eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Pyoderma Gangrenosum

Author: J Mark Jackson, MD, Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville
Coauthor(s): Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Dec 8, 2009

Introduction

Background

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition of uncertain etiology. Pyoderma gangrenosum was first described in 1930. It is associated with systemic diseases in at least 50% of patients who are affected. The diagnosis is made by excluding other causes of similar appearing cutaneous ulcerations, including infection, malignancy, vasculitis, collagen vascular diseases, diabetes, and trauma. Ulcerations of pyoderma gangrenosum may occur after trauma or injury to the skin in 30% of patients; this process is termed pathergy.

The 2 primary variants of pyoderma gangrenosum are the classic ulcerative form, usually observed on the legs, and a more superficial variant known as atypical pyoderma gangrenosum that tends to occur on the hands. Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic abscesses. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation.1 Other organs systems that may be involved include the heart, the central nervous system, the GI tract, the eyes,2,3 the liver, the spleen, bones, and lymph nodes. The prognosis of pyoderma gangrenosum is generally good; however, recurrences may occur and residual scarring is common. Therapy of pyoderma gangrenosum involves the use of anti-inflammatory agents, such as corticosteroids, and immunosuppressive agents.

Pathophysiology

The pathophysiology of pyoderma gangrenosum is poorly understood, but dysregulation of the immune system, specifically altered neutrophil chemotaxis, is believed to be involved.

Frequency

United States

Pyoderma gangrenosum occurs in about 1 person per 100,000 people each year.

Mortality/Morbidity

Death from pyoderma gangrenosum is rare, but it may occur due to an associated disease or as the result of therapy. Pain is a usual complaint of patients and may require narcotics. Pathergy, or the development of pyoderma gangrenosum – like lesions at the site of skin trauma, may create problems with wound healing, especially after surgical procedures.

Race

No racial predilection is apparent for pyoderma gangrenosum.

Sex

Pyoderma gangrenosum affects both sexes. A slight female predominance may exist.

Age

All ages may be affected, but pyoderma gangrenosum predominantly occurs in the fourth and fifth decades of life. Children may be affected, but they account for only 3-4% of the total number of cases. Pyoderma gangrenosum may affect children and adolescents. Nothing is clinically distinctive about these patients other than their age.4

Clinical

History

  • Patients with pyoderma gangrenosum usually describe the initial lesion as a bite reaction, with a small, red papule or pustule changing into a larger ulcerative lesion. Often, patients give a history of a brown recluse or other spider bite, but they have no evidence that a spider actually caused the initial event.
  • Pain is the predominant historical complaint.
  • Arthralgias and malaise may often be present.
  • A complete history should be taken with special focus on the organ systems listed below to determine any underlying systemic disease. Systemic illnesses are seen in 50% of patients with pyoderma gangrenosum and may occur prior to, concurrently or following the diagnosis.
    • Commonly associated diseases include inflammatory bowel disease, either ulcerative colitis or regional enteritis/Crohn disease, and a polyarthritis that is usually symmetric and may be either seronegative or seropositive; and hematologic diseases/disorders, such as leukemia or preleukemic states, predominantly myelocytic in nature or monoclonal gammopathies (primarily immunoglobulin A [IgA]).5,6
    • Less common associated diseases include other forms of arthritis, such as psoriatic arthritis, osteoarthritis, or spondyloarthropathy; hepatic diseases, including hepatitis and primary biliary cirrhosis; myelomas (IgA type predominantly); and immunologic diseases, such as lupus erythematosus and Sjögren syndrome.7,8

Physical

  • Two main variants of pyoderma gangrenosum exist: classic and atypical. Several other variants may exist.
    • Classic pyoderma gangrenosum, as shown in the image below, is characterized by a deep ulceration with a violaceous border that overhangs the ulcer bed. These lesions of pyoderma gangrenosum most commonly occur on the legs, but they may occur anywhere on the body.

    • Classic or typical pyoderma gangrenosum. This pat...

      Classic or typical pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.

      [ CLOSE WINDOW ]
      Classic or typical pyoderma gangrenosum. This pat...

      Classic or typical pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.

    • Atypical pyoderma gangrenosum, as shown in the image below, has a vesiculopustular "juicy" component. This is usually only at the border, is erosive or superficially ulcerated, and most often occurs on the dorsal surface of the hands, the extensor parts of the forearms, or the face.

    • Atypical pyoderma gangrenosum.

      Atypical pyoderma gangrenosum.

      [ CLOSE WINDOW ]
      Atypical pyoderma gangrenosum.

      Atypical pyoderma gangrenosum.

    • Classic pyoderma gangrenosum may occur around stoma sites; this type is known as peristomal pyoderma gangrenosum and is shown in the image below. This form is often mistaken for a wound infection or irritation from the appliance.

    • Peristomal pyoderma gangrenosum.

      Peristomal pyoderma gangrenosum.

      [ CLOSE WINDOW ]
      Peristomal pyoderma gangrenosum.

      Peristomal pyoderma gangrenosum.

    • Pyoderma gangrenosum may occur on the genitalia; this form is termed vulvar or penile pyoderma gangrenosum. This variant must be differentiated from sexually transmitted diseases.9
    • An intraoral form of the disease, known as pyostomatitis vegetans, has been reported and occurs primarily in patients with inflammatory bowel disease.
    • Extracutaneous neutrophilic disease may be evident upon ocular examination and has also been reported in the lungs, liver, and bones.7,10

Causes

No specific causes of pyoderma gangrenosum have been identified, but trauma to the skin in patients with pyoderma gangrenosum may induce new lesions. This is called pathergy.

More on Pyoderma Gangrenosum

Overview: Pyoderma Gangrenosum
Differential Diagnoses & Workup: Pyoderma Gangrenosum
Treatment & Medication: Pyoderma Gangrenosum
Follow-up: Pyoderma Gangrenosum
Multimedia: Pyoderma Gangrenosum
References
[ CLOSE WINDOW ]

References

  1. Brown TS, Marshall GS, Callen JP. Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: a rarely recognized extracutaneous manifestation of a neutrophilic dermatosis. J Am Acad Dermatol. Jul 2000;43(1 Pt 1):108-12. [Medline].

  2. Ayyala RS, Armstrong S. Corneal melting and scleromalacia perforans in a patient with pyoderma gangrenosum and acute myeloid leukemia. Ophthalmic Surg Lasers. Apr 1998;29(4):328-31. [Medline].

  3. Happle R, Schiffer HP, Kövary PM. Ocular involvement in pyoderma gangrenosum. Arch Dermatol. Nov 1977;113(11):1612. [Medline].

  4. Graham JA, Hansen KK, Rabinowitz LG, Esterly NB. Pyoderma gangrenosum in infants and children. Pediatr Dermatol. Mar 1994;11(1):10-7. [Medline].

  5. Branagan NM, Higgins SP, Halim SA, Le TH. Systemic polyarteritis nodosa mimicking pyoderma gangrenosum in a rare association with small lymphocytic leukaemia/chronic lymphocytic leukaemia. Clin Exp Dermatol. Jul 2009;34(5):e127-9. [Medline].

  6. Namazi MR, Kerchner KR, Pichardo RO. Essential type II mixed cryoglobulinemia causing pyoderma gangrenosum-like ulcers. ScientificWorldJournal. 2008;8:228. [Medline].

  7. Vadillo M, Jucgla A, Podzamczer D, Rufi G, Domingo A. Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia. Br J Dermatol. Sep 1999;141(3):541-3. [Medline].

  8. Ahmad K, Ramsay B. Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. Clin Exp Dermatol. Jan 2009;34(1):46-8. [Medline].

  9. McCalmont CS, Leshin B, White WL, Greiss FC Jr, Jorizzo JL. Vulvar pyoderma gangrenosum. Int J Gynaecol Obstet. Jun 1991;35(2):175-8. [Medline].

  10. Brown TS, Marshall GS, Callen JP. Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: a rarely recognized extracutaneous manifestation of a neutrophilic dermatosis. J Am Acad Dermatol. Jul 2000;43(1 Pt 1):108-12. [Medline].

  11. Nybaek H, Olsen AG, Karlsmark T, Jemec GB. Topical therapy for peristomal pyoderma gangrenosum. J Cutan Med Surg. Jul-Aug 2004;8(4):220-3. [Medline].

  12. Jackson JM. TNF- alpha inhibitors. Dermatol Ther. Jul-Aug 2007;20(4):251-64. [Medline].

  13. Fedi MC, Quercetani R, Lotti T. Recalcitrant pyoderma gangrenosum responsive to cyclosporine. Int J Dermatol. Feb 1993;32(2):119. [Medline].

  14. Matis WL, Ellis CN, Griffiths CE, Lazarus GS. Treatment of pyoderma gangrenosum with cyclosporine. Arch Dermatol. Aug 1992;128(8):1060-4. [Medline].

  15. Wilson DM, John GR, Callen JP. Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder:report of a patient with rheumatoid arthritis, pustular vasculitis,pyoderma gangrenosum, and Sweet''s syndrome with an excellent response tocyclosporine therapy. J Am Acad Dermatol. Feb 1999;40(2 Pt 2):331-4. [Medline].

  16. Daniels NH, Callen JP. Mycophenolate mofetil is an effective treatment for peristomal pyoderma gangrenosum. Arch Dermatol. Dec 2004;140(12):1427-9. [Medline].

  17. Eaton PA, Callen JP. Mycophenolate mofetil as therapy for pyoderma gangrenosum. Arch Dermatol. Jul 2009;145(7):781-5. [Medline].

  18. August PJ, Wells GC. Pyoderma gangrenosum treated with azathioprine and prednisolone. Br J Dermatol. 1974;91:80-2.

  19. Burruss JB, Farmer ER, Callen JP. Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum. J Am Acad Dermatol. Nov 1996;35(5 Pt 1):720-4. [Medline].

  20. Johnson RB, Lazarus GS. Pulse therapy. Therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol. Feb 1982;118(2):76-84. [Medline].

  21. Zonana-Nacach A, Jiménez-Balderas FJ, Martínez-Osuna P, Mintz G. Intravenous cyclophosphamide pulses in the treatment of pyoderma gangrenosum associated with rheumatoid arthritis: report of 2 cases and review of the literature. J Rheumatol. Jul 1994;21(7):1352-6. [Medline].

  22. Brooklyn TN, Dunnill MG, Shetty A, Bowden JJ, Williams JD, Griffiths CE, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. Apr 2006;55(4):505-9. [Medline].

  23. Kaur MR, Lewis HM. Severe recalcitrant pyoderma gangrenosum treated with infliximab. Br J Dermatol. Sep 2005;153(3):689-91. [Medline].

  24. Fernández A, Velasco A, Prieto V, Canueto J, Alvarez A, Rodríguez A. Response to Infliximab in Atypical Pyoderma Gangrenosum Associated With Ulcerative Colitis. Am J Gastroenterol. Aug 27 2008;[Medline].

  25. Cummins DL, Anhalt GJ, Monahan T, Meyerle JH. Treatment of pyoderma gangrenosum with intravenous immunoglobulin. Br J Dermatol. Dec 2007;157(6):1235-9. [Medline].

  26. Aractingi S, Mallet V, Pinquier L, Chosidow O, Vignon-Pennamen MD, Degos L, et al. Neutrophilic dermatoses during granulocytopenia. Arch Dermatol. Oct 1995;131(10):1141-5. [Medline].

  27. Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP. Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore). Jan 2000;79(1):37-46. [Medline].

  28. Cairns BA, Herbst CA, Sartor BR, Briggaman RA, Koruda MJ. Peristomal pyoderma gangrenosum and inflammatory bowel disease. Arch Surg. Jul 1994;129(7):769-72. [Medline].

  29. Callen JP. Pyoderma gangrenosum and related disorders. Med Clin North Am. Sep 1989;73(5):1247-61. [Medline].

  30. Callen JP. Pyoderma gangrenosum. Lancet. Feb 21 1998;351(9102):581-5. [Medline].

  31. Callen JP, Jackson JM. Pyoderma gangrenosum: an update. Rheum Dis Clin North Am. Nov 2007;33(4):787-802, vi. [Medline].

  32. Charles CA, Bialy TL, Falabella AF, Eaglstein WH, Kerdel FA, Kirsner RS. Poor prognosis of arthritis-associated pyoderma gangrenosum. Arch Dermatol. Jul 2004;140(7):861-4. [Medline].

  33. Chow RK, Ho VC. Treatment of pyoderma gangrenosum. J Am Acad Dermatol. Jun 1996;34(6):1047-60. [Medline].

  34. Dourmishev AL, Miteva I, Schwartz RA. Pyoderma gangrenosum in childhood. Cutis. Oct 1996;58(4):257-62. [Medline].

  35. Gettler S, Rothe M, Grin C, Grant-Kels J. Optimal treatment of pyoderma gangrenosum. Am J Clin Dermatol. 2003;4(9):597-608. [Medline].

  36. Goldstein F, Krain R, Thornton JJ. Intralesional steroid therapy of pyoderma gangrenosum. J Clin Gastroenterol. Dec 1985;7(6):499-501. [Medline].

  37. Hay CR, Messenger AG, Cotton DW, Bleehen SS, Winfield DA. Atypical bullous pyoderma gangrenosum associated with myeloid malignancies. J Clin Pathol. Apr 1987;40(4):387-92. [Medline].

  38. Hayani A, Steuber CP, Mahoney DH Jr, Levy ML. Pyoderma gangrenosum in childhood leukemia. Pediatr Dermatol. Dec 1990;7(4):296-8. [Medline].

  39. Holt PJ, Davies MG, Saunders KC, Nuki G. Pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine (Baltimore). Mar 1980;59(2):114-33. [Medline].

  40. Hughes AP, Jackson JM, Callen JP. Clinical features and treatment of peristomal pyoderma gangrenosum. JAMA. Sep 27 2000;284(12):1546-8. [Medline].

  41. Hurwitz RM, Haseman JH. The evolution of pyoderma gangrenosum. A clinicopathologic correlation. Am J Dermatopathol. Feb 1993;15(1):28-33. [Medline].

  42. Jorizzo JL, Solomon AR, Zanolli MD, Leshin B. Neutrophilic vascular reactions. J Am Acad Dermatol. Dec 1988;19(6):983-1005. [Medline].

  43. Kaplan B, Trau H, Sofer E, Feinstein A, Schewach-Millet M. Treatment of pyoderma gangrenosum with clofazimine. Int J Dermatol. Aug 1992;31(8):591-3. [Medline].

  44. Keltz M, Lebwohl M, Bishop S. Peristomal pyoderma gangrenosum. J Am Acad Dermatol. 1993;27:360-64.

  45. Ko CB, Walton S, Wyatt EH. Pyoderma gangrenosum: associations revisited. Int J Dermatol. Aug 1992;31(8):574-7. [Medline].

  46. Koester G, Tarnower A, Levisohn D, Burgdorf W. Bullous pyoderma gangrenosum. J Am Acad Dermatol. Nov 1993;29(5 Pt 2):875-8. [Medline].

  47. McCulloch AJ, McEvoy A, Jackson JD, Jarvis EH. Severe steroid responsive pneumonitis associated with pyoderma gangrenosum and ulcerative colitis. Thorax. Apr 1985;40(4):314-5. [Medline].

  48. Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med. May 1985;55(217):173-86. [Medline].

  49. Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol. Mar 1996;34(3):395-409; quiz 410-2. [Medline].

  50. Prystowsky JH, Kahn SN, Lazarus GS. Present status of pyoderma gangrenosum. Review of 21 cases. Arch Dermatol. Jan 1989;125(1):57-64. [Medline].

  51. Rand RP, Olerud JE, Verrier ED. Pyoderma gangrenosum after coronary artery bypass grafting. Ann Thorac Surg. Apr 1993;55(4):1016-8. [Medline].

  52. Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol. Nov 2004;43(11):790-800. [Medline].

  53. Takeuchi K, Kyoko H, Hachiya M, Ohara A, Tsukimoto I, Kanto H, et al. Pyoderma gangrenosum of the skin and respiratory tract in a 5-year-old girl. Eur J Pediatr. May 2003;162(5):344-5. [Medline].

  54. Tjandra JJ, Hughes LE. Parastomal pyoderma gangrenosum in inflammatory bowel disease. Dis Colon Rectum. Sep 1994;37(9):938-42. [Medline].

  55. Urano S, Kodama H, Kato K, Nogura K. Pyoderma gangrenosum with systemic involvement. J Dermatol. Jul 1995;22(7):515-9. [Medline].

  56. Urano S, Kodama H, Kato K, Nogura K. Pyoderma gangrenosum with systemic involvement. J Dermatol. Jul 1995;22(7):515-9. [Medline].

  57. Vidal D, Puig L, Gilaberte M, Alomar A. Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features. J Dermatolog Treat. Jun 2004;15(3):146-52. [Medline].

  58. Vignon-Pennamen MD, Wallach D. Neutrophilic disease: a review of extracutaneous manifestations. Eur J Dermatol. 1995;5:449-55.

  59. von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. Dec 1997;137(6):1000-5. [Medline].

  60. Yoshida C, Kojima H, Ishigaki T, Katsura Y, Kaneko S, Suzukawa K, et al. Association of pyoderma gangrenosum and sterile osteomyelitis in a patient having myelodysplastic syndrome with der(1;7)(q10;q10). Eur J Haematol. Feb 2004;72(2):149-53. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

pyoderma gangrenosum, PG, classic PG, classic pyoderma gangrenosum, atypical PG, atypical pyoderma gangrenosum, pyostomatitis vegetans, vulvar pyoderma gangrenosum, penile pyoderma gangrenosum, peristomal pyoderma gangrenosum

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

J Mark Jackson, MD, Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville
J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Acne and Rosacea Society, American Medical Association, Kentucky Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

David P Fivenson, MD, Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan
David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis  investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.