Prevalence of pyoderma gangrenosum and Sweet's syndrome in inflammatory bowel disease at a tertiary healthcare center

Background and aim: dermatological manifestations are normally found in one third of patients with inflammatory bowel disease. In this study, the prevalence, clinical characteristics, intestinal disease activity, and treatment response of neutrophilic dermatoses (pyoderma gangrenosum and Sweet's syndrome) were determined in patients with inflammatory bowel disease. Methods: a retrospective, observational study was performed in patients with inflammatory bowel disease and neutrophilic dermatoses between March 2012 and March 2018. Results: of 444 patients analyzed, 10 complied with the inclusion criteria. Seven had pyoderma gangrenosum and three presented Sweet's syndrome; and one patient developed both pathologies. The prevalence of neutrophilic dermatoses was 2.3 % (10/444), comprising 1.6 % with pyoderma gangrenosum and 0.7 % with Sweet's syndrome. Six out of seven patients with pyoderma gangrenosum were female and had ulcerative colitis. The most frequent clinical presentation of pyoderma gangrenosum was the ulcerative subtype. Active moderate-to-severe intestinal disease was found in 71.4 % of patients. Biological therapy was prescribed to three patients with partial response to corticosteroids and persistent intestinal disease activity. This therapy was not indicated for cutaneous manifestations only. Three patients with moderate-to-severe Crohn's disease presented classical (n = 2) and pustular (n = 1) Sweet's syndrome. A complete response was achieved in all Sweet's syndrome cases treated with corticosteroids. Biological therapy was prescribed to control intestinal disease activity. Conclusions: pyoderma gangrenosum was the most frequent cutaneous manifestation of neutrophilic dermatoses, predominantly in females with ulcerative colitis, and highly associated with intestinal disease activity. Anti-tumor necrosis factor was effective in patients with partial cutaneous and intestinal disease response.