Acrodermatitis enteropathica-like simulating severe atopic dermatitis: a case report.

Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder of zinc (Zn) deficiency. AE is the clinical phenotype of Zn deficiency and is characterized by pustular and bullous dermatitis with an acral and periorificial distribution, frequently associated with pustular paronychia, angular stomatitis, glossitis, generalized alopecia and diarrhoea. Initially the term AE was used to describe the congenital form. This genetic defect has been mapped to 8q24 and the defective gene identified as SLC39A4, which encodes the zinc transporter Zip4. Nowadays, AE is also associated with the acquired form of Zn deficiency resulting from impaired intestinal absorption due to gastrointestinal diseases or by poor consumption of the mineral. In this case it has been denominated as AE like (AEL) disease. The diagnosis of AE is made by clinical presentation, dietary survey, laboratory tests, and histopathological findings (characteristic skin lesions). In some patients the differential diagnosis with other skin diseases is difficult and can generate delay in the correct diagnosis and appropriate treatment. In this report we present an infant with AEL probably secondary to an inadequate dietary management due to suspicious atopic dermatitis (AD) related to cow’s milk allergy.