Abstract

D'ALESSANDRO, Virginia  and  PEREZ, Néstor. Diagnóstico temprano del síndrome de hiper IgE: un desafío. Arch. argent. pediatr. [online]. 2004, vol.102, n.4, pp.290-295. ISSN 0325-0075.

Faced to children with repeated, severe or opportunistic infections, the pediatrician must sometimes consider acquired or congenital immunodeficiencies as a diagnostic possibility. Some of the commonest immune defects, as human immunodeficiency virus infection or profound humoral defects, are usually easily ruled out. Some others require a high suspicion index; adequate specialized advice, or both. The hyper-IgE syndrome is an immunodeficiency characterized by chronic eczematous dermatitis, recurrent skin and sinopulmonary tract infections, coarse facies and very high serum IgE levels. Associated dental and skeletal features have been also recognised. Many immune abnormalities have been described in small series of patients affected with hyper-IgE syndrome; however, there is not a test of definitive diagnostic value for this syndrome. For the pediatrician, the suspicion and diagnosis of this syndrome is difficult, particularly in infants and in atypical cases. Two patients with final diagnosis of hyper-IgE, in whom their signs and symptoms were initially confusing, are described.

Keywords : Hyper IgE syndrome; Buckley's syndrome; Recurrent skin infections; Sinopulmonary infections; Pneumatoceles.

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