Acrodermatitis enteropathica-like eruption in metabolic disorders: acrodermatitis dysmetabolica is proposed as a better term

Acrodermatitis acidemica is a recently proposed term for the rash that is similar to acrodermatitis enteropathica, which is encountered in organic acidemias. However, acrodermatitis enteropathica-like eruption may be seen in metabolic disorders other than organic acidemias.

The aim of this study was to evaluate the clinical features of acrodermatitis enteropathica-like eruption secondary to metabolic disorders.

Clinical and demographic features of 12 patients with acrodermatitis enteropathica-like eruption were prospectively evaluated between 2004 and 2006 in this single-center study.

Among the 12 patients, underlying metabolic disorders included maple syrup urine disease (n = 5), methylmalonic acidemia (n = 3), phenylketonuria (n = 2), ornithine transcarbamylase deficiency (n = 1), and propionic acidemia (n = 1). Mean age at first presentation was 29.9 months. Mean duration of acrodermatitis enteropathica-like eruption at the time of presentation was 25.2 days. The diaper area was involved in all presentations. Plasma zinc level was measured in 62.5% (n = 10) of the presentations and all had normal levels. All phenylketonuria cases had a low plasma phenylalanine level, and a low plasma isoleucine level was observed in the propionic acidemia case and all maple syrup urine disease cases. The rash responded dramatically to appropriate diet management in all cases.

In this study, acrodermatitis enteropathica-like eruption was noted in various metabolic disorders, including organic acidemias. We suggest that acrodermatitis dysmetabolica might be a better term for acrodermatitis enteropathica-like eruption occurring secondary to metabolic disorders other than acquired zinc deficiency.