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Pathophys and Cause ⬇

Cause:Autosomal recessive and dominant forms

Signs and Symptoms ⬆ ⬇

Si:Severe blistering with minor trauma causing contractures and protein loss; die by age 30 yr in recessive forms

Treatment ⬆

Rx:Phenytoin, formerly thought helpful, but not proven so (Nejm 1992;327:163)