What is hypohidrotic ectodermal dysplasia?

hypohidrotic ectodermal dysplasia or anhidrotic ectodermal dysplasia concerns a group of rare congenital disorders that usually cause abnormal prenatal development of hair, skin, sweat glands and teeth. Physical organs and skeletal tissue may also be affected. Anomalies affect primarily male infants, but women can also inherit a disorder. Scientists generally classify syndromes such as autosomal dominant, autosomal recessive or X-connected. This great assembly of conditions includes Christ-Siemens-Touraine, Hermann Werner Siemens and Rapp-Hodgkin syndrome.

symptoms differ depending on whether the disorder is associated with gender or autosomal; The variety associated with sex usually represents more serious features. In addition to obvious physical deformities, the common feature of missing or non -functional skin glands to the inability of the body to hydrate the skin or sweat sweating. These children are easily exposed to hyperthermia and must use external cooling methodsmore organs.

children born in the form of hypohidrotic ectodermal dysplasia known as Christ-Siemens-Touraine syndrome have generally fragile, fine, short blond hair that is usually thin. Their dry, glossy smooth skin is grayish white and often lacks or contains non -functional oil and sweat glands. When these infants begin to grow teeth, they are also thin and malformed. The syndrome usually creates clear facial features that include deformed ears, flat seat bridged nose, protruding forehead and reinforced lips. Boys can have a particularly small figure and look feminine.

hypohidrotic ectodermal disorder of dysplasia known as Hermann Werner Siemens primarily causes skin conditions. Young people develop pilarois keratosis or impacts and rashes around hair follicles that cause closure and giving holes is the overall form of goose-bump. Trauma hair shaftIt lasts and at the same time forces the way to the surface, often leads to damage and changed appearance, because each hair becomes somewhat toured. The groups of these sewn hair produce a wool patch. The disorder initially affects the hair on the face and scalp, including eyebrows and eyelashes, but eventually affects the entire surface of the body.

2 The detection of the skin rash cycle can eventually produce baldness. These children can also experience thickened eyelids, have corneal deformations or lower jaws and be very sensitive to the sun.

RAPP Hodgkin is genodermatosis with characteristics reminiscent of many other hypohidrotic ectodermal dysplasia, in the fact that children usually have sparse hair and either lack or have incorrectfunccing of the skin glands. In addition, these infants can be born with split panels affecting the roof of the mouth and upper lip. Fingers and nails also grow abnormally, and young people usually experience retarded physical growth.