Atypical Forms of Pyoderma Gangrenosum in Inflammatory Bowel Disease: Report of Four Cases and Literature Review

Valéria Ferreira Martinelli,1,2 Pedro Martinelli Barbosa,3 Lucila Samara Dantas de Oliveira,1 Luísa de Andrade Lima Vieira de Melo,1 João Manoel Casa Nova,1 Carlos Alexandre Antunes de Brito2,4,5 1Department of Gastroenterology, Hospital das Clínicas, Federal University of Pernambuco, Recife, Pernambuco, Brazil; 2Department of Gastroenterology, Member of Organização Brasileira de Doença de Crohn e Retocolite – GEDIIB, São Paulo, Brazil; 3Department of Internal Medicine, Medical Sciences Center, Pernambucana of Health College, Recife, Pernambuco, Brazil; 4Department of Internal Medicine, Center of Medical Sciences of Federal University of Pernambuco, Pernambuco, Brazil; 5Department of Immunology, Autoimune Research Institute, Recife, Pernambuco, BrazilCorrespondence: Carlos Alexandre Antunes de Brito, Department of Immunology, Autoimune Research Institute, Avenue Rui Barbosa 715, Recife, Pernambuco, 52011-040, Brazil, Tel +55 81 31480101, Email cbritoc@gmail.comBackground: Cutaneous involvement is the second-most frequent extraintestinal manifestation of inflammatory bowel disease, with pyoderma gangrenosum (PG) a particularly relevant form because of its frequency, morbidity, and recurrence. The limited number of clinical trials involving PG increases the challenge to gastroenterologists in the management of this condition.Case Presentation: Four cases of atypical presentations of PG are reported. A 25-year-old patient with ulcerative colitis presented an extensive chronic ulcerative lesion on her left leg that was associated with significant bleeding; the intestinal disease was in remission under the use of azathioprine. The patient was on long-term use of 60 mg corticosteroid with no improvement in the skin disease; however, initiation of cyclosporine induced remission. In the second case, a 52-year-old woman was a carrier of Crohn’s disease, with a history of partial colectomy. The patient’s skin condition had evolved with a cutaneous lesion localized in the perineal region, buttocks, and colostomy pouch, simulating a case of impetigo, and this had been treated with antibiotic cycles without improvement. Lesion biopsy suggested a diagnosis of PG. Consequently, the patient was started on biological therapy with infliximab, and the PG regressed. In the third case, a 38-year-old woman with a history of pancolitis presented a picture of PG with an extensive and deep ulcerative lesion in the right breast. The lesion regressed after treatment with oral corticosteroid. The final case was a 44-year-old woman with Crohn’s disease suffering from Crohn’s disease pancolitis. The patient’s condition evolved with a mixed pattern with pustules, bullae, and ulcerative lesions in the vulva, oral cavity, gluteus, right auricular region, scalp, and left flank, and was resolved by administration of adalimumab.Conclusion: PG is an important and frequent manifestation of inflammatory bowel disease, with a spectrum of clinical variants, significant morbidity, and requiring a variety of therapeutic approaches.Keywords: inflammatory bowel disease, pyoderma gangrenosum, Crohn’s disease, ulcerative colitis, extraintestinal manifestations