Acrodermatitis dysmetabolica: lessons from two pediatric cases

Ayça Burcu Kahraman; Meryem Sıla Cosar; Ekrem Eren Dogan; Yaşar Ünlü; Cuneyt Ugur; Zafer Bagcı

doi:10.1515/jpem-2024-0542

Article

Acrodermatitis dysmetabolica: lessons from two pediatric cases

Ayça Burcu Kahraman , Meryem Sıla Cosar , Ekrem Eren Dogan , Yaşar Ünlü , Cuneyt Ugur and Zafer Bagcı

Published/Copyright: January 7, 2025

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From the journal Journal of Pediatric Endocrinology and Metabolism Volume 38 Issue 3

Abstract

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.

Keywords: acrodermatitis dysmetabolica; inherited metabolic disorders; maple syrup urine disease; nutritional deficiency; propionic acidemia

Corresponding author: Ayça Burcu Kahraman, MD, Division of Pediatric Metabolism, Konya City Hospital, University of Health Sciences, Konya, Türkiye, E-mail: aycaburcuoksuz@gmail.com

Acknowledgments

We thank the patients and their family members.

Research ethics: Not applicable.
Informed consent: Verbal and written informed consent was obtained from the parents for the use of the clinical and laboratory data and photographs with patient anonymity.
Author contributions: The authors have accepted responsibility for the entire content of this manuscript and approved its submission. A.B.K., M.S.C., and E.E.D. contributed to the conception and study design of this case report. M.S.C., E.E.D., C.U., and Z.B. were involved in the care of the patient. Pathological preparations were conducted by Y.Ü; A.B.K. drafted the manuscript; and C.U. and Z.B. critically revised the manuscript.
Use of Large Language Models, AI and Machine Learning Tools: No AI or ML tools were used for this manuscript.
Conflict of interest: The authors state no conflict of interest.
Research funding: The authors received no financial support for the research, authorship, and/or publication of this manuscript.
Data availability: Not applicable.

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Supplementary Material

This article contains supplementary material (https://doi.org/10.1515/jpem-2024-0542).

Received: 2024-11-12

Accepted: 2024-12-16

Published Online: 2025-01-07

Published in Print: 2025-03-26

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Articles in the same Issue

https://doi.org/10.1515/jpem-2024-0542

Keywords for this article

acrodermatitis dysmetabolica; inherited metabolic disorders; maple syrup urine disease; nutritional deficiency; propionic acidemia