Chronic granulomatous disease (CGD) is a primary immunodeficiency, it has a defect in phagocytosis and its estimated incidence is 1 in 250,000 live births. Recurrent infections and granulomas are the most common clinical manifestations. CGD is caused by a functional defect in one of the subunits of the NADPH oxidase, the patients have mutations in NADPH genes.
View Article and Find Full Text PDFCommon variable immunodeficiency (CVID) is an immunodeficiency characterized by an impaired ability to produce antibodies associated to multiple clinical phenotypes. The incidence is around 1/50,000 new borns. The age at diagnosis shows 2 peaks, between 1-5 and 16-20 years old.
View Article and Find Full Text PDFHypocomplementemic urticarial vasculitis is a type of urticarial vasculitis with multisystemic involvement and poor prognosis, sometimes associated with systemic lupus erythematosus. Several therapies have been attempted with no consensus on an effective therapeutic regimen. Intravenous immunoglobulin has been used in severe manifestations of systemic lupus erythematosus and recently in hypocomplementemic urticarial vasculitis.
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