A case of acquired angioedema with C1 esterase inhibitor deficiency / 천식및알레르기
Journal of Asthma, Allergy and Clinical Immunology
;
: 224-228, 1999.
Article
in Korean
| WPRIM
| ID: wpr-71220
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Skin
/
Autoantibodies
/
Wills
/
B-Lymphocytes
/
Complement C1s
/
Subcutaneous Tissue
/
Edema
/
Complement C1 Inhibitor Protein
/
Angioedemas, Hereditary
/
Angioedema
Type of study:
Prognostic study
Limits:
Adult
/
Humans
Language:
Korean
Journal:
Journal of Asthma, Allergy and Clinical Immunology
Year:
1999
Type:
Article
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