Conflict of interest The authors declared no conflicts of interest.
Localized Wegener’s granulomatosis
Article first published online: 17 JAN 2011
© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology
Journal of the European Academy of Dermatology and Venereology
Volume 25, Issue 12, pages 1466–1470, December 2011
How to Cite
Marzano, A.V., Balice, Y., Papini, M., Testa, R., Berti, E. and Crosti, C. (2011), Localized Wegener’s granulomatosis. Journal of the European Academy of Dermatology and Venereology, 25: 1466–1470. doi: 10.1111/j.1468-3083.2010.03970.x
- Issue published online: 14 NOV 2011
- Article first published online: 17 JAN 2011
- Received: 30 July 2010; Accepted: 20 December 2010
- anti-neutrophil cytoplasmic antibodies;
- granulomatous necrotizing vasculitis;
- Wegener’s granulomatosis
Background Wegener’s granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease.
Objectives Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG.
Methods The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied.
Results All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti-neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide.
Limitations The limited number of patients is counterbalanced by the rarity of the disease.
Conclusions Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.