Abstract

Acrodermatitis enteropathica (AE) is a rare genetic autosomal recessive disorder, characterized by periorificial dermatitis, alopecia, and diarrhea due to zinc deficiency. We report a case of a 9-month-old baby boy with hair loss for 2 months, diarrhea for 1.5 months, skin peeling starting around mouth, nose, anus, gradually spreading all over body over 1 month, and fever for 10 days. Due to superadded bacterial infections and altered clinical picture, he was diagnosed as a case of staphylococcal scalded skin syndrome. With low serum zinc levels and improvement of skin lesions and diarrhea within 8 days of starting oral zinc therapy, it was confirmed to be a case of acrodermatitis enteropathica. It is important to consider AE as one of the differential diagnoses in pediatric chronic diarrhea cases with acral and/or periorificial skin lesions to prevent delay in the zinc supplementation treatment and mortality.

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