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A case of acquired angioedema with C1 esterase inhibitor deficiency

Other Title
후천성으로 추정되는 C1 esterase inhibitor 결핍이 동반된 혈관 부종 1례
하, 창영; 김, 선신; 김, 헌종; 한, 동석; 조, 재웅; 정, 혁준; 김, 희연; 남, 동호; 박, 해심
Chʿŏnsik mit alrerugi, 19(2):224-228, 1999
Journal Title
Chʿŏnsik mit alrerugi; Journal of asthma, allergy and clinical immunology; Korean journal of asthma, allergy and clinical immunology; 천식 및 알레르기
Angioedema is a well-demarcated localized edema involving the deeper layers of the

skin, including the subcutaneous tissue. Angioedema occurs with C1 esterase inhibitor

(C1 INH) deficiency that may be inborn as an autosomal dominant characteristic or may

be acquired. Acquired angioedema(AAE) is a rare disorder characterized by adult onset

and lack of evidence of inheritance of the disease. Two types of AAE are known today;

type I in which there are lowering of functional C1 INH, an underlying disease such as

a B-cell disease, and no delectable autoantibodies to C1 INH, type Ⅱ with anti C1 INH

autoantibodies in the circulation without detectable underlying disease and with

depressed functional C1 INH levels. We experienced a case of angioedema in a 29-year

old man. He had no family history of angioedema and laboratory data showed depressed

C1-INH levels. We diagnosed the case as acquired type of angioedema. Even though we

could not measure anti-C1 INH auto-antibodies, we identified the case as type Ⅱ

because there was no evidence of underlying disease.
acquired angioedemaC1-INH deficiency
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Journal Papers > School of Medicine / Graduate School of Medicine > Allergy
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