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

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Skin Manifestations in Rheumatic Disease

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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.

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References

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  3. Lie JT. Wegener’s granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa. 1997;26:261–70.

    PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  5. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  7. Kluger N, Francès C. Cutaneous vasculitis and their differential diagnoses. Clin Exp Rheumatol. 2009;27 Suppl 2:S124–38.

    PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  9. Patten SF, Tomecki KJ. Wegener’s granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol. 1993;28:710–8.

    Article  PubMed  CAS  Google Scholar 

  10. Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003;48:311–40.

    Article  PubMed  Google Scholar 

  11. Cybulska A, Undas A, Sydor WJ, Flak A, Musial A. Wegener’s granulomatosis with massive skin necrosis. J Rheumatol. 2004;31:830–1.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology-Allergology, Pierre et Marie Curie University, Assistance Publique-Hôpitaux de Paris, Tenon Hospital, 4 rue de la Chine, Paris, 75020, France

    Camille Francès M.D.

  2. Division of Rheumatology, Mount Sinai Hospital/University Health Network, The Rebecca MacDonald Centre for Arthritis and Autoimmunity, 60 Murray Street, Ste 2-220, Toronto, ON, M5T 3L9, Canada

    Christian Pagnoux M.D., MPH

  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 Paris, Université Paris Descartes, 27, rue du Faubourg Saint-Jacques, Paris Cedex 14, 75679, France

    Loïc Guillevin M.D.

Authors
  1. Camille Francès M.D.
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  2. Christian Pagnoux M.D., MPH
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  3. Loïc Guillevin M.D.
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Corresponding author

Correspondence to Loïc Guillevin M.D. .

Editor information

Editors and Affiliations

  1. Division of Rheumatology, University of Florence Department of Biomedicine, Florence, Italy

    Marco Matucci-Cerinic

  2. Division of Rheumatology, UCLA Medical Center Department of Medicine, Los Angeles, California, USA

    Daniel Furst

  3. Department of Dermatology, Stanford University School of Medicine, Redwood City, California, USA

    David Fiorentino

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Francès, C., Pagnoux, C., Guillevin, L. (2014). Dermatologic Manifestations of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis). In: Matucci-Cerinic, M., Furst, D., Fiorentino, D. (eds) Skin Manifestations in Rheumatic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-7849-2_35

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  • DOI: https://doi.org/10.1007/978-1-4614-7849-2_35

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-7848-5

  • Online ISBN: 978-1-4614-7849-2

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