Acrodermatitis dysmetabolica: Lessons from two pediatric cases
Journal of Pediatric Endocrinology and Metabolism, cilt.38, sa.3, ss.299-304, 2025 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 38 Sayı: 3
- Basım Tarihi: 2025
- Doi Numarası: 10.1515/jpem-2024-0542
- Dergi Adı: Journal of Pediatric Endocrinology and Metabolism
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, EMBASE, MEDLINE
- Sayfa Sayıları: ss.299-304
- Anahtar Kelimeler: acrodermatitis dysmetabolica, inherited metabolic disorders, maple syrup urine disease, nutritional deficiency, propionic acidemia
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
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.