CASE REPORT

Pyoderma gangrenosum occurring at the peri-ileal pouch-analanastomosis in a patient with ulcerative colitis: report of a case

Koji Tanaka • Toshimitsu Araki • Yoshiki Okita •

Hiroyuki Fujikawa • Mikio Kawamura •

Keiichi Uchida • Yasuhiko Mohri • Masato Kusunoki

Received: 25 April 2012 / Accepted: 3 October 2012

� Springer Japan 2012

Abstract Pyoderma gangrenosum (PG) is an idiopathic,

inflammatory, ulcerative skin disorder; the etiology and

pathogenesis of which are still poorly understood. It is one

of the most important extraintestinal manifestations that

can appear in the course of ulcerative colitis (UC).

Although skin ulcers with destructive and necrotizing

components are more commonly observed on the lower

extremities and trunk, PG at stomal sites (peri-stomal PG)

has been increasingly reported in patients with inflamma-

tory bowel diseases (IBDs), including UC. Although PG at

various surgical sites has been reported as an unusual

presentation of PG, postoperative PG developing at the

ileal pouch-anal anastomosis (IPAA) has not been reported

previously. We herein report the first published case of a

patient with UC who developed peri-anastomotic PG after

IPAA during the postoperative tapering of systemic

corticosteroids.

Keywords Pyoderma gangrenosum � Ulcerative

colitis � Ileal pouch-anal anastomosis

Case report

A 48-year-old female who had been diagnosed with ste-

roid-dependent chronic active UC 21 years prior was

referred to our department for surgical intervention. Med-

ical treatment had been initiated 21 years prior upon the

diagnosis of left-sided UC. Her past medical history

included corticosteroids, azathioprine and infliximab. She

had developed sepsis due to azathioprine-induced pancy-

topenia. The use of infliximab therapy to reduce the steroid

dose also failed. Corticosteroids were used not only to

control disease flare-ups but also to maintain remission.

She was being maintained on prednisolone (20 mg/day) at

the time of admission. She had steroid-dependent since her

diagnosis of UC, and the total steroid dosage was estimated

to be approximately 30,000 mg. However, she had no

apparent steroid-related complications before surgery.

She underwent a total proctocolectomy and ileal pouch-

anal anastomosis (IPAA) with loop ileostomy in June 2011.

Her postoperative course was uneventful. Intravenous

cortisol was administered at 200 mg/day perioperatively

for steroid coverage. Oral prednisolone was administered at

10 mg/day for weeks, and then tapered to 7.5 mg/day for

weeks.

Three months later, she visited our outpatient clinic

because of a rapidly increasing severe pain at her anus

which had begun a few days before her visit. She was on

oral prednisolone at 5 mg/day. On physical examination,

anal ulceration was found along the entire circumference of

the IPAA (Fig. 1a, b). Subsequent endoscopy revealed no

abnormality of the ileal mucosa in either the ileal pouch or

pre-pouch ileum (Fig. 1c). The ulcer was characterized by

an undermined border and a purulent necrotic base, which

was located at the anal skin without the involvement of the

ileal pouch mucosa. There was no anastomotic leakage

clinically or radiographically. There was no infectious or

neoplastic cause associated with this ulceration. She had no

skin disorders related to UC or steroid use prior to surgery.

Based on her history of associated systemic disease

(UC), her typical clinical presentation (a characteristic

painful skin ulcer) and the exclusion of other diseases, we

K. Tanaka (&) � T. Araki � Y. Okita � H. Fujikawa �M. Kawamura � K. Uchida � Y. Mohri � M. Kusunoki

Department of Gastrointestinal and Pediatric Surgery,

Mie University Graduate School of Medicine,

2-174 Edobashi, Tsu, Mie 514-8507, Japan

e-mail: qouji@clin.medic.mie-u.ac.jp

123

Surg Today

DOI 10.1007/s00595-012-0463-7

considered this ulcerated lesion to be PG occurring at the

peri-IPAA. She had no ulcerated lesions at the peri-ileos-

tomy site or other surgical sites. Her lower extremities and

trunk, typical sites of PG, were also normal.

Both systemic (oral prednisolone at 40 mg/day) and

topical steroid therapy were initiated for her perianastomotic

PG. She responded rapidly to the steroid therapy. The anal

ulcer was completely healed weeks after the beginning of

steroid therapy. As a consequence, the anal ulcer and pain

disappeared (Fig. 1d). The dose of prednisolone was reduced

to 30 mg/day for week, and then tapered to 20 mg/day for an

additional week. The steroid dose was gradually reduced

thereafter. On oral prednisolone at 10 mg/day, the patient

had no recurrence of peri-IPAA PG at the month follow-up

examination. Radiological examination of the pouch using a

water-soluble contrast agent indicated no evidence of leaks

of the pouch or anastomosis, stricture of anastomosis or

abnormality of the evacuation status. Accordingly, we plan

to close the loop ileostomy.

Discussion

In 1930, Brunsting reported the first five patients with PG,

who presented with rapidly progressive and painful sup-

purative skin ulcers with necrotic and undermined borders

[1]. To date, the etiology and pathogenesis of PG are still

poorly understood [2, 3]. PG is also known to be one of the

most important extraintestinal manifestations of UC, the

frequency of which has been reported to vary between 1

and 10 % [4, 5].

Since the initial report, PG has been described at various

sites in the literature, although it classically occurs on the

lower legs, trunk and upper extremities and at peri-stomal

sites. PG can occur anywhere on the body. Unusual pre-

sentations of PG related to surgical sites have also been

reported in patients who have undergone splenectomy,

cesarean section, gastrectomy tube insertion, pacemaker

insertion and coronary artery bypass grafting [6].

In our series, a total of 246 consecutive UC patients with

total proctocolectomy and IPAA were identified between

September 2000 and December 2011. PG was observed in

12 (4.9 %) of these 246 patients. Four patients were

diagnosed with PG before surgery, and eight had peristo-

mal PG after surgery. This is the only case of peri-IPAA

PG in our experience. To the best of our knowledge, this is

also the first report of postoperative peri-IPAA PG in UC in

the literature.

The precise etiology and pathogenesis of PG remain

unclear. The association of PG with autoimmune disorders

and the successful response to immunosuppressant agents

suggest that immunological disturbances may play an

Fig. 1 a Macroscopic view of

the anal ulcer. b An anal ulcer

was found along the entire

circumference of the IPAA.

c The endoscopic examination

revealed no abnormality of the

ileal mucosa in either the ileal

pouch or pre-pouch ileum.

d The anal ulcer disappeared

after steroid therapy

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important role in the pathogenesis of PG. Moreover, the

response to therapy (immunosuppressants) is one of the

important factors for the diagnosis of PG, in addition to the

clinical history, clinical features, ulcer features and asso-

ciated diseases [3].

Accordingly, the ulcerated lesion at the peri-IPAA site

presented in the present report was diagnosed as PG based

on its response to steroids, the patient’s clinical history,

the clinical features and the ulcer features, although its

anatomical location was extremely unusual. The pathergy

phenomenon after surgical trauma may be associated with

the pathogenesis of both peri-stomal PG and postoperative

peri-IPAA PG. In our case, the peri-IPAA PG appeared to

develop during the steroid tapering after total proctoco-

lectomy for steroid-dependent UC. PG can occur before,

during, or after the onset of UC. Whether the disease

activity of UC is related to PG is controversial. Poritz and

colleagues [7] reported that UC patients with residual

disease in the rectum after total colectomy experienced

peristomal PG just after the end of their steroid taper.

They also showed that the UC patients whose peristomal

PG resolved with infliximab had recurrence during the

drug withdrawal. These findings suggest that the devel-

opment or recurrence of PG may be associated with the

withdrawal or reduction of treatment, such as steroids or

infliximab.

The mainstay of the management of PG is immuno-

suppression. Currently, there is no standard treatment or

guideline for PG, since multiple therapeutic options, such

as corticosteroids, cyclosporine, azathioprine, cyclophos-

phamide and tacrolimus have been used without uniform

efficacy. Recently, several case reports and randomized

trials have demonstrated a favorable response of patients

to anti-TNF-a drugs, including infliximab [8, 9], etaner-

cept [10] and adalimumab [11, 12] or topical tacrolimus

[13, 14].

Although a causal relationship between the occurrence

of PG and the reduction of steroids after restorative proc-

tocolectomy has not been established, careful steroid

tapering for patients with steroid-dependent UC should be

considered [15]. An optimal steroid taper regimen might

prevent the occurrence of PG in such cases.

Conflict of interest The authors have no conflict of interest to

disclose.

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