Eczema and X-linked agammaglobulinaemia.
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.
Hunter
H L
HL
Department of Dermatology, Belfast City Hospital Trust, Belfast, UK. helenhunter5@hotmail.com
McKenna
K E
KE
Edgar
J D M
JD
D000361:Agammaglobulinemia / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D000740:Anemia / Q000150:complications / Q000628:therapy; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D004306:Dose-Response Relationship, Immunologic; D004485:Eczema / Q000150:complications / Q000188:drug therapy; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000009:adverse effects; D007155:Immunologic Factors / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D012737:Sex Factors; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors