Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia

• Published in: Hautarzt 2015-10, Vol.66 (10), p.723-725
• Main Authors: Klossowski, N, Braun, S A, von Gruben, V, Losem, C, Plewe, D, Homey, B, Meller, S
• Format: Article
• Language: ger
• Subjects: Angioedema - complications; Angioedema - diagnosis; Angioedema - drug therapy; Antineoplastic Agents - administration & dosage; Chronic Disease; Complement C1 Inactivator Proteins - deficiency; Diagnosis, Differential; Drug Therapy, Combination - methods; Humans; Immunosuppressive Agents - administration & dosage; Leukemia, Lymphocytic, Chronic, B-Cell - complications; Leukemia, Lymphocytic, Chronic, B-Cell - diagnosis; Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy; Male; Middle Aged; Omalizumab - administration & dosage; Rituximab - administration & dosage; Treatment Outcome; Urticaria - diagnosis; Urticaria - drug therapy; Urticaria - etiology
• ISSN: 1432-1173
• DOI: 10.1007/s00105-015-3679-9

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. Thus, polychemotherapy targeting the slow-growing lymphoproliferative disease including rituximab was initiated, which resulted in remission of both the urticaria and the angioedema.