Acrodermatitis dysmetabolica: lessons from two pediatric cases
Ayça Burcu Kahraman, Meryem Sıla Cosar, Ekrem Eren Dogan, Yaşar Ünlü, Cuneyt Ugur, Zafer BagcıAbstract
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.