Acrodermatitis Dysmetabolica in an Infant with Maple Syrup Urine Disease

Acrodermatitis dysmetabolica (AD) was reported in an infant with maple syrup urine disease (MSUD), a genetic disorder affecting the degradation of branched-chain amino acids (BCAAs). The infant developed symptoms similar to acrodermatitis enteropathica (AE), including dermatitis, diarrhea, and alopecia, after a period of inadequate BCAA formula intake. Unlike AE, which is linked to zinc deficiency, AD in this case was due to low isoleucine levels, as the infant had normal zinc levels. Increasing the isoleucine intake improved the condition, highlighting the need for careful management of BCAA levels in MSUD treatment to prevent AD.