[Anhidrotic ectodermal dysplasia syndrome in the neonatal period - case report] / Síndrome da displasia ectodérmica anidrótica no período neonatal - relato de caso.

OBJECTIVE: To describe a rare syndrome affecting children, and to urge pediatricians to consider this diagnosis when investigating idiopathic fever in neonates. METHODS: We report a case of anhidrotic ectodermal dysplasia syndrome in a 10 day old newborn in the neonatal intensive care unit at Hospital Geral de Caxias do Sul. The child presented recurrent episodes of fever since the first days of life. RESULTS: The patient presented dry mucus, dry skin, and fever. Skin biopsy was performed in the childacute;s back. Absence of sweat and sebaceous glands as well as hypoplasia of follicular structures were identified. The patient is receiving adequate treatment, and is being followed as an outpatient. CONCLUSION: Anhidrotic ectodermal dysplasia syndrome is a rare disorder that must be considered when investigating newborns with recurrent episodes of fever. During the neonatal period, the clinical manifestations of the disease are subtle and unspecific. Such findings become more visible after a few months of life. There is no definitive treatment for this syndrome.