Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report

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.

A case of hypocomplementemic urticarial vasculitis syndrome with cold urticaria / 대한피부과학회지

A 38-year-old man presented with a 2-year history of recurreit irticaria, abdominal pain and arthralgia of his finger joints. These symptoms were not con rolled in spite of taking medication and had a tendence to be aggravated in the winter. Laboratory findings revealed hvpocomlementemia, hypergammaglobulinemia and a positive rect on with an ice cube test. Skin biopsy specimen taken from the urticarial lesion of the left upper arm showed leukocytoclastic vasculitis with perivascular neutrophilic infiltrition, nuclear dusts, and extravasation of red blood cells. We thus made the diagnosis of hypocomplementemic urtirarial vasculitis syndrome with cold urticaria. The patient was treatcd ith systemic corticosteroids and antihistamines.