A case of acquired angioedema with C1 esterase inhibitor deficiency.
- Author:
Chang Young HA
;
Sun Sin KIM
;
Hun Jong KIM
;
Dong Suk HAN
;
Jae Woong CHO
;
Hyuck Jun CHUNG
;
Hee Yeon KIM
;
Dong Ho NAHM
;
Hae Sim PARK
- Publication Type:Case Report
- Keywords:
acquired angioedema;
Cl-INH deficiency
- MeSH:
Adult;
Angioedema*;
Angioedemas, Hereditary*;
Autoantibodies;
B-Lymphocytes;
Complement C1 Inhibitor Protein*;
Complement C1s*;
Edema;
Humans;
Skin;
Subcutaneous Tissue;
Wills
- From:Journal of Asthma, Allergy and Clinical Immunology
1999;19(2):224-228
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Angioedema is a well-demarcated localized edema involving the deeper layers of the skin, including the subcutaneous tissue. Angioedema occurs with Cl esterase inhibitor (Cl 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 Cl INH, an underlying disease such as a B-cell disease, and no detectable autoantibodies to Cl INH, type II with anti Cl INH autoantibodies in the circulation without detectable underlying disease and with depressed functional Cl 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 Cl-INH levels. We diagnosed the case as acquired type of angioedema. Even though we could not measure anti-Cl INH auto-antibodies, we identified the case as type II because there was no evidence of underlying disease.
