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

Abstract:

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.

journal_name

Pediatr Dermatol

journal_title

Pediatric dermatology

authors

Tabanlioğlu D,Ersoy-Evans S,Karaduman A

doi

10.1111/j.1525-1470.2008.00803.x

subject

Has Abstract

pub_date

2009-03-01 00:00:00

pages

150-4

issue

2

eissn

0736-8046

issn

1525-1470

pii

PDE803

journal_volume

26

pub_type

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