title: Fulminant primary manifestations of Wegener's granulomatosis might not be pauci-immune.
abstract: Wegener's granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener's granulomatosis and multi-organ involvement (upper respiratory tract, lung, kidneys, skin and general symptoms), who showed pronounced immunoglobulin and complement deposits within the skin biopsy. Our observation supports the hypothesis that immune complex deposits may be under-recognized in early lesions of ANCA-associated Wegener's granulomatosis.
authors.affiliation: Nephrology Division, Department I of Internal Medicine.
authors.forename: Ulf
authors.initials: U
authors.lastname: Schönermarck
authors.affiliation: Department of Dermatology and Allergy.
authors.forename: Maja
authors.initials: M
authors.lastname: Grahovac
authors.affiliation: Department of Dermatology and Allergy.
authors.forename: Miklós
authors.initials: M
authors.lastname: Sárdy
authors.affiliation: Department of Anaesthesiology , University Hospital Munich-Grosshadern, Ludwig-Maximilians-University, Munich , Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Dolch
authors.affiliation: Department of Dermatology and Allergy.
authors.forename: Andreas
authors.initials: A
authors.lastname: Wollenberg
keywords: ANCA; Wegener’s granulomatosis; pauci-immune; vasculitis