Abstract

PUEBLA-MIRANDA, Miriam; MARTINEZ-LUNA, Eduwiges  and  VEGA-MEMIJE, María Elisa. Hyperimmunoglobulin E syndrome: report of two cases. Bol. Med. Hosp. Infant. Mex. [online]. 2009, vol.66, n.6, pp.545-552. ISSN 1665-1146.

Background. Hyperimmunoglobulin E syndrome is a rare systemic immunodeficiency characterized by eczematous dermatitis, recurrent cold abscesses, lung infections with pneumatoceles, coarse facial appearance, high IgE levels and eosinophilia. Case reports. Case 1: We report the case of an 11-year-old female with a history of recurrent lung infections, recurrent gastroenteritis, eczematous dermatitis affecting the skin folds and cold abscesses. Laboratory studies showed elevated eosinophils (16 070) and IgE 4864 IU. The patient received treatment with g-globulin, showing adequate clinic response to treatment. Case 2: We present the case of a 12-year-old female with a history of recurrent otitis and suppurative conjunctivitis, showing widespread and chronic infected eczema. Laboratory studies showed elevated IgE (3 000 IU). She was treated with dapsone, trimethoprim/sulfamethoxazole and methotrexate. Conclusions. We presented two patients with eczematous skin, recurrent infections and increased IgE levels, which are compatible with hyperimmunoglobulin E autosomal recessive syndrome.

Keywords : hyperimmunoglobulin E syndrome; Job's syndrome; eczema; lung infection; cold abscesses; congenital immunodeficiency.

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