Introduction:

dysmetabolic acrodermatitis can occur in patients with inborn errors of metabolism, such as maple syrup urine disease (MSUD).

Case description:

this is a male patient who, at eight days of age, presented hypotonia, lethargy, anorexia, apneas and cyanosis. Due to the neurological manifestations, an inborn error of metabolism is suspected, requesting metabolic screening. Due to elevated plasma levels of leucine, isoleucine and valine, a diagnosis of MSUD was established, starting a diet free of these amino acids. At five months of age, he was identified with disseminated dermatosis: confluent erythematosquamous plaques, poorly delimited, considered as dysmetabolic acrodermatitis; after seven days of adjusting therapy, improvement of the dermatological lesions was observed. He remained stable until 2 years of age, when he was readmitted for dermatosis exacerbation and metabolic acidosis; however, the patient died despite intensive care management.

Conclusions:

in dysmetabolic acrodermatitis, skin lesions can be a marker of decompensation of a metabolic disease. Early identification of this complication can help offer timely treatment.

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