잦은 감염과 홍반성 피부 경결이 있었던 IgA 및 IgG2, IgG4 결핍증 1예
A Case of IgA, IgG2, IgG4 Deficiency with Recurrent Infection and Erythematous Indurated Nodules
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초록
IgA deficiency is the most common form of primary immunodeficiency in Caucacians, but its prevalence has been reported to be low in Asians. Some patients with IgA deficiency also have deficits in 1 or more IgG subclasses. Patients with combined IgG2 and IgG4 subclass deficiency are likely to have a high susceptibility to infection or more frequently progress to various immunodeficiency. IgA deficiency may occur in conjunction with autoimmune disorders, among which lupus erythematosus and rheumatoid arthritis are the most common. IgA deficiencies with vascular involvement such as Henoch-Schönlein syndrome, Behcet’s disease, phacomatosis pigmentovascularis, necrotizing vasculitis with a polyarteritis nodosa-like pattern have been described. We present herein a 4-year-old girl with a history of recurrent infection and multiple 1- to 2-cm erythematous, painful, tender, indurated cutaneous nodules accompanied by IgA, IgG2 and IgG4 deficiency.
Abstract
IgA deficiency is the most common form of primary immunodeficiency in Caucacians, but its prevalence has been reported to be low in Asians. Some patients with IgA deficiency also have deficits in 1 or more IgG subclasses. Patients with combined IgG2 and IgG4 subclass deficiency are likely to have a high susceptibility to infection or more frequently progress to various immunodeficiency. IgA deficiency may occur in conjunction with autoimmune disorders, among which lupus erythematosus and rheumatoid arthritis are the most common. IgA deficiencies with vascular involvement such as Henoch-Schönlein syndrome, Behcet’s disease, phacomatosis pigmentovascularis, necrotizing vasculitis with a polyarteritis nodosa-like pattern have been described. We present herein a 4-year-old girl with a history of recurrent infection and multiple 1- to 2-cm erythematous, painful, tender, indurated cutaneous nodules accompanied by IgA, IgG2 and IgG4 deficiency.
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