranes and life-threatening infections during the newborn period.[1]
Last updated: 9/9/2011

Is intelligence affected in those with KID syndrome?

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.[2]
Last updated: 9/9/2011

Are the skin manifestations of KID syndrome contagious?

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.[2] 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.[3] 

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/
Last updated: 9/15/2011

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