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J Korean Med Sci. 2017 Dec;32(12):2064-2068. doi: 10.3346/jkms.2017.32.12.2064.

Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report.

Author information

1
Department of Medicine, Graduate School, Kyung Hee University, Seoul, Korea.
2
Division of Rheumatology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul, Korea.
3
Division of Nephrology, Asan Chungmu Hospital, Asan, Korea.
4
Division of Nephrology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul, Korea.
5
Division of Rheumatology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul, Korea. aprildaum@hanmail.net.

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.

KEYWORDS:

Hypocomplementemic Urticarial Vasculitis Syndrome; Membranous Nephropathy; Myositis

PMID:
29115092
PMCID:
PMC5680509
DOI:
10.3346/jkms.2017.32.12.2064
[Indexed for MEDLINE]
Free PMC Article

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