Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP, et al.
Medicine. Date of publication 2000 Jan 1;volume 79(1):37-46.
1. Medicine (Baltimore). 2000 Jan;79(1):37-46.
Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis
on time to remission. Case review of 86 patients from 2 institutions.
Bennett ML(1), Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP.
Author information:
(1)Department of Dermatology, Wake Forest University School of Medicine,
Winston-Salem, North Carolina, USA.
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.
PMID: 10670408 [Indexed for MEDLINE]
