Pyoderma gangrenosum in refractory celiac disease: a case report

BMC Gastroenterology

Giovanni Monteleone²  Francesco Pallone²  Augusto Orlandi¹  Elena Campione²  Irene Marafini²  Roberta Caruso²  Silvia Sedda² 

[1] Department of Biomedicine and Prevention, Anatomic Pathology Unit, University of Rome “Tor Vergata”, Rome, Italy;Department of Systems Medicine, University of Rome “Tor Vergata”, Via Montpellier, 1, 00133 Rome, Italy

关键词: Pyoderma gangrenosum;    Celiac disease;    Cutaneous ulcers;    Gluten;   

Others  :  1121879
DOI  :  10.1186/1471-230X-13-162

 received in 2013-08-07, accepted in 2013-11-22,  发布年份 2013

【 摘 要 】

Background

Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis characterized by painful cutaneous ulcerations and often associated with systemic inflammatory and neoplastic diseases. Here we report the first case of pyoderma gangrenosum in a patient with refractory celiac disease.

Case presentation

A 52-year-old woman with a previously diagnosed refractory celiac disease resistant to steroids and immunosuppressive drugs presented to our hospital for a rapidly growing, painful inflammatory skin lesion of the left leg. Physical examination revealed a painful lesion with focal ulceration, necrosis and pus discharge with active inflammatory borders at the external part of the left leg. Histological evaluation of a skin biopsy and analysis of inflammatory cytokines and matrix-degrading proteases in lesional skin samples confirmed the clinical suspicion of pyoderma gangrenosum. Treatment with oral prednisone was rapidly followed by a complete healing of the skin lesion but no improvement of symptoms/signs of malabsorption.

Conclusion

Treatment of the patient with systemic steroids healed the skin lesion without improving the underlying refractory celiac disease. This observation raises the possibility that refractory celiac disease and pyoderma gangrenosum may be immunologically different.

【 授权许可】

   
2013 Sedda et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

【 参考文献 】

• [1]Hadi A, Lebwohl M: Clinical features of pyoderma gangrenosum and current diagnostic trends. J Am Acad Dermatol 2011, 64(5):950-954.
• [2]von den Driesch P: Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol 1997, 137(6):1000-1005.
• [3]Crowson AN, Mihm MC Jr, Magro C: Pyoderma gangrenosum: a review. J Cutan Pathol 2003, 30(2):97-107.
• [4]Powell FC, Su WP, Perry HO: Pyoderma gangrenosum: classification and management. J Am Acad Dermatol 1996, 34(3):395-409. quiz 410–392
• [5]Powell FC, O’Kane M: Management of pyoderma gangrenosum. Dermatol Clin 2002, 20(2):347-355. viii
• [6]Graham JA, Hansen KK, Rabinowitz LG, Esterly NB: Pyoderma gangrenosum in infants and children. Pediatr Dermatol 1994, 11(1):10-17.
• [7]Weenig RH, Davis MD, Dahl PR, Su WP: Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 2002, 347(18):1412-1418.
• [8]Ludvigsson JF, Leffler DA, Bai JC, Biagi F, Fasano A, Green PH, Hadjivassiliou M, Kaukinen K, Kelly CP, Leonard JN, et al.: The Oslo definitions for coeliac disease and related terms. Gut 2013, 62(1):43-52.
• [9]Oka M, Berking C, Nesbit M, Satyamoorthy K, Schaider H, Murphy G, Ichihashi M, Sauter E, Herlyn M: Interleukin-8 overexpression is present in pyoderma gangrenosum ulcers and leads to ulcer formation in human skin xenografts. Lab Invest 2000, 80(4):595-604.
• [10]Marzano AV, Cugno M, Trevisan V, Fanoni D, Venegoni L, Berti E, Crosti C: Role of inflammatory cells, cytokines and matrix metalloproteinases in neutrophil-mediated skin diseases. Clin Exp Immunol 2010, 162(1):100-107.
• [11]Di Sabatino A, Corazza GR: Coeliac disease. Lancet 2009, 373(9673):1480-1493.
• [12]Humbert P, Pelletier F, Dreno B, Puzenat E, Aubin F: Gluten intolerance and skin diseases. Eur J Dermatol 2006, 16(1):4-11.