Abstract

DE LA GUARDIA-PENA, Odalis M; USTARIZ-GARCIA, Catalino R; GARCIA-GARCIA, María de los Ángeles  and  MORERA-BARRIOS, Luz M. Hyper IgE syndrome. A case report. Rev Cubana Hematol Inmunol Hemoter [online]. 2012, vol.28, n.3, pp.299-305. ISSN 0864-0289.

Hyper IgE syndrome is a rare immunodeficiency initially described by Buckley in 1971. From 1972 to 2008, approximately 250 cases have been reported worldwide. Its clinical diagnosis is established through Grimbacher criteria and prognosis depends on timely diagnosis and treatment. A male patient aged 60, mestizo, with a history of having received surgical treatment of cervical spondylotic myelopathy more than 15 years ago is presented. In 1995, he also had a stroke and essential hypertension treatment. From his early ages he had multiple deep skin abscesses which were surgically removed; he also had several pneumonias that began in youth, as well as history of skin eczema that evolved by crises from severe to mild. This patient has a characteristic facies: broad nasal bridge, fleshy nose, prominent forehead. Due to this patient's history, the high dosage of IgE, high eosinophil count and the delay in the opsonophagocytic index, he was diagnosed as hyper IgE syndrome. He was treated with immunomodulators, parenteral B complex vitamin therapy, cimetidine, vitamin C in high doses and antihistamines. Clinical improvement of the initial condition was achieved after one year follow-up.

Keywords : hyper IgE syndrome; Job´s disease.

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