RESTRICTIVE DERMOPATHY

By Gary M. White, MD


Restrictive dermopathy is a rare, autosomal recessive congenital skin disorder characterized by abnormally rigid skin, flexion contractures, and a chacteristic facial anomaly consisting of micrognathia, low-set ears, fixed open mouth, small pinched nose, and eyes with an antimongoloid slant.

Treatment

Unfortunately, the disorder is usually lethal.

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