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

Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete
N KlossowskiS Meller

Abstract

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.

References

Dec 1, 2012·Annals of Dermatology·Ozgur KartalSener Osman
Jan 31, 2014·BMJ Case Reports·Erin O'Donnell, Rachel Havyer
Mar 29, 2014·Allergy·M CicardiUNKNOWN HAWK under the patronage of EAACI (European Academy of Allergy and Clinical Immunology)
Jun 9, 2015·Journal of the European Academy of Dermatology and Venereology : JEADV·M MaurerM Sánchez-Borges

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