Wegener's granulomatosis.

Abstract

Wegener's granulomatosis is a fatal systemic disease; its course may be acute or chronic. Pathologically it is identified by three constant abnormal findings; this triad is pathognomonic of the syndrome: Necrotizing granulomatous ulceration of the upper portion of the respiratory tract. Granulomatous inflammation of the lungs. Focal glomerulitis. Lurking in the background there is to be found a disseminate necrotizing vasculitis affecting both arteries and veins. Clinically, the illness is characterized by signs of rhinitis, severe sinusitis accompanied by ulceration, and necrosis of the tissues of the central portion of the face. Following these initial findings there appears recurrent pneumonitis along with a wide diversity of bizarre signs and symptoms resulting from systemic vascular involvement. Finally, signs of kidney disease appear ending in uremia. Although these profound changes are of a systemic nature, Wegener's granulomatosis is of considerable dermatologic interest; often the initial visible lesion may be a cutaneous granuloma, and later in the course of the disease other types of skin involvement may be observed, particularly subcutaneous nodules which are often found over the path of small arteries. Indeed, the