Naslov
Pathology quiz case 1. Wegener granulomatosis

Autori
Kovač, Lana ; Bumber, Boris ; Bilić, Mario ; Hutinec, Zdenka ; Topić, Iva

Izvornik
Archives of otolaryngology-head & neck surgery (0886-4470) 134 (2008), 10; 1116-1118

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, stručni

Ključne riječi
Wegeners granulomatosis; systemic vasculitis

Sažetak
Wegener's granulomatosis (WG) is an uncommon systemic vasculitis that typically involves upper airways, lungs and kidneys, but any organ can be involved including the skin. About 15% of patients will develop skin lesions at some point during the course of disease. The most common cutaneous presentation in WG is palpable purpura and necrotic ulceration. It can appear at the same time or after the signs of the disease in other organs such as lungs and kidneys have occurred. The most common cutaneous histopathologic presentation of WG is leukocytoclastic vasculitis, however, granulomatous inflammation with or without vasculitis and palisaded extravascular granulomas may also occur. Before immunosuppressive therapy, WG was associated with a very high mortality rate. Glucocorticoids combined with cyclophosphamide or methotrexate are the only two regimens that have been shown to induce remission of active WG with major organ involvement.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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