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A 6 month old female child presented with vesicular and crusted lesions together with well defined, erythematous, glazed, moist plaques on perioral, perigenital, and perianal regions for last one month (fig 1). It was associated with intermittent diarrhoea. She was the first child of consanguineous parents and was normal at birth. She had been weaned from breast feeding over the past two and a half months.
Her anthropometric measurements were normal. Serum albumin was normal, but alkaline phosphatase (200 IU/l) and zinc levels (20 μg/dl) were low. The patient was diagnosed as having acrodermatitis enteropathica and was started on an oral preparation of zinc sulphate (0.2 mg/kg/day). There was marked improvement of all lesions within 48 hours and complete clearing by 7 days (fig 2). Continuation of maintenance oral zinc was advised.
Acrodermatitis enteropathica is a rare autosomal recessive disorder. There is a genetic defect of the synthesis, structure, or function of a zinc binding ligand normally present in the intestine. The clinical features usually start following weaning, when the protective effect of the zinc binding ligand from the mother’s milk is no longer present. Periorificial dermatitis is the hallmark for diagnosis and the response to oral or parenteral zinc is dramatic.