Question: A 55-year-old woman with longstanding ileocolonic Crohn's disease presented with a facial lesion. She had previously undergone a total proctocolectomy and end-ileostomy for disease that was refractory to prednisone, 6-mercaptopurine, infliximab, and adalimumab. In 2010, she developed an ulcerating lesion on her face, between the left naris and left upper lip. A biopsy showed dermal neutrophilic infiltration (Figure A), and wound cultures did not demonstrate infection. The lesion worsened despite treatment with certolizumab pegol, topical tacrolimus, and intralesional steroids. The patient was admitted for severe facial pain associated with worsening of the ulcerating lesion. She had no gastrointestinal symptoms. On physical examination, there was a 2 × 2-cm ulcer superior to the left upper lip with a full-thickness defect, through which a tooth could be seen (Figure B). The borders of the ulcer had a raised, violaceous edge overhanging the ulcer bed. What is the diagnosis? Look on page 1258 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Pyoderma gangrenosum (PG) typically involves the lower extremities, but infrequently affects other areas including the face.1Wittekindt C. Luers J.C. Klussmann J.P. et al.Pyoderma gangrenosum in the head and neck.Arch Otolaryngol Head Neck Surg. 2007; 133: 83-85Crossref PubMed Scopus (10) Google Scholar Others have reported improvement with various immunosuppressive agents, anti-tumor necrosis factor (TNF) therapy, and intravenous immunoglobulin (IVIg).2Lachapelle J.M. Marot L. Jablonska S. Superficial granulomatous pyoderma gangrenosum of the face, successfully treated by ciclosporine: a long-term follow-up.Dermatology. 2001; 202: 155-157Crossref PubMed Scopus (36) Google Scholar PG classically exhibits pathergy with any trauma, including surgical manipulation.3Davis J.C. Landeen J.M. Levine R.A. Pyoderma gangrenosum: skin grafting after preparation with hyperbaric oxygen.Plast Reconstr Surg. 1987; 79: 200-207Crossref PubMed Scopus (78) Google Scholar Although biopsy results alone cannot be used to diagnose PG, the histopathology of lesions usually shows significant dermal neutrophilic infiltration with occasional cases showing leukocytoclastic vasculitis (Figure A).2Lachapelle J.M. Marot L. Jablonska S. Superficial granulomatous pyoderma gangrenosum of the face, successfully treated by ciclosporine: a long-term follow-up.Dermatology. 2001; 202: 155-157Crossref PubMed Scopus (36) Google Scholar Our patient presented with full-thickness penetration of the lip secondary to PG that progressed despite treatment with an anti-TNF drug and topical tacrolimus. She was started on a continuous infusion of intravenous cyclosporine in conjunction with corticosteroids. One week after starting cyclosporine, the lesion had partially improved. However, she experienced septic complications necessitating discontinuation of cyclosporine and was thus given 2 doses of IVIg.4Gupta A.K. Shear N.H. Sauder D.N. Efficacy of human intravenous immune globulin in pyoderma gangrenosum.J Am Acad Dermatol. 1995; 32: 140-142Abstract Full Text PDF PubMed Scopus (82) Google Scholar Broad-spectrum antibiotics were also initiated. In addition, she underwent hyperbaric oxygen therapy in preparation for wound closure, to minimize infection and promote angiogenesis.3Davis J.C. Landeen J.M. Levine R.A. Pyoderma gangrenosum: skin grafting after preparation with hyperbaric oxygen.Plast Reconstr Surg. 1987; 79: 200-207Crossref PubMed Scopus (78) Google Scholar Three sutures were used to close the full-thickness defect (Figure C). She improved on these treatment modalities and was discharged on certolizumab pegol, oral corticosteroids, and topical tacrolimus. Four months after presentation, the lesion had completely healed (Figure D). Although most literature cautions against surgical manipulation of PG owing to pathergy, this situation required wound closure to allow the patient to eat, and prevent potential wound seeding from oropharyngeal organisms while on significant immunosuppression. This case of complicated facial PG failing anti-TNF therapy was thus successfully treated using intravenous cyclosporine, IVIg, and hyperbaric oxygen in conjunction with surgical wound closure.
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