Wegener's granulomatosis: Cutaneous and oral mucosal disease

Background : Wegener's granulomatosis is a complex disease that can be difficult to diagnose, especially if the classic triad of necrotizing granulomas of the respiratory tract, generalized vasculitis, and glomerulitis are not present. A delay in diagnosis may result in irreversible kidney damage and ultimately death. Objective : This study was done to identify clinical and histologic findings of the skin and oral mucosa that may aid in the diagnosis of this disease. Methods : The records of 40 patients with Wegener's granulomatosis were reviewed. Results : Of these patients 12 had cutaneous lesions, 2 had oral mucosal lesions, and 2 had both cutaneous and oral mucosal lesions. Cutaneous and oral mucosal lesions were a presenting sign in 10% and 5% of patients, respectively. Overall, 25% demonstrated at least one of the specific cutaneous histopathologic findings described for Wegener's granulomatosis, that is, necrotizing vasculitis, granulomatous vasculitis, and palisading granuloma. One patient had gingival hyperplasia with petechiae, considered to be pathognomonic for Wegener's granulomatosis. Conclusion : This study demonstrates that cutaneous or oral mucosal findings may serve as valuable diagnostic aids when evaluating a patient suspected of having Wegener's granulomatosis.