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

  • Camille Francès
  • Christian Pagnoux
  • Loïc GuillevinEmail author


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.


Pyoderma Gangrenosum Polyarteritis Nodosa Leukocytoclastic Vasculitis Necrotizing Vasculitis Strauss Syndrome 
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  1. 1.
    Guillevin L, Cordier JF, Lhote F, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener’s granulomatosis. Arthritis Rheum. 1997;40:2187–98.PubMedCrossRefGoogle Scholar
  2. 2.
    Pinching AJ, Lockwood CM, Pussell BA, et al. Wegener’s granulomatosis: observations on 18 patients with severe renal disease. Q J Med. 1983;52:435–60.PubMedGoogle Scholar
  3. 3.
    Lie JT. Wegener’s granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa. 1997;26:261–70.PubMedGoogle Scholar
  4. 4.
    Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98:76–85.PubMedCrossRefGoogle Scholar
  5. 5.
    Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.PubMedCrossRefGoogle Scholar
  6. 6.
    Daoud MS, Gibson LE, De Remee RA, Specks U, El-Azhary RA, Su WPD. Cutaneous Wegener’s granulomatosis: clinical, histopathologic and immunopathologic features of thirty patients. J Am Acad Dermatol. 1994;31:605–12.PubMedCrossRefGoogle Scholar
  7. 7.
    Kluger N, Francès C. Cutaneous vasculitis and their differential diagnoses. Clin Exp Rheumatol. 2009;27 Suppl 2:S124–38.PubMedGoogle Scholar
  8. 8.
    Francès C, Lê Thi Huong D, Piette JC, et al. Wegener’s granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994;130:861–7.PubMedCrossRefGoogle Scholar
  9. 9.
    Patten SF, Tomecki KJ. Wegener’s granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol. 1993;28:710–8.PubMedCrossRefGoogle Scholar
  10. 10.
    Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003;48:311–40.PubMedCrossRefGoogle Scholar
  11. 11.
    Cybulska A, Undas A, Sydor WJ, Flak A, Musial A. Wegener’s granulomatosis with massive skin necrosis. J Rheumatol. 2004;31:830–1.PubMedGoogle Scholar
  12. 12.
    Manchanda Y, Tejasvi T, Handa R, Ramam M. Strawberry gingiva: a distinctive sign in Wegener’s granulomatosis. J Am Acad Dermatol. 2003;49:335–7.PubMedCrossRefGoogle Scholar
  13. 13.
    Comfere NI, Macaron NC, Gibson LE. Cutaneous manifestations of Wegener’s granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol. 2007;34:739–47.PubMedCrossRefGoogle Scholar
  14. 14.
    Finan MC, Winkelmann RK. The cutaneous extravascular necrotizing granuloma (Churg–Strauss granuloma) and systemic disease: a review of 27 cases. Medicine (Baltimore). 1983;62:142–58.Google Scholar
  15. 15.
    Wilmoth GJ, Perniciaro C. Cutaneous extravascular necrotizing granuloma (Winkelmann granuloma): confirmation of the association with systemic disease. J Am Acad Dermatol. 1996;34:753–9.PubMedCrossRefGoogle Scholar
  16. 16.
    Obermoser G, Zelger B, Zangerle R, Sepp N. Extravascular necrotizing palisaded granulomas as the presenting skin sign of systemic lupus erythematosus. Br J Dermatol. 2002;147:371–4.PubMedCrossRefGoogle Scholar
  17. 17.
    Brons RH, de Jong MC, de Boer NK, Stegeman CA, Kallenberg CG, Tervaert JW. Detection of immune deposits in skin lesions of patients with Wegener’s granulomatosis. Ann Rheum Dis. 2001;60:1097–102.PubMedCrossRefGoogle Scholar

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© 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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