Rheumatology International

, Volume 38, Issue 6, pp 1139–1151 | Cite as

Pyoderma gangrenosum-like ulcerations in granulomatosis with polyangiitis: two cases and literature review

  • Giovanni Genovese
  • Simona Tavecchio
  • Emilio Berti
  • Franco Rongioletti
  • Angelo Valerio MarzanoEmail author
Cases with a Message


Granulomatosis with polyangiitis (GPA) is a systemic necrotizing small vessel vasculitis associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Skin manifestations, mostly represented by palpable purpura, papulonodular lesions and livedo reticularis, are present in up to 50% of the cases. Ulcerations with undermined, raised erythematous–violaceous border resembling pyoderma gangrenosum (PG) have rarely been reported as skin involvement in GPA. The presence of circulating ANCAs with a cytoplasmic labelling pattern, the involvement of internal organs, particularly of the lung, and the absence on histology of a mainly neutrophilic infiltrate in early phases of the cutaneous lesions may be regarded as clues to rule out true PG and confirm the diagnosis of GPA skin ulcerations simulating PG. Herein, we describe two paradigmatic cases of such a unique presentation of GPA and a literature review focusing on clinicopathological features of GPA presenting with PG-like ulcerations in the skin has been provided. Moreover, referring to the scenario observed in these two cases, an easy-to-use working approach for the differential diagnosis between the two conditions has also been proposed.


Granulomatosis with polyangiitis ANCA-associated vasculitis Pyoderma gangrenosum 



We would like to thank Dr. Maria Laura Jannone Molaroni for her help in reviewing and interpreting CT scans and Dr. Thomas Fazio for his assistance in English language editing.

Author contributions

The cases were diagnosed and followed up by AVM, FR and EB. AVM, FR and GG conceived and planned the presented case report. GG and AVM wrote the manuscript with support from ST, EB and FR. Pathologic evaluation was performed by EB and FR. Literature data were analyzed by GG and ST under the guidance of AVM. All authors read and approved the final manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Written informed consent was obtained from the patients prior to submission of these case reports and any accompanying images.


  1. 1.
    Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11. CrossRefPubMedGoogle Scholar
  2. 2.
    Lutalo PM, D’Cruz DP (2014) Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun 48–49:94–98. CrossRefPubMedGoogle Scholar
  3. 3.
    Marzano AV, Raimondo MG, Berti E, Meroni PL, Ingegnoli F (2017) Cutaneous manifestations of ANCA-associated small vessels vasculitis. Clin Rev Allergy Immunol 53:428–438. CrossRefPubMedGoogle Scholar
  4. 4.
    Marzano AV, Vezzoli P, Berti E (2013) Skin involvement in cutaneous and systemic vasculitis. Autoimmun Rev 12:467–476. PubMedCrossRefGoogle Scholar
  5. 5.
    Daoud MS, Gibson LE, DeRemee RA, Specks U, el-Azhary RA, Su WP (1994) Cutaneous Wegener’s granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol 31:605–612CrossRefPubMedGoogle Scholar
  6. 6.
    Reed WB, Jensen AK, Konwaler BE (1963) The cutaneous manifestations in Wegener’s granulomatosis. Acta Derm Venereol 43:250–264PubMedGoogle Scholar
  7. 7.
    Frumholtz L, Laurent-Roussel S, Aumaître O et al (2017) Clinical and pathological significance of cutaneous manifestations in ANCA-associated vasculitides. Autoimmun Rev 16:1138–1146. CrossRefPubMedGoogle Scholar
  8. 8.
    Kesseler ME (1982) Wegener’s granulomatosis. Clin Exp Dermatol 7:103–108CrossRefPubMedGoogle Scholar
  9. 9.
    Lerner EA, Dover JS (1986) Malignant pyoderma: a manifestation of Wegener’s granulomatosis? J Am Acad Dermatol 15:1051–1052CrossRefPubMedGoogle Scholar
  10. 10.
    Handfield-Jones SE, Parker SC, Fenton DA, Newton JA, Greaves MW (1992) Wegener’s granulomatosis presenting as pyoderma gangrenosum. Clin Exp Dermatol 17:197–200CrossRefPubMedGoogle Scholar
  11. 11.
    Boudny C, Nievergelt H, Braathen LR, Simon D (2008) Wegener’s granulomatosis presenting as pyoderma gangrenosum. J Dtsch Dermatol Ges 6:477–479. CrossRefPubMedGoogle Scholar
  12. 12.
    Feighery C, Conlon N, Abuzakouk M (2010) Skin ulcer presentation of Wegener’s granulomatosis. BMJ Case Rep 23:2010. CrossRefGoogle Scholar
  13. 13.
    Sen M, Dogra S, Rathi M, Sharma A (2016) Successful treatment of large refractory pyoderma gangrenosum-like presentation of granulomatosis with polyangiitis with rituximab. Int J Rheum Dis 20:2200–2202. CrossRefPubMedGoogle Scholar
  14. 14.
    Kass A, Fagan JD, Long P (2017) Granulomatosis with polyangiitis presenting with pyoderma gangrenosum-like ulceration and negative cytoplasmic antineutrophilic cytoplasmic antibodies in a child. Pediatr Dermatol 34:231–234. CrossRefGoogle Scholar
  15. 15.
    Kindle SA, Camilleri MJ, Gibson LE, Davis MD (2017) Granulomatosis with polyangiitis mimicking classic inflammatory bowel disease-associated pyoderma gangrenosum. Int J Dermatol 56:e1-e3. CrossRefPubMedGoogle Scholar
  16. 16.
    Zarraga MB, Swenson N, Glick B (2017) Pyoderma Gangrenosum-associated granulomatosis with polyangiitis: a case report and literature review. J Clin Aesthet Dermatol 10:40–42PubMedPubMedCentralGoogle Scholar
  17. 17.
    de Cambourg G, Mahé A, Banea S, Moulinas C, Blaison G (2016) Granulomatosis with polyangiitis revealed by a cutaneous ulceration mimicking a pyoderma gangrenosum: two patients. Rev Med Interne 37:632–635. CrossRefPubMedGoogle Scholar
  18. 18.
    Edwards MB, Buckerfield JP (1978) Wegener’s granulomatosis: a case with primary mucocutaneous lesions. Oral Surg Oral Med Oral Pathol 46:53–63CrossRefPubMedGoogle Scholar
  19. 19.
    Chyu JY, Hagstrom WJ, Soltani K, Faibisoff B, Whitney DH (1984) Wegener’s granulomatosis in childhood: cutaneous manifestations as the presenting signs. J Am Acad Dermatol 10:341–346CrossRefPubMedGoogle Scholar
  20. 20.
    Micali G, Cook B, Ronan S, Yadgir J, Solomon LM (1994) Cephalic pyoderma gangrenosum (PG)-like lesions as a presenting sign of Wegener’s granulomatosis. Int J Dermatol 33:477–480CrossRefPubMedGoogle Scholar
  21. 21.
    Kouba DJ, Mimouni D, Ha CT, Nousari CH (2003) Limited Wegener’s granulomatosis manifesting as malignant pyoderma with corneal melt. Int J Dermatol 42:902–904CrossRefPubMedGoogle Scholar
  22. 22.
    Sinovich V, Snow J (2003) Protracted superficial Wegener’s granulomatosis. Australas J Dermatol 44:207–214CrossRefPubMedGoogle Scholar
  23. 23.
    Jacob SE, Martin LK, Kerdel FA (2003) Cutaneous Wegener’s granulomatosis (malignant pyoderma) in a patient with Crohn’s disease. Int J Dermatol 42:896–898CrossRefPubMedGoogle Scholar
  24. 24.
    Wright AC, Gibson LE, Davis DM (2015) Cutaneous manifestations of pediatric granulomatosis with polyangiitis: a clinicopathologic and immunopathologic analysis. J Am Acad Dermatol 72:859–867. CrossRefPubMedGoogle Scholar
  25. 25.
    Belliveau MJ, Jakubovic BD, Mahendira D, Jakubovic HR, Nijhawan N (2016) Necrotizing eyelid inflammation heralding granulomatosis with polyangiitis. Am J Med 129:e7-8. CrossRefPubMedGoogle Scholar
  26. 26.
    Haun P, Wanchu A, Korcheva V, Fett N (2016) A preauricular ulcer in a patient with conjunctival injection and persistent lacrimation: challenge. Am J Dermatopathol 38:211, 244–245. CrossRefGoogle Scholar
  27. 27.
    Aymard B, Bigard MA, Thompson H, Schmutz JL, Finet JF, Borrelly J (1990) Perianal ulcer: an unusual presentation of Wegener’s granulomatosis. Report of a case. Dis Colon Rectum 33:427–430CrossRefPubMedGoogle Scholar
  28. 28.
    Perkins GD, Moudgil H, Jones R (2000) Pyoderma gangrenosum. Thorax 55:347CrossRefPubMedGoogle Scholar
  29. 29.
    Tashtoush B, Memarpour R, Johnston Y, Ramirez J (2014) Large pyoderma gangrenosum-like ulcers: a rare presentation of granulomatosis with polyangiitis. Case Rep Rheumatol 2014:850364. PubMedPubMedCentralCrossRefGoogle Scholar
  30. 30.
    Wallach D, Vignon-Pennamen MD (2006) From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research. J Am Acad Dermatol 55:1066–1071CrossRefPubMedGoogle Scholar
  31. 31.
    Marzano AV, Borghi A, Wallach D, Cugno M (2017) A comprehensive review of neutrophilic diseases. Clin Rev Allergy Immunol 55:114–130. CrossRefGoogle Scholar
  32. 32.
    Marzano AV, Borghi A, Meroni PL, Cugno M (2016) Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation. Br J Dermatol 175:882–891. CrossRefPubMedGoogle Scholar
  33. 33.
    Marzano AV, Damiani G, Ceccherini I, Berti E, Gattorno M, Cugno M (2017) Autoinflammation in pyoderma gangrenosum and its syndromic form (pyoderma gangrenosum, acne and suppurative hidradenitis). Br J Dermatol 176:1588–1598. CrossRefPubMedGoogle Scholar
  34. 34.
    Marzano AV, Cugno M, Trevisan V, Fanoni D, Venegoni L, Berti E, Crosti C (2010) Role of inflammatory cells, cytokines and matrix metalloproteinases in neutrophil-mediated skin diseases. Clin Exp Immunol 162:100–107. CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Marzano AV, Fanoni D, Antiga E, Quaglino P, Caproni M, Crosti C, Meroni PL, Cugno M (2014) Expression of cytokines, chemokines and other effector molecules in two prototypic autoinflammatory skin diseases, pyoderma gangrenosum and Sweet’s syndrome. Clin Exp Immunol 178:48–56. CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Wollina U (2007) Pyoderma gangrenosum—a review. Orphanet J Rare Dis 2:19. CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Hong SB, Lee MH (2004) A case of propylthiouracil-induced pyoderma gangrenosum associated with antineutrophil cytoplasmic antibody. Dermatology 208:339–341.
  38. 38.
    Thomas RH, Payne CM, Black MM (1982) Wegener’s granulomatosis presenting as pyoderma gangrenosum. Clin Exp Dermatol 7:523–527CrossRefPubMedGoogle Scholar
  39. 39.
    Szõcs HI, Torma K, Petrovicz E, Hársing J, Fekete G, Kárpáti S, Horváth A (2003) Wegener’s granulomatosis presenting as pyoderma gangrenosum. Int J Dermatol 42:898–902CrossRefPubMedGoogle Scholar
  40. 40.
    Wong YW, Lyon CC, Benbow EW, Bradley BL, Beck MH (2003) Pyoderma gangrenosum in a thoracotomy wound associated with a pulmonary cavitating lesion. Clin Exp Dermatol 28:274–276CrossRefPubMedGoogle Scholar
  41. 41.
    Le Hello C, Bonte I, Mora JJ, Verneuil L, Noël LH, Guillevin L (2002) Pyoderma gangrenosum associated with Wegener’s granulomatosis: partial response to mycophenolate mofetil. Rheumatology 41:236–237CrossRefPubMedGoogle Scholar
  42. 42.
    Donmez S, Pamuk ON, Gedik M, AKR, Bulut G (2014) A case of granulomatosis with polyangiitis and pyoderma gangrenosum successfully treated with infliximab and rituximab. Int J Rheum Dis 17:471–475. CrossRefPubMedGoogle Scholar
  43. 43.
    Murthy RK, Jackson J, Chatham WW, Sami N (2016) Extensive pyoderma gangrenosum associated with granulomatosis with polyangiitis with both responsive to rituximab. J Clin Rheumatol 22:393–395. CrossRefPubMedGoogle Scholar
  44. 44.
    Gibson LE, Daoud MS, Muller SA, Perry HO (1997) Malignant pyodermas revisited. Mayo Clin Proc 72:734–736CrossRefPubMedGoogle Scholar
  45. 45.
    Gastman B, Hashem AM, Djohan R et al (2016) Malignant pyoderma associated with granulomatosis with polyangiitis (Wegener Granulomatosis) as a unique indication for facial vascularized composite allotransplantation: Part I. Plast Reconstr Surg 137:1007–1015. CrossRefGoogle Scholar
  46. 46.
    Holle JU, Gross WL, Holl-Ulrich K, Ambrosch P, Noelle B, Both M, Csernok E, Moosig F, Schinke S, Reinhold-Keller E (2010) Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis 69:1934–1939. CrossRefPubMedGoogle Scholar
  47. 47.
    Marzano AV, Balice Y, Papini M, Testa R, Berti E, Crosti C (2011) Localized Wegener’s granulomatosis. J Eur Acad Dermatol Venereol 25:1466–1470. CrossRefPubMedGoogle Scholar
  48. 48.
    Jiménez-Gallo D, Albarrán-Planelles C, Linares-Barrios M, Rodríguez-Hernández C, Martínez-Rodríguez A, García-Moreno E, Bravo-Monge R (2013) Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine. Clin Exp Dermatol 38:878–882. CrossRefPubMedGoogle Scholar
  49. 49.
    Jeong HS, Layher H, Cao L, Vandergriff T, Dominguez AR (2016) Pyoderma gangrenous associated with levamisole-adulterated cocaine: Clinical, serologic and histopathologic findings in a cohort of patients. J Am Acad Dermatol 74:892–898. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Dermatology Unit, Department of Physiopathology and TransplantationUniversità degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoMilanItaly
  2. 2.Section of Dermatology, Department of Medical Sciences and Public HealthUniversity of CagliariCagliariItaly

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