Amy P. Hughes, J. Mark Jackson, Jeffrey P. Callen. Clinical Features and Treatment of Peristomal Pyoderma Gangrenosum. JAMA. 2000;284(12):1546–1548. doi:10.1001/jama.284.12.1546
Author Affiliations: Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Louisville, Ky.
Context Peristomal pyoderma gangrenosum (PPG), an unusual variant of pyoderma
gangrenosum, has been reported almost exclusively in patients with inflammatory
bowel disease (IBD) and is frequently misdiagnosed.
Objective To better characterize the clinical manifestations, diagnosis, and management
Design, Setting, and Patients Retrospective analysis of 7 patients with PPG observed in a university-affiliated
community setting between 1988 and December 1999.
Main Outcome Measures Clinical and histopathologic features, associated disorders, and microbiologic
Results Two patients had Crohn disease, 2 had ulcerative colitis, and 3 had
abdominal cancer. Five patients had at least 1 relapse of PPG after initial
healing. Although 3 of 4 patients with IBD had active bowel disease, a parallel
course with PPG occurred in only 1 patient. Both patients whose stoma was
relocated developed an ulcer at the new site. Effective therapies included
topical superpotent corticosteroids; intralesional injection of triamcinolone
acetonide at the ulcer margin; topical cromolyn sodium; oral dapsone, prednisone,
cyclosporine, mycophenolate mofetil; and intravenous infliximab.
Conclusion Our experiences demonstrate that although PPG has been most often reported
in patients with IBD, it may occur in the absence of IBD. Biopsy of the skin
lesion is not diagnostic but excludes other causes. Relocation of the stoma
may be associated with a new ulceration and should be avoided. Trauma to the
skin of a predisposed patient may elicit the pustules or ulcerations associated
Pyoderma gangrenosum is an idiopathic, inflammatory, ulcerative condition
of the skin, initially described by Brunsting et al.1
The characteristic lesion is an ulceration with a well-defined, undermined,
violaceous border.2 Pyoderma gangrenosum has
been reported in association with inflammatory bowel disease (IBD), various
arthritides, and hematologic diseases.3- 6
Early lesions are often pustular, and fistulous tracts may also occur. Pyoderma
gangrenosum is frequently painful, and healing typically results in a cribriform
scar. The diagnosis is confirmed by exclusion of other processes, in particular
infections and neoplasia. Cultures fail to reveal pathogenic organisms, and
biopsy demonstrates a nonspecific inflammatory reaction usually characterized
by dermal infiltration of neutrophils.
Peristomal pyoderma gangrenosum (PPG) is unusual and is frequently misdiagnosed
as a stitch abscess, contact dermatitis, irritation from leaking feces or
urine, extension of underlying Crohn disease, or a wound infection. It is
primarily reported in patients with IBD.7- 11
The onset of PPG from the creation of the stoma is extremely variable. There
is no single effective therapy for PPG.
We performed a retrospective medical record analysis of 7 patients with
PPG seen between 1988 and December 1999. Patients were included if they had
peristomal ulceration(s) with a violaceous undermined border or a surface
with multiple fistulous tracts of longer than 4 weeks' duration. In each case
infection was excluded by culture results; neoplasia, extension of underlying
IBD, and vasculitis were excluded by biopsy findings. We reviewed the clinical
features, associated disorders, histopathologic features, microbiologic findings,
and the time from the creation of the stoma to the development of PPG, as
well as the time to PPG recurrence when the stoma was moved.
Two patients had ulcerative colitis, 2 had Crohn disease, and 3 had
abdominal malignancies (Table 1).
The specific type of IBD was confirmed grossly and histopathologically. Of
the patients with solid tumors, 2 had an enteric urostomy following cystectomy
for bladder carcinoma and 1 had a colostomy following proctosigmoidectomy
for rectal adenocarcinoma. Three of the 4 patients with IBD had multiple ulcers
at the time of initial presentation.
Histopathologic examination of specimens from all patients revealed
a nonspecific inflammatory reaction consisting of a mixture of neutrophils,
lymphocytes, and histiocytes. None of the biopsies revealed neoplasia, vasculitis,
or granulomatous inflammation.
The time to onset of PPG after surgery ranged from 2 months to 25 years.
Patients who had their original stoma relocated because of PPG developed an
ulceration at the site of the new stoma shortly following the surgery (Figure 1). Patient 4 had a lesion of classic
pyoderma gangrenosum on her leg 10 years prior to the development of PPG and
has had chronic polyarthritis. She had no colon or rectum at the time of either
ulcerative lesion and has had no evidence of active IBD since her colectomy,
yet PPG occurred. Three of the 4 patients with IBD had active bowel disease
in association with the occurrence or recurrence of PPG. Patient 3 had a subtotal
colectomy; following proctotectomy her lesions promptly healed and have not
recurred for more than 3 years, unlike the situation in patient 4. In patients
1 and 2, systemic antibiotics that improved the intestinal symptoms did not
lead to improvement of PPG. Patient 1 was treated with infliximab and had
a prompt and sustained response of her bowel disease. Her PPG almost completely
resolved after the third month's infusion, but recurred within 4 weeks. Five
patients have had multiple episodes of PPG.
Therapy was empiric and the responses varied (Table 1). All patients had been treated with 1 or more courses of
broad-spectrum antibiotics for a presumed skin infection prior to the diagnosis
of PPG. Topical clobetasol propionate (a class I topical corticosteroid) used
in conjunction with intralesional (dermal) injection of triamcinolone acetonide
was effective in 3 patients, ineffective in 2 patients, and possibly useful
as adjunctive therapy in 2 patients. Topical 2% cromolyn sodium solution was
effective in 1 patient and ineffective in another. Three patients required
the addition of an immunosuppressive agent (cyclosporine [n = 2] or mycophenolate
mofetil [n = 1]) because of systemic corticosteroid failure or adverse effects.
Patient 4 had repeated responses to oral dapsone with each of her 3 relapses
(Figure 1); in contrast patient
6's initial episode responded to dapsone, but a relapse failed to respond.
Classic pyoderma gangrenosum is associated with systemic disease in
half of such patients,3- 6
whereas all of our small group of patients with PPG had a systemic disorder.
Inflammatory bowel disease occurs in 15% to 20% of patients with classic pyoderma
gangrenosum, and until recently accounted for almost all patients with PPG.11,12 Tumors of the colon, bladder, prostate,
bronchus, ovary, breast, and adrenal gland have been associated with sporadic
cases of classic pyoderma gangrenosum.2 Malignancies
were the reason for stoma creation in 3 of our patients
There was wide variability in the time from formation of the enterostomy/colostomy
to the onset of PPG (2 months to 25 years).11- 13
Dermatologists consider pathergy to be a process
whereby, in susceptible persons, trauma to the skin results in pustules and/or
ulcers. Pathergy may have functioned with the debridement, grafting, and relocation
of the stoma in our patients to cause PPG. A seemingly insignificant degree
of trauma related to irritation of the adhesive of the appliance or leakage
of urine or feces may invoke pathergy, resulting in PPG in predisposed persons.
Pathergy has been suspected in up to 30% of patients with pyoderma gangrenosum,2- 5 and occurs
in other neutrophilic dermatoses including Behçet disease, Sweet syndrome,
and the blind loop syndrome.14
The PPG in 2 of our 7 patients responded to topical superpotent corticosteroids
and intralesional injection of triamcinolone acetonide. However, the majority
(6/7) required some form of systemic therapy, including dapsone, cyclosporine,
mycophenolate mofetil, or infliximab. The latter agents allowed discontinuation
of systemic corticosteroids in 4 patients with corticosteroid-induced diabetes
mellitus, cataracts, glaucoma, or facial swelling.
The approach to treatment of patients with PPG is empiric. In patients
with active IBD, measures to treat the underlying disease must be started
along with conservative local wound care and topical application of a potent
or superpotent corticosteroid. Patients without active bowel disease may try
a topical corticosteroid. In cases that fail to respond, topical cromolyn
sodium solution or tacrolimus ointment may be effective.16 After
2 to 4 weeks of local therapy without response, systemic therapy should be
considered. Prednisone, 0.5 to 1.0 mg/kg per day, is standard therapy for
pyoderma gangrenosum, whether peristomal or on other sites; however, we often
consider other agents because of the adverse effects that commonly accompany
use of systemic corticosteroids. A variety of agents can be tried, but our
first-line therapy is dapsone for 4 to 6 weeks, then cyclosporine15 or mycophenolate mofetil.
Peristomal pyoderma gangrenosum may cause serious morbidity in patients
who require placement of a stoma. The majority of our patients were initially
misdiagnosed as having a skin infection, tending to confirm our belief that
PPG is underreported.7,11