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Acrodermatitis dysmetabolica: lessons from two pediatric cases
Ayça Burcu Kahraman1, Meryem Sıla Cosar2, Ekrem Eren Dogan3
1Division of Pediatric Metabolism, Konya City Hospital, University of Health Sciences, Konya, Türkiye.
Objectives:
Acrodermatitis dysmetabolica (AD) is a dermatologic manifestation associated with inherited metabolic disorders (IMDs), distinct from acrodermatitis enteropathica, which occurs solely due to zinc deficiency.
Case Presentation:
This report presents two pediatric cases: a 30-month-old girl with maple syrup urine disease (MSUD) experiencing AD secondary to severe isoleucine deficiency due to a protein-restricted diet, showing improvement with dietary adjustments, and a 2.5-month-old boy infant with propionic acidemia (PA) who developed AD alongside septic shock, which progressed despite intervention.
Conclusions:
These cases emphasize the importance of identifying AD in IMDs and the critical need for meticulous monitoring of amino acid levels, as deficiencies may lead to severe complications.
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