Acrodermatitis Enteropathica-Like Eruption in Metabolic Disorders: Acrodermatitis Dysmetabolica Is Proposed as a Better Term


PEDIATRIC DERMATOLOGY, cilt.26, sa.2, ss.150-154, 2009 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26 Konu: 2
  • Basım Tarihi: 2009
  • Doi Numarası: 10.1111/j.1525-1470.2008.00803.x
  • Sayfa Sayıları: ss.150-154


Background: 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. Objective: The aim of this study was to evaluate the clinical features of acrodermatitis enteropathica-like eruption secondary to metabolic disorders. Methods: Clinical and demographic features of 12 patients with acrodermatitis enteropathica-like eruption were prospectively evaluated between 2004 and 2006 in this single-center study. Results: 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. Conclusion: 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.