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Related Experiment Video

Updated: May 27, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

Localized Wegener's granulomatosis.

A V Marzano1, Y Balice, M Papini

  • 1UO Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Dipartimento di Anestesiologia, Terapia Intensiva e Scienze Dermatologiche, Università degli Studi di Milano, Milan, Italy. angelovalerio.marzano@policlinico.mi.it

Journal of the European Academy of Dermatology and Venereology : JEADV
|November 15, 2011
PubMed
Summary

Localized Wegener's granulomatosis (WG) can present as aggressive facial and upper airway lesions. This rare subset of WG, even with negative ANCA, may respond to prednisone and cyclophosphamide.

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Area of Science:

  • Rheumatology
  • Vasculitis Research
  • Dermatology

Background:

  • Wegener's granulomatosis (WG) is a rare vasculitis affecting small to medium vessels, typically impacting upper airways, lungs, and kidneys.
  • While mucocutaneous lesions are common, localized WG presenting solely in these areas is rarely reported.
  • This study focuses on localized WG, examining its unique presentation and characteristics.

Purpose of the Study:

  • To investigate the clinical presentation, treatment response, and histological findings of localized Wegener's granulomatosis.
  • To characterize the specific types and locations of skin and mucosal lesions in localized WG.
  • To evaluate the disease course and treatment efficacy in patients with localized WG.

Main Methods:

  • Retrospective analysis of medical records from three patients (two female, one male) diagnosed with localized WG.

Related Experiment Videos

Last Updated: May 27, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

  • Mean follow-up duration of 10 years.
  • Evaluation of clinical, histological, and laboratory data, including anti-neutrophil cytoplasmic antibody (ANCA) status.

    • Main Results:

    • All patients exhibited facial plaques with infiltration of nasal and palatal mucosae and cartilages, with one case involving palatal bone perforation.
    • Anti-neutrophil cytoplasmic antibodies (ANCA) were negative in all patients.
    • The condition was refractory to various immunosuppressants but showed an incomplete but positive response to prednisone combined with cyclophosphamide.

    Conclusions:

    • The presented cases suggest a rare subset of WG localized to the facial region and upper airway mucosa.
    • This localized form exhibits locally aggressive behavior, leading to significant cartilage and bony destruction.
    • Despite the rarity and limited patient numbers, these findings highlight a distinct clinical entity within WG.