Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report.
10.3346/jkms.2017.32.12.2064
- Author:
Su Woong JUNG
1
;
Yun Young CHOI
;
In Seung CHOI
;
Seulki KIM
;
Kyung Hwan JEONG
;
Ran SONG
;
Sang Hoon LEE
;
Hyung In YANG
;
Seung Jae HONG
;
Yeon Ah LEE
Author Information
1. Department of Medicine, Graduate School, Kyung Hee University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Hypocomplementemic Urticarial Vasculitis Syndrome;
Membranous Nephropathy;
Myositis
- MeSH:
Angioedema;
Complement System Proteins;
Glomerulonephritis, Membranous*;
Humans;
Immunosuppressive Agents;
Influenza A virus;
Joints;
Kidney;
Lung;
Male;
Myositis;
Skin;
Vasculitis*
- From:Journal of Korean Medical Science
2017;32(12):2064-2068
- CountryRepublic of Korea
- Language:English
-
Abstract:
Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity.