Takayasu arteritis and cutaneous necrotizing vasculitis

Takayasu arteritis (TA) is an inflammatory arteriopathy involving predomina ntly the aorta and its main branches. The disease evolves in two phases: a first, nonspecific inflammatory stage and a late 'pulseless' stage, in whic h complications related to arterial stenosis and aneurysm formation predomi nate. In both phases, skin manifestations, such as inflammatory nodules, er ythema-nodosum- and pyoderma-gangrenosum-like ulcers, have been described. We report 2 patients with TA, who had cutaneous necrotizing vasculitis as p resenting manifestation of the disease. A review of the literature revealed 8 similar cases. TA does not only involve large arteries, but also small b lood vessels. The observation that in TA the inflammatory process of the la rge arteries affects regions of the walls supplied by the vasa vasorum, the anatomy of which bears resemblance to the cutaneous vessel system, suggest s that primary involvement of small vessels contributes to the development of the clinicopathological features of TA. Knowledge of the skin manifestat ions associated with TA remains important for its diagnosis and prompt inst auration of lifesaving treatment Copyright (C) 2000 S. Karger AG, Basel.