Acquired Acrodermatitis Enteropathica

Abstract

Fatima Zahra ELFATOIKI, Anass ABBOUR, Fouzia HALI, Soumiya CHIHEB

Introduction: Acrodermatitis enteropathica (AE) is a rare disorder characterized by impaired zinc absorption, leading to severe zinc deficiency. The condition primarily manifests as an autosomal recessive disorder due to mutations in the SLC39A4 gene. However, an acquired form of AE may occur in exclusively breastfed infants due to insufficient maternal dietary zinc levels.

Case Presentation: We report a case of a 6-month-old infant, born at term to consanguineous parents, presenting with erythematous and papulovesicular lesions in the perineum with areas of scaling, stomatitis, and severe paronychia involving all nails. Sparse, brittle hair was also noted. The infant, exclusively breastfed since birth, developed these symptoms at 3 months of age, with progressive worsening. There were no gastrointestinal, nor neurological symptoms. Serum zinc levels were significantly reduced confirming zinc deficiency. Oral zinc supplementation (10 mg/kg/day) resulted in rapid improvement within three weeks.

Discussion: Acrodermatitis enteropathica, in its acquired form, is linked to inadequate maternal zinc intake during lactation. The absence of systemic symptoms in this case suggests localized manifestations of zinc deficiency, consistent with milder forms of the condition. Early recognition of AE based on clinical presentation, combined with serum zinc assessment, is critical to prevent progression to systemic involvement, including immune dysfunction and growth retardation.

Conclusion: Early diagnosis and intervention with oral zinc supplementation are essential to ensure favorable outcomes in acquired acrodermatitis enteropathica. Moreover, maternal nutritional optimization during pregnancy and lactation is pivotal for preventing zinc deficiency-related conditions in infants.

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