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Acrodermatitis dysmetabolica

K Flores, R Chikowski, D S Morrell
Acrodermatitis dysmetabolica (AD) is a rare, newly termed, and poorly understood disease that appears to be clinically similar to acrodermatitis enteropathica (AE). Both diseases are characterized by the triad of periorificial and acral dermatitis, diarrhoea, and alopecia. Unlike AE, which is caused by zinc deficiency, AD is caused by numerous metabolic disorders. One such disorder is maple syrup urine disease (MSUD), a genetic deficiency of branched chain α-ketoacid dehydrogenase, the enzyme that degrades the branched-chain amino acids (BCAAs) isoleucine, leucine and valine...
August 2016: Clinical and Experimental Dermatology
Sarah Strathie Page, Rachael Foster
Acrodermatitis dysmetabolica is an umbrella term encompassing the other metabolic causes of an erosive periorificial and acral dermatitis that mimics acrodermatitis enteropathica. Causes include acquired zinc, amino acid, biotin, and fatty acid deficiencies. We present the case of an exclusively breastfed, 2-month-old boy with known cystic fibrosis admitted with failure to thrive and erosive dermatitis. A diagnosis of acrodermatitis dysmetabolica was made when investigations revealed a normal zinc level but low amino acid levels...
March 2016: Pediatric Dermatology
Liqiao Ma, Stephanie Savory, Nnenna G Agim
We report a case of acquired protein energy malnutrition with associated zinc deficiency in an 18-month-old boy with type 1 glutaric acidemia. Physical examination findings included generalized nonpitting edema, widespread desquamative plaques, and sparse hair with a reddish tinge. Laboratory abnormalities included low levels of zinc, albumin, alkaline phosphatase, and iron. A review of skin manifestations of nutritional deficiencies, specifically kwashiorkor, is presented, as well as the relatively new entity called acrodermatitis dysmetabolica...
July 2013: Pediatric Dermatology
Duru Tabanlioğlu, Sibel Ersoy-Evans, Ayşen Karaduman
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...
March 2009: Pediatric Dermatology
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