A 9-month-old infant presents with symmetrical vesicobullous dermatitis around the mouth, anus, and on the hands and feet, along with chronic diarrhea, alopecia, and photophobia. Serum alkaline phosphatase is low. Which of the following is the MOST appropriate long-term treatment?

• A Topical corticosteroids alone
• B Lifelong oral zinc supplementation ✓
• C Biotin supplementation
• D Pancreatic enzyme replacement

Explanation

Acrodermatitis enteropathica is an autosomal recessive disorder of intestinal zinc absorption caused by mutations in the SLC39B4 gene encoding ZIP4 transporter. The triad of periorificial and acral dermatitis, alopecia, and diarrhea with low alkaline phosphatase, a zinc-dependent enzyme, is diagnostic. Treatment is lifelong oral zinc supplementation, which produces complete clinical remission. Topical steroids do not correct the metabolic defect.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.