Hereditary angioedema is considered an inherited disorder of the complement system, manifested by repeated crises of angioedema involving the skin, the gastrointestinal and respiratory tracts. Biopsies of normal skin obtained from 5 patients, diagnosed with hereditary angioedema, were found to be positive for fibrin and in one case also positive for C4. The sections stained with anti-fibrin serum showed fibrin deposits around isolated epidermal cells, while C4 staining revealed a homogeneous pattern along the epidermis. Fluorescence patterns and laboratory findings before and after treatment with danazol and/or anti-fibrinolytic treatment are reported.
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Background: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterisation of the AAE-C1-INH cohort in UK is required to inform management.
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From the Division of Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, California and.
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