21April2018

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Hypohidrotic Ectodermal Dysplasia- Drug Today Medical Times

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Commonly called the 'vampire disorder', Hypohidrotic Ectodermal Disorder (HED) affects one in 17,000.

People inflicted with the disorder suffer from pointed teeth and have severe hair, nails and skin problems. The shape of their teeth makes them look similar to the blood sucking creatures in fiction. Among severe cases of HED signs of premature aging along with thin and pale bodies, with dark circles under and around the eyes and missing or pointed teeth are observed.

HED is one of about 150 types of ectodermal dysplasia in humans. Most people suffering from this disease have a reduced ability to sweat (hypohidrosis) due to less number of sweat glands as compared to normal individuals. Due to the inability to sweat properly, the body temperature increases to dangerous levels (hyperthermia), particularly in hot weather. In some cases, this situation can be life threatening.

Those affected must keep a check on their body temperature regularly and remain away from sunlight and heat for long periods of time. HED is the most common form of ectodermal dysplasia, affecting one in every 17,000 people, including American actor, Michael Berryman. Most cases are caused due to mutation in the EDA gene, inherited in an X-linked recessive pattern. Males are affected by X-linked disorders much more frequently than females as fathers cannot pass X-linked traits to their sons.

Hair in affected individuals are usually sparse on the scalp and body (Hypotrichosis), light-colored, brittle, and slow growing. HED patients are also characterized by absent teeth (hypodontia) or malformed teeth. Other distinctive facial features include thin, wrinkled, and dark-colored skin around the eyes along with some chronic skin problems such as eczema and a bad smelling nasal discharge (ozena).

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