Advertisement

Pyoderma Gangrenosum

  • Jyoti Yadav
  • Sanjay Singh
Chapter

Abstract

Pyoderma gangrenosum (PG) is a rare, non-infectious, inflammatory disease of unknown etiology, characterized by sterile neutrophilic infiltration of the skin. It is commonly associated with underlying systemic disease [1–4]. Pyoderma gangrenosum is a rare, non-infectious inflammatory neutrophilic dermatosis of unknown etiology characterised by ulcerations of the skin and often associated with systemic disorders. Clinically it has many variants- classic or ulcerative, pustular, bullous, vegetative and pyostomatitis vegetans. Diagnosis is by clinical features and exclusion of other causes. Treatment depends on extent o disease and presence of underlying disorders. Corticosteroids are the drug of choice.

Keywords

Inflammatory Bowel Disease Pyoderma Gangrenosum Collagenous Colitis Leukocyte Adhesion Deficiency Ecthyma Gangrenosum 
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.

Notes

Acknowledgements

Authors are grateful to Dr Najeeba Riyaz, MD, DVD, DNB, MRCP, Professor and Head, Department of Dermatology, Government Medical College, Calicut, Kerala, for providing the clinical photographs.

References

  1. 1.
    Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol. 1930;22:655–80.Google Scholar
  2. 2.
    Powell FC, Su WPD. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol. 1996;34:395–409.CrossRefPubMedGoogle Scholar
  3. 3.
    Callen JP. Pyoderma gangrenosum. Lancet. 1998;351:581–5.CrossRefPubMedGoogle Scholar
  4. 4.
    Bennett ML, Jackson JM, Jorizzo JL, et al. Pyoderma gangrenosum: a comparison of typical and atypical forms with an emphasis on time to remission. Medicine. 2000;79:37–46.CrossRefPubMedGoogle Scholar
  5. 5.
    Broq L. Nouvelle contribution a’ l’e’tude du phagedenisme geometrique. ANN Dermatol Syphiligr (Paris). 1916;6:1–39.Google Scholar
  6. 6.
    Greenstein AJ, Janowitz HD, Sachar DB. The extra-intestinal complications of Crohn’s disease and ulcerative colitis: a study of 700 patients. Medicine (Baltimore). 1976;55:401–12.CrossRefGoogle Scholar
  7. 7.
    Callen JP. Neutrophilic dermatoses. Dermatol Clin. 2002;20:409–19.CrossRefPubMedGoogle Scholar
  8. 8.
    Powell FC, Collins C. Pyoderma gangrenosum. Clin Dermatol. 2000;18:283–93.CrossRefPubMedGoogle Scholar
  9. 9.
    Marzano AV, Tourlaki A, Alessi E, Caputo R. Widespread idiopathic pyoderma gangrenosum evolved from ulcerative to vegetative type: a 10-year history with a recent response to infliximab. Clin Exp Dermatol. 2007;33:156–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Powell FC, Schroeter AL, Su WP, et al. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985;55:173–86.PubMedGoogle Scholar
  11. 11.
    Van den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137:1000–5.CrossRefPubMedGoogle Scholar
  12. 12.
    Dick DC, Mackie RM, Patrick WJA, et al. Pyoderma gangrenosum in infancy. Acta Derm Venereol. 1982;62:348–50.PubMedGoogle Scholar
  13. 13.
    Madke B, Pande S, Khopkar U. Phenomena in dermatology. Indian J Dermatol Venereol Leprol. 2011;77:264–75.CrossRefPubMedGoogle Scholar
  14. 14.
    Samitz MH. Cutaneous vasculitis in association with ulcerative colitis. Cutis. 1966;2:383–7.Google Scholar
  15. 15.
    Ebringer A, Doyles AE, Harris GS. Dermonecrotic factor I: nature and properties of a dermonecrotic factor to guinea pig skin found in human serum. Br J Exp Pathol. 1969;50:559–65.PubMedPubMedCentralGoogle Scholar
  16. 16.
    Samitz MH, Dana AS, Rosemberg P. Cutaneous vasculitis in association with Crohn’s disease: review of statistics of skin complications. Cutis. 1970;6:51–6.Google Scholar
  17. 17.
    Lotti T, Ghersetich I, Comacchi C, et al. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39:667–87.CrossRefPubMedGoogle Scholar
  18. 18.
    Marks DJB, Rahman FZ, Novelli M, et al. An exuberant inflammatory response to E. coli: implications for the pathogenesis of ulcerative colitis and pyoderma gangrenosum. Gut. 2006;55:1662–3.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Lazarus GS, Goldsmith LA, Rocklin RE, et al. Pyoderma gangrenosum, altered delayed hypersensitivity, and polyarthritis. Arch Dermatol. 1972;105:46–51.CrossRefPubMedGoogle Scholar
  20. 20.
    Brooklyn TN, Williams AN, Dunnill MGS, Probert CS. T-cell receptor repertoire in pyoderma gangrenosum: evidence for clonal expansions and trafficking. Br J Dermatol. 2007;157:960–6.CrossRefPubMedGoogle Scholar
  21. 21.
    Nerella P, Daniela A, Guido M, et al. Leukocyte chemotaxis and pyoderma gangrenosum. Int J Dermatol. 1985;24:45–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Jackson JM, Callen JP. Pyoderma gangrenosum: an expert commentary. Expert Rev Dermatol. 2006;1:391–400.CrossRefGoogle Scholar
  23. 23.
    Wollina U. Pyoderma gangrenosum—a review. Orphanet J Rare Dis. 2007;2:19.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Oka M, Berking C, Nesbit M, et al. Interleukin-8 overexpression is present in pyoderma gangrenosum ulcers and leads to ulcer formation in human skin xenografts. Lab Invest. 2000;80:595–604.CrossRefPubMedGoogle Scholar
  25. 25.
    Oka M. Pyoderma gangrenosum and interleukin-8. Br J Dermatol. 2007;157:1279–81.CrossRefPubMedGoogle Scholar
  26. 26.
    Manguso F, Sanges M, Staiano T, et al. Cigarette smoking and appendectomy are risk factors for extraintestinal manifestations in ulcerative colitis. Am J Gastroenterol. 2004;99:327–34.CrossRefPubMedGoogle Scholar
  27. 27.
    Hoffman MD. Pyoderma gangrenosum associated with c-ANCA (h-lamp-2). Int J Dermatol. 2001;40:135–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Alberts JH, Sams HH, Miller JL. Familial ulcerative pyoderma gangrenosum: a report of two kindreds. Cutis. 2002;69:427–30.PubMedGoogle Scholar
  29. 29.
    Khandpur S, Mehta S, Reddy BS. Pyoderma gangrenosum in two siblings: a familial predisposition. Pediatr Dermatol. 2001;18:308–12.CrossRefPubMedGoogle Scholar
  30. 30.
    Shoham NG, Centola M, Mansfield E, et al. Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway. Proc Natl Acad Sci U S A. 2003;100:13501–6.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Cox NH, Jorizzo JL, Bourke JF, Savage COS. Vasculitis, neutrophilic dermatosis and related disorders. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s textbook of dermatology. 8th ed. Chichester: Wiley Blackwell; 2010. p. 50.64–73.Google Scholar
  32. 32.
    Ahmadi S, Powell FC. Pyoderma gangrenosum: uncommon presentations. Clin Dermatol. 2005;23:612–20.CrossRefPubMedGoogle Scholar
  33. 33.
    Wilson-Jones E, Winkelmann RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol. 1988;18:511–21.CrossRefPubMedGoogle Scholar
  34. 34.
    Quimby SR, Gibson LE, Winkelmann RK. Superficial granulomatous pyoderma: clinicopathologic spectrum. Mayo Clin Proc. 1989;64:37–43.CrossRefPubMedGoogle Scholar
  35. 35.
    Lichter MD, Welykyj SE, Gradini R, et al. Superficial granulomatous pyoderma. Int J Dermatol. 1991;30:418–21.CrossRefPubMedGoogle Scholar
  36. 36.
    Su WPD, Davis MD, Weenig RH, et al. Pyoderma gangrenosum: clinicopathological correlation and proposed diagnostic criteria. Int J Dermatol. 2004;43:790–800.CrossRefPubMedGoogle Scholar
  37. 37.
    Moschella SL, Davis Mark DP. Neutrophilic dermatoses. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Bolognia’s textbook of dermatology. 3rd ed. USA: Elsevier Saunders; 2012. p. 427–31.Google Scholar
  38. 38.
    Vidal DG, Puig L, Gilaberte M, Alomar A, et al. Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features. J Dermatol Treat. 2004;15:146–52.CrossRefGoogle Scholar
  39. 39.
    Ackerman D, Abu-Elmagd K, Venkataramanan K, et al. Recalcitrant psoriasis and pyoderma gangrenosum treated with FK506. J Invest Dermatol. 1991;96:536.Google Scholar
  40. 40.
    Lee MR, Cooper MJ. Mycophenolate mofetil in pyoderma gangrenosum. J Dermatol Treat. 2004;15:303–7.CrossRefGoogle Scholar
  41. 41.
    Cox NH, Palmer JG. Bowel-associated dermatitis-arthritis syndrome associated with ileo-anal pouch anastomosis, and treatment with mycophenolate mofetil. Br J Dermatol. 2003;149:1296–7.CrossRefPubMedGoogle Scholar
  42. 42.
    Daniels LH, Callen JP. Mycophenolate mofetil is an effective treatment for peristomal pyoderma gangrenosum. Arch Dermatol. 2004;140:1427–9.CrossRefPubMedGoogle Scholar
  43. 43.
    Gettler S, Rothe M, Grin C, Grant-Kels J, et al. Optimal treatment of pyoderma gangrenosum. Am J Clin Dermatol. 2003;4:597–608.CrossRefPubMedGoogle Scholar
  44. 44.
    Stichweh DS, Punaro M, Pascual V. Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome. Pediatr Dermatol. 2005;22:262–5.CrossRefPubMedGoogle Scholar
  45. 44.
    Johnson RB, Lazarus GS. Pulse therapy: therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol. 1982;118:76–84.CrossRefPubMedGoogle Scholar
  46. 45.
    Pomerantz RG, Husni ME, Mody E, Qureshi AA. Adalimumab for treatment of pyoderma gangrenosum. Br J Dermatol. 2007;157:1274–5.CrossRefPubMedGoogle Scholar
  47. 46.
    Pastor N, Betlloch I, Pascua JC, et al. Pyoderma gangrenosum treated with anti-TNF alpha therapy (etanercept). Clin Exp Dermatol. 2005;31:152–3.CrossRefGoogle Scholar
  48. 47.
    Hubbard VG, Friedmann AC, Goldsmith P. Systemic pyoderma gangrenosum responding to infliximab and adalimumab. Br J Dermatol. 2005;152:1059–61.CrossRefPubMedGoogle Scholar
  49. 48.
    Reguiaï Z, Grange F. The role of anti-tumor necrosis factor-alpha therapy in pyoderma gangrenosum associated with inflammatory bowel disease. Am J Clin Dermatol. 2007;8:67–77.CrossRefPubMedGoogle Scholar
  50. 49.
    Munro CS, Cox NH. Pyoderma gangrenosum associated with Behçet’s syndrome: response to thalidomide. Clin Exp Dermatol. 1988;13:408–10.PubMedGoogle Scholar
  51. 50.
    Federman GL, Federman DG. Recalcitrant pyoderma gangrenosum treated with thalidomide. Mayo Clin Proc. 2000;75:842–4.CrossRefPubMedGoogle Scholar
  52. 51.
    Brenner M, Ruzicka T, Plewig G, Thomas P, Herzer P. Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra. Br J Dermatol. 2009;161:1199–201.CrossRefPubMedGoogle Scholar
  53. 52.
    Kaminska R, Ikaheimo R, Hollmen A. Plasmapheresis and cyclophosphamide successful treatments for pyoderma gangrenosum. Clin Exp Dermatol. 1999;24:81–5.CrossRefPubMedGoogle Scholar
  54. 53.
    Fujimoto E, Fujimoto N, Kuroda K, Tajima S. Leukocytapheresis treatment for pyoderma gangrenosum. Br J Dermatol. 2004;151:1090–2.CrossRefPubMedGoogle Scholar
  55. 54.
    Okuma K, Mitsuishi K, Hasegawa T, et al. A case report of steroid and immunosuppressant-resistant pyoderma gangrenosum successfully treated by granulocytapheresis. Therap Apher Dial. 2007;11:387–90.CrossRefGoogle Scholar
  56. 55.
    Dirschka T, Kastner U, Behrens S, et al. Successful treatment of pyoderma gangrenosum with intravenous human immunoglobulin. J Am Acad Dermatol. 1998;39:789–90.CrossRefPubMedGoogle Scholar
  57. 56.
    Cummins DL, Anhalt GJ, Monahan T, Meyerle JH. Treatment of pyoderma gangrenosum with intravenous immunoglobulin. Br J Dermatol. 2007;157:1235–9.CrossRefPubMedGoogle Scholar
  58. 57.
    Kreuter A, Reich-Schupke S, Stücker M, et al. Intravenous immunoglobulin for pyoderma gangrenosum. Br J Dermatol. 2008;158:856–7.CrossRefPubMedGoogle Scholar
  59. 58.
    Kontochristopoulos GJ, Stavropoulos PG, Gregoriou S, et al. Treatment of pyoderma gangrenosum with low-dose colchicine. Dermatology. 2004;209:233–6.CrossRefPubMedGoogle Scholar
  60. 59.
    Cliff S, Holden CA, Thomas PR, et al. Split skin grafts in the treatment of pyoderma gangrenosum. Ann Plast Surg. 2001;46:23–8.CrossRefGoogle Scholar
  61. 60.
    Limova M, Mauro T. Treatment of pyoderma gangrenosum with cultured keratinocyte autografts. J Dermatol Surg Oncol. 1994;20:833–6.CrossRefPubMedGoogle Scholar
  62. 61.
    de Imus G, Golomb C, Wilkel C, et al. Accelerated healing of pyoderma gangrenosum treated with bioengineered skin and concomitant immunosuppression. J Am Acad Dermatol. 2001;44:61–6.CrossRefPubMedGoogle Scholar

Copyright information

© Springer India 2016

Authors and Affiliations

  1. 1.Department of Dermatology and VenerologyInstitute of Medical Sciences, Banaras Hindu UniversityVaranasiIndia

Personalised recommendations