Hereditary angioedema: 44 years of diagnostic delay.
نویسندگان
چکیده
We report a 64-year-old man who suffered from recurrent visible swelling attacks since the age of 20 as well as episodes with severe upper airway edema, resulting in 4 emergency tracheotomies. Eventually after 44 years he was diagnosed with hereditary angioedema (HAE) type II. The aims of this report is to emphasize the importance of awareness concerning HAE, which does not respond to traditional anti-allergic therapy, and remind physicians to test for functional C1-INH deficiency.
منابع مشابه
Report on the First Survey of Iranian Patients with Hereditary Angioedema
Background: Hereditary angioedema (HAE) is a rare autosomal dominant primary immunodeficiency with complement system defect characterized by recurrent episodes of angioedema involving the skin or mucosa of the upper respiratory and gastrointestinal tracts. Objective: To characterize the clinical and laboratory data of hereditary angioedema in Iran. Methods: Patients with probable diagnosis of a...
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Hereditary angioedema is a rare disorder of complement system which is often seen with autosomal dominant hereditary. Clinical characteristics include non- pruritic and non-pitting mucocutaneous edema that could involve all parts of the body. This study reports seven cases of hereditary angioedema with classical manifestations accompanied by low function of C1INH (type 2). One death occurred du...
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عنوان ژورنال:
- Dermatology online journal
دوره 22 4 شماره
صفحات -
تاریخ انتشار 2016