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

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  • 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 Ulcerativecolitis 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. KusunokiDepartment 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 ofthe anal ulcer. b An anal ulcerwas found along the entire

    circumference of the IPAA.

    c The endoscopic examinationrevealed no abnormality of the

    ileal mucosa in either the ileal

    pouch or pre-pouch ileum.

    d The anal ulcer disappearedafter steroid therapy

    Surg Today

    123

  • 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 patients 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 todisclose.

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    Pyoderma gangrenosum occurring at the peri-ileal pouch-anal anastomosis in a patient with ulcerative colitis: report of a caseAbstractCase reportDiscussionConflict of interestReferences

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