Hypocomplementemic urticarial vasculitis syndrome — a case report
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Abstract
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon small-vessel vasculitis presenting as a chronic urticaria with hypocomplementemia and the presence of anti-C1q autoantibodies. In most patients it is accompanied by a wide variety of extracutaneous manifestations such as arthritis, glomerulonephritis, ocular inflammation and pulmonary disease. We present a case of 49-year-old male with a 3-year history of recurrent urticaria accompanied by arthralgia, myalgia and gastrointestinal symptoms. The additional laboratory assessments revealed the presence of circulating anti-C1q autoantibodies in high titer. Histology of the lesional skin biopsy was consistent with leukocytoclastic vasculitis. The diagnosis of HUVS was confirmed in accordance to the currently adopted criteria. The described case emphasizes the necessity of high clinical suspicion when caring for patients with chronic urticaria and concomitant systemic symptoms.
Abstract
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon small-vessel vasculitis presenting as a chronic urticaria with hypocomplementemia and the presence of anti-C1q autoantibodies. In most patients it is accompanied by a wide variety of extracutaneous manifestations such as arthritis, glomerulonephritis, ocular inflammation and pulmonary disease. We present a case of 49-year-old male with a 3-year history of recurrent urticaria accompanied by arthralgia, myalgia and gastrointestinal symptoms. The additional laboratory assessments revealed the presence of circulating anti-C1q autoantibodies in high titer. Histology of the lesional skin biopsy was consistent with leukocytoclastic vasculitis. The diagnosis of HUVS was confirmed in accordance to the currently adopted criteria. The described case emphasizes the necessity of high clinical suspicion when caring for patients with chronic urticaria and concomitant systemic symptoms.
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Keywords
hypocomplementemic urticarial vasculitis syndrome; urticaria; vasculitis; autoinflammatory disease; hypocomplementemia
About this articleTitle
Hypocomplementemic urticarial vasculitis syndrome — a case report
Journal
Issue
Article type
Case report
Pages
8-11
Published online
2017-02-22
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3575
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2422
Bibliographic record
Forum Dermatologicum 2017;3(1):8-11.
Keywords
hypocomplementemic urticarial vasculitis syndrome
urticaria
vasculitis
autoinflammatory disease
hypocomplementemia
Authors
Dominik Samotij
Marta Wojciechowska-Zdrojowy
Adrian Pona
Adam Reich
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