Pyoderma gangrenosum in a patient with Crohn’s disease: Case report and a review of the literature

Piyoderma gangrenozum seyrek görülen ve enfeksiyöz olmayan bir nötrofilik cilt hastalığıdır. Etyolopatogenezi bilinmemektedir. Olguların yarısında altta yatan bir hastalık mevcuttur. En sık inflamatuvar bağırsak hastalığına eşlik eder. Tedavisinde immün baskılayıcı veya immünmodülatör ilaçlarla bazı topikal ajanlar kullanılmaktadır. Sistemik kortikosteroidler, tedavide ilk seçenek ilaçlardır. Crohn hastalığı zemininde gelişmiş ve başarılı bir şekilde tedavi edilmiş olan bir piyoderma gangrenozum vakası sunuldu. Crohn hastalığı, diyabetes mellitus ve hipertansiyonu olan 54 yaşında bir kadın, her iki bacağın ön iç tarafında kırmızımsı ve ağrılı lezyonları nedeniyle hastanemize başvurdu. Anormal laboratuar bulgusu olarak lökositoz (13500/μL) ve artmış sedimantasyon hızı (120 mm/saat) tespit edildi. Geniş spektrumlu antibiyotik tedavisi başlanıldı, ancak beklenen cevap alınamadı. Lezyon biyopsisinden histopatolojik değerlendirme yapıldı. Üst dermiste nekroz, şiddetli ödem, eritrosit ekstravazasyonuyla çevre dokuda rejeneratif değişiklikler, dermisin iç bölümünde nekrozu kuşatan mikst inflamatuvar reaksiyon görüldü. Kliniği, laboratuvar bulguları ve altta yatan hastalığı piyoderma gangrenozumu düşündürdü. Oral metil prednizolon başlanıldı, lezyonlarında düzelme görülmesi üzerine taburcu edildi. Ayaktan takiplerinde kortikosteroid dozu tedricen azaltılarak kesildi. Vakamız, sistemik kortikosteroidlerin piyoderma gangrenozum tedavisinde hâlâ favori ilaçlar olduğunu göstermiştir.

Crohn hastalığında piyoderma gangrenosum: Olgu sunumu ve literatürün gözden geçirilmesi

Pyoderma gangrenosum is a rare neutrophilic noninfectious dermatose. Etiopathogenesis remains unclear, but in half of cases, there is an associated underlying disease. Inflammatory bowel disease is the most common underlying disorder. Systemic immunosuppressive or immunomodulator drugs and some topical agents are used in treatment of pyoderma gangrenosum. Systemic corticosteroids are the first-choice of treatment. We reported a case with Crohn’s disease associated with pyoderma gangrenosum. She was successfully treated with oral methyl prednisolon. The case was a 54-year-old woman who admitted to hospital because of erythematous, painful plaques on the right and left pretibial surfaces. She had a history of Crohn’s disease, diabetes mellitus, and hypertension. An elevated white blood cell count (13500/μL) and high erythrocyte sedimentation rate (120 mm/h) were detected. A regime of broad-spectrum antibiotics was started, but response was poor. Histopathological assessment of biopsy specimens showed necrosis, severe edema and erythrocyte extravasations in superficial dermis, regenerative changes in adjacent epithelium, and mixed inflammatory reaction surrounding necrosis in the inner part of the dermis. Based on these clinical and laboratory findings, poor response to antibiotics and underlying disease; her skin lesions were considered as pyoderma gangrenosum. Oral methylprednisolone was started and her skin lesions improved. The steroid dose was tapered and finally stopped under outpatient follow-up. In conclusion, our patient also showed that corticosteroids continue to be the first-choice therapy in pyoderma gangrenosum.

___

  • 1. Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol Syph 1930;22: 655-80.
  • 2. Schwaegerle SM, Bergfeld WF, Senitzer D, Tidrick RT. Pyoderma gangrenosum: a review. J Am Acad Dermatol 1988;18:559-68.
  • 3. Perry HO. Pyoderma gangrenosum. South Med J 1969; 62:899-908.
  • 4. Cairns BA, Herbst CA, Sartor BR, Briggaman RA, Koruda MJ. Peristomal pyoderma gangrenosum and inflammatory bowel disease. Arch Surg 1994; 129: 769-72.
  • 5. Trost LB, McDonnell JK.Important cutaneous manifestations of inflammatory bowel disease. Postgrad Med J 2005;81:580-5.
  • 6. Yüksel I, Başar O, Ataseven H,et al. Mucocutaneous manifestations in inflammatory bowel disease. Inflamm Bowel Dis 2009;15:546-50.
  • 7. Schoetz D Jr, Coller JA, Veidenheimer MC. Pyoderma gangrenosum and Crohn’s disease: eight cases and a review of the literature. Dis Colon Rectum 1983;26:155-158.
  • 8. Wollina U. Pyoderma gangrenosum--a review. Orphanet J Rare Dis 2007;2:19-26.
  • 9. Aseni P, Di Sandro S, Mihaylov P, Lamperti L, De Carlis LG. Atypical presentation of pioderma gangrenosum complicating ulcerative colitis: rapid disappearance with methylprednisolone. World J Gastroenterol 2008;14:5471-3.
  • 10. Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol 2009;10:301-2.
  • 11. Schöfer H, Baur S. Successful treatment of postoperative pyoderma gangrenosum with cyclosporin. J Eur Acad Dermatol Venereol 2002;16:148-51.
  • 12. Kouklakis G, Moschos J, Leontiadis GI, et al. Infliximab for treatment of pyoderma gangrenosum associated with clinically inactive Crohn’s disease. A case report. Rom J Gastroenterol 2005;14:401-3.
  • 13. Regueiro M, Valentine J, Plevy S, Fleisher MR, Lichtenstein GR. Infliximab for treatment of pyoderma gangrenosum associated with inflammatory bowel disease. Am J Gastroenterol. 2003;98:1821-6.
  • 14. Miranda MF. Pyoderma gangrenosum treated with sulfasalazine and dapsone. Indian J Dermatol Venereol Leprol 2002;68:160-1.
  • 15. Lynch WS, Bergfeld WF. Pyoderma gangrenosum responsive to minocycline hydrochloride. Cutis 1978; 21: 535-8.
  • 16. Richardson JB, Callen JP. Pyoderma gangrenosum treated successfully with potassium iodide. J Am Acad Dermatol 1993; 28:1005-7.
  • 17. Paolini O, Hebuterne X, Flory P, Charles F, Rampal P. Treatment of pyoderma gangrenosum with colchicine. Lancet 1995; 345: 1057-8.
  • 18. Gupta AK, Shear NH, Sauder DN. Efficacy of human intravenous immune globulin in pyoderma gangrenosum. J Am Acad Dermatol. 1995; 32:140-2.
  • 19. Kaminska R, Ikäheimo R, Hollmen A. Plasmapheresis and cyclophosphamide as successful treatments for pyoderma gangrenosum. Clin Exp Dermatol 1999;24:81-5.
  • 20. Callen JP, Case JD, Sager D. Chlorambucil: an effective corticosteroid-sparing therapy for pyoderma gangrenosum. J Am Acad Dermatol 1989; 21:515-9.
  • 21. Cave DR, Burakoff R. Pyoderma gangrenosum associated with ulcerative colitis: treatment with disodium cromoglycate. Am J Gastroenterol 1987;82:802-4.
  • 22. Thomas CY Jr, Crouch JA, Guastello J. Hyperbaric oxygen therapy for pyoderma gangrenosum. Arch Dermatol 1974;110:445-6.