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International journal of Immunopathology, allergology, infectology.

Hypocomplementemic urticarial vasculitis syndrome

Kolkhir P.V.

Department of Skin and Venereal Diseases of I.M. Sechenov‘s First Moscow State Medical University, Moscow, Russia

Background: Different autoimmune diseases can present with organs malfunctioning and chronic urticaria symptoms in particular such a rare disease as hypocomplementemic urticarial vasculitis syndrome (HUVS). To improve diagnosis of HUVS by specialists, we present a literature review.
Method: Review of selected literature.
Results and conclusions: In addition to chronic urticaria symptoms, HUVS is characterized by severe systemic manifestations in different organs, decreased serum level of complement components, and appearance of specific markers, such as anti-C1q. The diagnosis is confirmed by skin biopsy, which is the “gold standard” of diagnosis. The condition often requires combined treatment with two immunosuppressive drugs. HUVS is a very rare disease, but, because of the multisystem manifestations, it can be encountered by any specialist (allergist, rheumatologist, ophthalmologist, dermatologist, nephrologist, general practitioner, pediatrician, etc.), therefore doctors should consider this condition when dealing with such patients in order to reduce the risk of HUVS misdiagnosis as chronic urticaria or another immunological disease.


Urticarial vasculitis, HUVS, hypocomplementemia, chronic urticaria, autoimmune disease

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Kolkhir P.V. Immunopathology, allergology, infectology 2013; 1:41-52. DOI: 10.14427/jipai.2013.1.41