[Cutaneous manifestations as the initial presentation of Wegener's granulomatosis]

Wegener's granulomatosis is a rare systemic vasculitis, characterized by involvement of the upper airways, lungs and kidneys; other organs may also be affected. Cutaneous lesions occur frequently during the disease course but seldom as its initial presentation. Digital necrosis and splinter hemorrhages of fingernails are described, but very rarely.

We report the case of a 55-year-old man presenting multisystemic Wegener's granulomatosis with mucosal and cutaneous involvement at initial presentation: tongue and labial ulcers, digital necrosis, splinter hemorrhages of fingernails, and purpura. The PR3-ANCA titer was high. Imaging and the histological findings of both cutaneous and renal biopsies suggest a diagnosis of Wegener's granulomatosis. He was treated with prednisone, cyclophosphamide, and cotrimoxazole. Cutaneous and mucosal symptoms disappeared 6 weeks after treatment began.

The particularity of this case is the association of four separate cutaneous manifestations of Wegener's granulomatosis - two of them very rare - as the initial presentation of this multisystemic disease.