It is rare and commonly occurs in young women affecting larger arteries, commonly the aortic arch. It may lead to thrombosis and narrowing of the lumen of arteries feeding the extremities, hence the term pulseless disease . It does not affect the superficial vessels of the skin. In advanced cases, there may be secondary skin changes including erythema nodosum, pyodermatous ulcers, and urticaria. Histologically, there is a granulomatous vasculitis.
Older lesions have fibrosis with narrowing of the lumen and thrombosis.
Sternberg S. Diagnostic Surgical Pathology. Lipincott Williams and Wilkins 1999. Commonly Used Terms Vasculitis - See term.