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Clin Exp Dermatol ; 33(2): 148-50, 2008 Mar.
Article En | MEDLINE | ID: mdl-18076688

An 8-year-old boy presented with eczematous skin lesions, recurrent otitis media and unexplained pyrexias. X-linked agammaglobulinaemia was diagnosed and treatment commenced with intravenous immunoglobulin replacement therapy. X-linked agammaglobulinaemia (XLA) is a primary immunodeficiency syndrome associated with a deficiency of B lymphocytes, caused by a defect in the expression of Bruton's tyrosine kinase. It affects only boys and usually presents before the age of 2 years with recurrent bacterial sinopulmonary infections. IgG levels are usually <2 g/L (normal range 5.4-16.1) and IgM and IgA are usually undetectable. The commonest cutaneous features of XLA are pyogenic skin infections; however, eczema can occur with increased frequency. We report a child who presented with multiple discrete eczematous lesions who subsequently developed eczematous exacerbations several days after administration of intravenous immunoglobulin (IVIg) replacement therapy.


Agammaglobulinemia , Genetic Diseases, X-Linked , Agammaglobulinemia/diagnosis , Agammaglobulinemia/drug therapy , Agammaglobulinemia/immunology , Anemia/complications , Anemia/therapy , B-Lymphocytes/immunology , Child , Dose-Response Relationship, Immunologic , Eczema/complications , Eczema/drug therapy , Genetic Diseases, X-Linked/diagnosis , Genetic Diseases, X-Linked/drug therapy , Genetic Diseases, X-Linked/immunology , Humans , Immunoglobulins, Intravenous/administration & dosage , Immunoglobulins, Intravenous/adverse effects , Immunologic Factors/administration & dosage , Immunologic Factors/adverse effects , Male , Sex Factors , Treatment Outcome
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