Case Rep Hematol 2018 10;2018:7809535. Epub 2018 Jan 10.
Department of Medicine, Reading Hospital, West Reading, PA, USA.
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J Emerg Med 2011 Nov 16;41(5):e99-e101. Epub 2008 May 16.
Department of Internal Medicine, College of Medicine, Inha University, Incheon, South Korea.
Angioedema due to acquired C1 esterase deficiency is a rare condition and a non-inflammatory disease characterized by episodes of edema of the mucosa of the upper airway or gastrointestinal tract. The purpose of this case report is to heighten awareness among emergency physicians of a peritonitis-like condition that can develop into angioedema due to acquired C1 esterase inhibitor deficiency, and thereby help to prevent false diagnosis resulting in unnecessary surgical intervention. We report the case of a 21-year-old man who presented to the Emergency Department (ED) with abdominal pain. Read More
Eur J Gastroenterol Hepatol 2013 Apr;25(4):507-13
Departments of Internal Medicine, Sint Franciscus Gasthuis, 3045 PM Rotterdam, The Netherlands.
Acquired angioedema is a rare disorder caused by an acquired deficiency of C1 inhibitor. It is characterized by nonpitting, nonpruritic subcutaneous or submucosal edema of the skin, or of the respiratory or gastrointestinal tract. When localized in the gastrointestinal tract, it can cause severe abdominal pain, mimicking an acute surgical abdomen, or chronic recurrent pain of moderate intensity. Read More
Hautarzt 2015 Oct;66(10):723-5
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
Acquired angioedema due to C1 inhibitor deficiency (C1-INH-AAE) is characterized by recurrent edema of the subcutaneous and/or submucosal tissue without wheals and negative family history of angioedema. Here, we present the case of a patient with a chronic lymphatic B cell leukemia who suffered from both C1-INH-AAE and chronic spontaneous urticaria. Oral corticosteroids, antihistamines, and the anti-IgE antibody omalizumab were applied to treat the chronic urticaria in combination with the plasma-derived C1 esterase inhibitor concentrate Berinert® and the bradykinin B2 receptor antagonist icatibant, but the symptoms did not improved significantly. Read More
Ann Dermatol Venereol 2018 Apr 16. Epub 2018 Apr 16.
Dermatologie, CHRU Minjoz, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.
Background: Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Read More