Pyoderma Gangrenosum A Comparison of Typical and Atypical Forms with an Emphasis on Time to Remission. Case Review of 86 Patients from 2 Institutions

Abstract

Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.

Pyoderma gangrenosum as a initial manifestation of ulcerative proctocolitis

“…The relation between between PG and the extension, length and severity of IBD is controversial. PG is a relatively rare extraintestinal presentation of ulcerative proctocolitis, and its incidence ranges from 2 and 12% 17,18,19 , affecting both genders and all age groups. It is also associated with Chron's disease, however, the prevalence of such association is lower than the one observed for ulcerative proctocolitis 20,21 .…”

Atypical pyoderma gangrenosum following total knee replacement surgery: first report in dermatologic literature

“…4 Systemic associations of classic PG like inflammatory bowel disease, myeloproliferative disorders and inflammatory arthritis were absent. Those with atypical PG like myelogenous leukemia, myelodysplastic disorders, refractory anemias, IgA paraproteinemia, were also absent 5 Also, a satellite lesion of PG developing on the thigh was mistakenly incised by the surgeon and led to pathergy which can develop with minor injuries including cuts or may follow even needle stick injuries or surgery. 6 FIGURE 1: Atypical Pyoderma gangrenosum appearing at and extending from the site of the total knee replacement surgery FIGURE 2: Iatrogenic Pathergy occurring at the satellite lesion of PG which was drained surgically by the treating surgeon This is the first time that atypical pyoderma gangrenosum following the trauma of a total knee replacement surgery has been described in international dermatologic literature.…”

Pyoderma gangrenosum (1èrepartie) : mise au point

“…Dans une étude [7], faite aux USA, on retrouve une prédo-minance ethnique : plus de 70 % des cas de PG toucheraient des Blancs mais il s'agit probablement d'un biais de recrutement car cette différence n'apparait dans aucune autre étude.…”

“…Le PG se développe de façon prédominante sur les membres inférieurs (75-80 %) et les membres supérieurs (77 %) dans les formes atypiques [7].…”

“…Callen et al [17] et quelques auteurs [7] ont proposé une classification différente de celle exposée précédemment.…”