RESUMEN
Acrodermatitis enteropathica (AE) is a rare inherited form of defective zinc absorption usually manifesting as eczematous and erosive dermatitis preferably over periorificial and acral areas which at times mimics various hereditary palmoplantar keratodermas (PPK). We reported a 6-year-old boy who presented with hard plaques over both palm and sole, and periorificial areas, and flexion attitude of digits without any history of diarrhea and prolonged illness. The patient improved dramatically with zinc supplementation including correction of flexion deformities of the fingers. There was improvement of serum alkaline phosphatase level within 3 months. The lesions reappeared again whenever there was discontinuation of therapy for few weeks, thus confirming it to be zinc deficiency dermatoses. This kind of manifestation has rarely been described in earlier literature.