Ectodermal Dysplasia Osteosclerosis

Overview

Type of disease: Rare conditions

Ectodermal dysplasia osteosclerosis, also known as Cote-Katsantoni syndrome, is a rare genetic condition that affects a person’s hair, skin, teeth, and sweat glands. People with ectodermal dysplasia are usually born with wiry, brittle, and pale hair and also with milky white nails. Their skin is often dry, wrinkled, and darker in color around their joints. People with ectodermal dysplasia usually have missing teeth, or teeth that are small and pointed. Their sweat glands may not work like other peoples causing them to easily overheat. This is because they can not sweat as much as other people, meaning that they can not cool off and their body temperature can get too high. In addition to these symptoms, people with ectodermal dysplasia osteosclerosis have abnormally dense bones and low levels of neutrophils in the blood. Neutrophils are a part of the bodies defense system to fight disease and infection. With low levels of neutrophils, a person can get sick more easily and are more likely to get an infection from something such as a cut on their finger. There are various resources to help manage the condition. Doctors seek help from various therapists and community services to provide support and care for families affected by ectodermal dysplasia osteosclerosis.Talk with your doctor if you or your child has been diagnosed with ectodermal dysplasia osteosclerosis to find the best treatment for you.

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