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Dermatologic Manifestations of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)

  • Camille Francès
  • Christian Pagnoux
  • Loïc GuillevinEmail author
Chapter

Abstract

Granulomatosis with polyangiitis (Wegener’s granulomatosis; GPA) is a small-sized vessel ANCA-associated vasculitis. Dermatologic lesions may be the initial manifestations (8–13 %) or occur later during the course of the disease (12–67 % of the patients). Most frequent skin manifestations include purpura, mainly on the lower limbs, sometimes necrotic, papulonecrotic lesions, subcutaneous nodules, livedo reticularis, mucosal and skin ulcerations or ulcers, and gangrene. Hypertrophic gingivitis is another rare but suggestive manifestation. On histology, purpura is usually secondary to leukocytoclastic vasculitis, whereas papulonecrotic lesions and ulcerations correspond to leukocytoclastic or granulomatous vasculitis involving small vessels or extravascular granuloma(s). Small nodules mainly reflect necrotizing vasculitis involving medium-sized arterioles of the deep dermis or hypodermis that may be suggestive of polyarteritis nodosa for the pathologist.

Keywords

Pyoderma Gangrenosum Polyarteritis Nodosa Leukocytoclastic Vasculitis Necrotizing Vasculitis Strauss Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  • Camille Francès
    • 1
  • Christian Pagnoux
    • 2
  • Loïc Guillevin
    • 3
    Email author
  1. 1.Department of Dermatology-AllergologyPierre et Marie Curie University, Assistance Publique-Hôpitaux de Paris, Tenon HospitalParisFrance
  2. 2.Division of RheumatologyMount Sinai Hospital/University Health Network, The Rebecca MacDonald Centre for Arthritis and AutoimmunityTorontoCanada
  3. 3.Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Necrotizing Vasculitides and Systemic Sclerosis, Hôpital Cochin, Assistance Publique–Hôpitaux de ParisUniversité Paris DescartesParis Cedex 14France

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