Resumo

PEREZ-ELIZONDO, AD  e  LOPEZ-LARA, ND. Erythroderma in infancy associated with immunodeficiency: A case report. Rev. argent. dermatol. [online]. 2016, vol.97, n.4, pp.50-56. ISSN 1851-300X.

Exfoliative dermatitis or erythroderma in infancy is rare. Clinicians need to be alert to the possible diagnosis of Omenn syndrome (OS), a rare form of combined immunodeficiency in infants presenting with recurrent infections, erythroderma, lymphadenopathy, hepatosplenomegaly, eosinophilia, and increased serum IgE levels. OS is fatal unless treated by hematopoietic stem cell transplantation. We described a seven months of age female patient with respiratory infection and diarrhea, associated with hepatosplenomegaly and disseminated lymphadenopathy. Were evident laboratory anomalies suspected a complex immunodeficiency problem.

Palavras-chave : Omenn syndrome; severe combined immunodeficiency; bone marrow transplantation.