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概要

A Case of Acrodermatitis Enteropathica

Jonathan Sutton and Thurein Newin

Clinicians should be mindful of all paediatric skin diseases, which present in a similar manner during the first months of life. Acrodermatitis enteropathica (AE) is a rare, bullous disorder that has a significant impact on the child’s quality of life and is fatal if untreated. AE is either a recessively inherited or a transiently acquired disorder. The hereditary form is caused by a genetic mutation in intestinal zinc absorption whereas the acquired form is caused by low nutritional intake or decreased peripheral release of zinc from the blood. This case report describes AE which manifested in a similar way to other blistering skin disorders, but showed a dramatic clinical improvement to oral zinc therapy. AE is characterised by a triad of periorificial and acral dermatitis, diarrhoea and alopecia. Both forms of AE affect males and females equally. The cutaneous lesions are annular, erythematous, scaly, crusted plaques, which are well demarcated. As the disease progresses these plaques become vesicobullous, pustular and erosive. The lesions are distributed symmetrically, around body openings such as the mouth anus, eyes and skin of the elbows, knees, hands and feet. The alopecia diffusely affects the eyelashes, eyebrows and scalp. As described in the case, secondary infection by candida albicans or staphylococcus aureus may complicate the disease. Classically the disease presented after the child was making the transition from breast milk to solid foods. Breast milk has comparatively higher zinc content and absorbability compared to infant formulas, cow’s milk or solid foods. This case emphasises the need for an open mind when treating childhood skin disorders. Once bullous skin disorders are suspected the case should be referred promptly to a specialist. An early diagnosis and prompt treatment of AE reduces mortality and prevents the long-term consequences of zinc deficiency.