Background: Pyoderma gangraenosum is an immune-mediated, inflammatory, neutrophilic dermatosis of unknown etiology, which represents one of the extraintestinal manifestations of inflammatory bowel disease. It is a rare disease that occurs in less than 1% of patients with inflammatory bowel disease and with the same ratio in patients with Crohn's disease and ulcerative colitis.
Main observations:A 36-year-old woman was diagnosed with ulcerative colitis 6 years before admission to our dermatology department with an acute disseminated pyoderma gangraenosum with mucosal involvement, during a flare of ulcerative colitis. Disease progression was interrupted by intravenous administration of the tumor necrosis factor-α inhibitor infliximab at 5mg/kg at weeks 0, 2, and 6 (1st cycle) and every 8 weeks thereafter. Improvement of intestinal, skin and oral manifestations was evident already after the 1st cycle of treatment and has been maintained since (at least 16 months).
Conclusions:This case report is one of very few on disseminated pyoderma gangraenosum with oral involvement complicating ulcerative colitis, where infliximab was shown to have a rapid efficacy on skin, mucosal and bowel symptoms. (J Dermatol Case Rep. 2015; 9(3): 62-66) Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.
IntroductionPyoderma gangraenosum (PG) is a severe ulcerating, primarily sterile, neutrophilic dermatosis. It can occur in association with systemic diseases in adults, the most common of which is ulcerative colitis (UC). 1 PG has been reported in 1-10% of patients with UC, 2 affecting equally men and women with a peak age incidence between 25 and 54 years. Approximately 50-78% of patients with PG have an underlying systemic disease 3 such as inflammatory bowel disease (IBD), myeloproliferative and rheumatological diseases. 4 PG has been reported to occur before, during, after or even independently of the onset of an underlying IBD.
5Four clinical and histological variants of PG have been described: ulcerative, pustular, bullous, and vegetative. 4 We present here a female patient with severe, disseminated PG, refractory to conventional treatment, during a clinical flare of a previously diagnosed UC. Both entities responded to infliximab, a chimeric monoclonal antibody that inhibits tumor necrosis factor-α (TNF-α).
Case report
Medical HistoryA 36-year-old female was diagnosed with UC in 2005. The leading symptom was bloody diarrhea. Under treatment with mesalazine, remission was maintained for nearly 5 years. In October 2010, the patient relapsed with bloody diarrhea (at DOI: http://dx.doi.org/10.3315/jdcr.2015DOI: http://dx.doi.org/10.3315/jdcr. .1206 62 least eight stools per day), fever and diffuse abdominal pain with increasing severity of the symptoms. She was admitted to an internal medicine department, where she was treated with antibiotics (levofloxacin and metronidazol), mesalazine, sulfasalazin and low-dose corticosteroids (20 mg prednisone daily). After tw...