Acrodermatitis-enteropathica-ähnliche Hautveränderungen bei Ahornsirupkrankheit

Maple syrup urine disease is a rare congenital disorder of the amino acid metabolism with neurologic and ophthalmologic manifestations as well as dry and visible thickened skin and dull, brittle hair. Beyond that, acrodermatitis enteropathica-like skin lesions may appear as a consequence of a restrictive dietary therapy leading to amino acid, zinc or niacin deficiency. In the following we report on a patient with maple syrup urine disease presenting with acrodermatitis enteropathica-like symptoms, both clinically and histologically. The manifold causes of the acrodermatitis enteropathica-like syndrome are discussed.