Localized Wegener's granulomatosis

A. V. Marzano, Y. Balice, M. Papini, R. Testa, E. Berti, C. Crosti

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1466-1470
Number of pages5
JournalJournal of the European Academy of Dermatology and Venereology
Volume25
Issue number12
DOIs
Publication statusPublished - Dec 2011

Fingerprint

Granulomatosis with Polyangiitis
Nasal Cartilages
Antineutrophil Cytoplasmic Antibodies
Skin
Nasal Mucosa
Immunosuppressive Agents
Vasculitis
Prednisone
Cyclophosphamide
Cartilage
Medical Records
Mouth
Mucous Membrane
Kidney
Bone and Bones
Lung

Keywords

  • anti-neutrophil cytoplasmic antibodies
  • granulomatous necrotizing vasculitis
  • skin
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Dermatology
  • Infectious Diseases

Cite this

Localized Wegener's granulomatosis. / Marzano, A. V.; Balice, Y.; Papini, M.; Testa, R.; Berti, E.; Crosti, C.

In: Journal of the European Academy of Dermatology and Venereology, Vol. 25, No. 12, 12.2011, p. 1466-1470.

Research output: Contribution to journalArticle

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