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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.
Journal of Pediatric Endocrinology & Metabolism : JPEM
|January 6, 2025
Summary
Acrodermatitis dysmetabolica (AD) in inherited metabolic disorders (IMDs) can arise from specific amino acid deficiencies. Early identification and monitoring of nutrient levels are crucial for managing this condition.
Area of Science:
- Metabolic disorders
- Dermatology
- Pediatrics
Background:
- Acrodermatitis dysmetabolica (AD) is a skin condition linked to inherited metabolic disorders (IMDs).
- It is distinct from acrodermatitis enteropathica, which results from zinc deficiency.
Observation:
- Two pediatric cases of AD in IMDs are presented.
- A girl with maple syrup urine disease (MSUD) developed AD due to isoleucine deficiency from dietary restrictions.
- An infant with propionic acidemia (PA) developed AD during septic shock.
Findings:
- The MSUD case showed improvement with dietary adjustments.
- The PA case showed disease progression despite interventions.
Implications:
- These cases highlight the importance of recognizing AD in IMDs.
- Meticulous monitoring of amino acid levels is critical to prevent severe complications in patients with IMDs.
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