[Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia].
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.
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
N
N
Klossowski
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
S A
SA
Braun
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
V
V
von Gruben
Praxis Hämatologie und Onkologie Neuss, Neuss, Deutschland.
C
C
Losem
Praxis Hämatologie und Onkologie Neuss, Neuss, Deutschland.
D
D
Plewe
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
B
B
Homey
Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland. Stephan.Meller@uni-duesseldorf.de.
S
S
Meller
D000799:Angioedema / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D000970:Antineoplastic Agents / Q000008:administration & dosage; D002908:Chronic Disease; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination / Q000379:methods; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D000069444:Omalizumab / Q000008:administration & dosage; D000069283:Rituximab / Q000008:administration & dosage; D016896:Treatment Outcome; D014581:Urticaria / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*
D000970:Antineoplastic Agents; D003174:Complement C1 Inactivator Proteins; D007166:Immunosuppressive Agents; D000069444:Omalizumab; D000069283:Rituximab
Icatibant; Lymphoproliferative disorders; Omalizumab; Rituximab; Urticaria