Pyoderma gangrenosum: Clinical presentation and outcome in 18 cases and review of the literature

Abstract

Pyoderma gangrenosum (PG) is an idiopathic ulcerative neutrophilic inflammatory skin disease characterized by variable clinical presentation and outcome. Because its incidence is low, no prospective randomized controlled trials and only a few large case studies on PG have been reported. We demonstrate the clinical presentation and outcome in 18 cases with severe and chronic PG. In our 18 patients, the female/male ratio was 3.5:1, the mean onset age was 53.1 yrs (range 23-78); six cases (33%) had associated diseases (inflammatory bowel disease [n = 2, 11%], monoclonal gammopathy [n = 2, 11%], rheumatoid arthritis [n = 1, 6%], diabetes mellitus [n = 1, 6%]). Anatomic locations involved were lower leg (n = 14, 78%), abdomen (n = 5, 28%), arm (n = 3, 17%), breast (n = 2, 11%), and buttocks (n = 1,6%). Five patients (28%) had multiple lesions (n > or = 2). Immunosuppressive monotherapies (n = 3, 17%) and polytherapies (n = 15, 83%) were used. 13 patients (72%) showed complete remission (mean duration to complete remission: 1.29 yrs), three patients (17%) persistent disease (mean duration: 8 yrs), contact was lost to one patient (6%) and one patient died (6%). Our observations add to the growing body of evidence that PG responds in most cases to systemic immunosuppressive treatment, with corticosteroids and cyclosporine representing first-line therapies. Besides reporting the clinical outcome in our 18 patients, we review the literature and discuss treatment recommendations that take additional factors including associated conditions, disease severity and localization of lesions into consideration.