Abstract:Acrodermatitis dysmetabolica (AD) describes eruptions characterized by the clinical triad of acral dermatitis, diarrhea, and alopecia. AD can be caused by various metabolic disorders one of which is maple syrup urine disease (MSUD). We present a 2-month-old boy diagnosed with MSUD by the age of 5 days and treated with branched-chain amino acid (BCAA) restricted diet, BCAAs formula, and thiamine supplementation. He was referred to dermatology with a 3-week history of diarrhea, progressive acrodermatitis enterop… Show more
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