Peristomal pyoderma gangrenosum and inflammatory bowel disease
B. A. Cairns, C. A. Herbst, B. R. Sartor, R. A. Briggaman and M. J. Koruda
Department of Surgery, University of North Carolina School of Medicine, Chapel Hill.
Pyoderma gangrenosum (PG) is a debilitating skin disease most often
associated with inflammatory bowel disease and is a reportedly rare cause
of peristomal ulceration. The lesions of PG rapidly evolve from small,
erythematous pustules to deep, painful, pyogenic ulcers within hours to
days of onset. Although the behavior and the appearance of the lesions of
peristomal PG are diagnostic, a lack of familiarity with PG often leads to
misdiagnosis and inappropriate therapy. This study reports four cases of
peristomal PG and discusses the 20 previously reported cases in patients
with inflammatory bowel disease. Seventy-five percent of patients were
female and 67% had Crohn's disease. All patients had colitis, including all
of the patients with Crohn's disease, 82% of whom had additional perineal
complications. The diagnosis of peristomal PG was based on clinical
appearance alone in 83% of cases. The onset of peristomal PG ranged from 2
weeks to 3 years following ostomy. The response to medical therapy was
variable. All cases (17 of 17) treated with high-dose corticosteroids and
local wound care responded, but five cases required additional therapy. No
patient was successfully treated with stoma revision. Risk factors for the
development of peristomal PG include Crohn's colitis, female gender, and
perineal disease. While most patients respond well to systemic steroids and
local wound care, up to one third of patients require long-term medical
management.