MALDONADO, Jorge E    IGLESIAS-GAMARRA, Antonio. Hypocomplementemic urticarial vasculitis: Clarification of a historical fact. []. , 21, 2, pp.84-90. ISSN 0121-8123.

Hypocomplementemic urticarial vasculitis has received extraordinary interest in the medical literature since the original publication in 1973 in Mayo Clinic Proceedings. The index case was identified in 1968 as a distinct clinical entity, by one of the co-authors of the first publication and of the current review, which closes a cycle of more than 45 years. The differential clinical characteristics of hypocomplementemic urticarial vasculitis syndrome determined that the 2012 consensus on nomenclature of Vasculitis of the american rheumatology association, designated the syndrome as a separate entity with its own place among the inflammatory vascular diseases. In the last few years, and particularly in the last few months, hypocomplementemic urticarial vasculitis syndrome has been recognized as a monogenic form of lupus erythematosus, an observation that completes the historical sequence of the disease, and places it among the spectrum of complement disorders. The emphasis of the article is centered on the initial historical aspects of the process, that have the unique merit of being recorded by the principal author and witness, and not previously published, albeit privately known by prominent rheumatologists and immunologists. The historical review of the evolution of the syndrome is based on a review of the literature, relating subsequent observations until the end of 2013, when the syndrome was recognized as a monogenic form of lupus erythematosus.

: Hypocomplementemic urticarial vasculitis; Systemic lupus erythematosus; Vasculitis; Urticaria.

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