Pyoderma gangrenosum in Crohn\'s disease: Report of a case

Pyoderma Gangrenosum in Crohn's Disease" Report "IV~7ILLIAM C. ~ I c G A R I T Y ,

of a Case*

M.D.,J-

STEPHEN M . BARNETT,

M.D.++

From the Emory University School of Medicine, Atlanta, Georgia

PYODERMA GANGRENOSUM, a r a r e progressive cutaneous lesion associated with idiopathic ulcerative colitis, 3 usually occurs on the extremities, and rarely on the trunk. 4 T h e lesions appear as small, painless, dark vesicles that enlarge rapidly and ulcerate, generally, in patients with active colitis. However, pyoderma gangrenosum does not reflect the severity or duration of the disease.2, 4 Although therapeutic responses to sulfonamides, corticosteroids, and azulfidine have been reported, complete healing can be expected with resolution of the colitis. 1 Primary skin grafting after debridement is usually unsuccessful. 2 Total colectomy with ileostomy is generally recommended for patients who have chronic idiopathic ulcerative colitis and pyoderma gangrenosum. T h e following case, however, apparently' represents an exception to the usual course of this disease process.

blood transfusions a n d corticosteroids were a d m i n istered. T h e gastrointestinal s y m p t o m s were alleviated a n d the abscess of the b u t t o c k healed. In April 1971, t h e p a t i e n t was r e a d m i t t e d for persistent n o n b l o o d y d i a r r h e a a n d a rectovaginal fistula. Sigmoidoscopy revealed patchy e r y t h e m a of the rectal mucosa. B a r i u m - e n e m a e x a m i n a t i o n showed mucosal ulcerations f r o m t h e cecum to t h e rectosigmoid a n d reduced d i a m e t e r of the colon. No ileal disease was f o u n d . T h e radiologic diagnosis at this time was ulcerative colitis, a n d t h e p a t i e n t was treated w i t h azulfidine a n d prednisone. H e r condition improved. In A u g u s t 1972, the p a t i e n t was hospitalized for vesicular lesions of the lower extremities. T h e r e was no history of t r a u m a . Vesicular lesions w i t h black centers a n d e r y t h e m a t o u s m a r g i n s were present o n b o t h legs, a n d cultures of t h e p u r u l e n t m a t e r i a l that e x u d e d from these areas grew Staphylococcus aureus a n d Aerobacter. Anaerobes were not present. T h e diagnosis was p y o d e r m a g a n g r e n o s u m . T o p i c a l w o u n d care with corticosteroid cream, systemic corticosteroid, a n d e r y t h r o m y c i n resulted in g r a d u a l i m p r o v e m e n t . T h e lesions healed in several weeks. T h e p a t i e n t c o n t i n u e d to have eight to ten stools daily a n d a feculent vaginal discharge from the rectovaginal fistula. In J a n u a r y 1974, the p a t i e n t was hospitalized for a small ulcer of the a b d o m i n a l wall below the umbilicus a n d severe p a i n in t h e knees, ankles, a n d left great toe. T h e colitis s y m p t o m s were simultaneously increased. W a r m t e n d e r effusions of b o t h knees were present. H e m o g l o b i n was 4 g/100 m l , total protein 4.0 g/100 m l with 1.1 g/100 m l a l b u min. A n t i n u c l e a r a n t i b o d y test, l u p u s erythematosus p r e p a r a t i o n , a n d r h e u m a t o i d arthritis latex test were negative. Uric acid was 5.0 rag/100 ml. T h e abdominal-wail ulcer was 0.5 to 1.0 cm in diameter, the m a r g i n s e r y t h e m a t o u s , a n d the center dark. D u r i n g the n e x t two days the lesion rapidly enlarged to 10 • 12 cm a n d looked m u c h like a synergistic infection of t h e a b d o m i n a l wall (Fig. 1). Aerobic a n d anaerobic cultures, i n c l u d i n g direct cultures of tissue, revealed no growth. Meanwhile, gentamicin a n d c l i n d a m y c i n were a d m i n i s t e r e d systemically, a n d N e o s p o r i n e | spray was a p p l i e d topically. T h e p a t i e n t h a d been receiving t r e a t m e n t

R e p o r t of a Case A 53-year-old white w o m e n was first a d m i t t e d to the hospital in December 1970 for acute colitis, characterized by 10 to 15 blood), bowel m o v e m e n t s per day, c r a m p i n g lower a b d o m i n a l pain, weight loss, a n d anemia. Proctoscopic e x a m i n a t i o n demonstrated friable beefy red rectal mucosa. An abscess of the left buttock was drained, a n d cultures of the w o u n d grew n o enteric pathogens. W h o l e * Received for publication May 17, 1976. 5-Joseph 13. W h i t e h e a d D e p a r t m e n t of Surgery. $ Emory University Affiliated Hospital Residency T r a i n i n g Program. Address r e p r i n t requests to Dr. McGarity: E m o r y University Clinic, 1365 Clifton Road, N.E., Atlanta, Georgia 30322.

49 Dis. CoL & Rect. Jan.-Feh. 1977

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McGARITY AND B A R N E T T

Fro. 1. Abdominal-wall ulcer, between the umbilicus and the symphysis pubis, 10 • 12 cm in diameter, with irregular necrotic margins.

Dis. Col. & Rect. J-am-Feb. 1977

material in their wake. A diag'nosis of pyoderma gangrenosum was established and confirmed by biopsy. Perivascular lymphocytic and mononucleocytic invasion, as well as transdermal invasion by lymphocytes, was described. Colectomy was im-possible through the large infected lesion of the abdominal wall. Radical wide excision of all involved tissue of the lower abdominal wall down to the rectus fascia was performed. Normal tissue was included in the margins of the resection (Fig. 2). T h e total abdominal-wall defect was 15 X 20 cm in diameter. A week later, a split-thickness skin graft was applied over the abdominal-wall defect (Fig. 3). While the abdominal wail was healing, additional purulent vesicular lesions developed on the right ankle and left great toe. T h e y were drained and debrided. T h e lesions had the gross appearance of pyoderma gangrenosum. Cultures grew no bacteria. A m o n t h after complete healing of the abdominal wail, total colectomy, abdominoperineal resection, and ileos'tomy were carried out. T h e incision was made directly through the grafted area, and both abdomina] and perineal wounds healed without difficulty (Fig. 4). T h e lesions on the toe and ankle similarly healed. T h e arthritis of the knees and ankles resolved after severaI weeks. All wounds remained well healed, and the patient was totally asymptomatic a year after operation. Microscopic evaluation of the coIon demonstrated extensive involvement with transmural inflammatory invasion. T h e final diagnosis was transmural granulomatous colitis (Crohn's disease). Comment

with dine The lent

systemically administered steroids and azulfifor four months, and these were discontinued. center of the lesion remained black, with purudischarge; the expanding margins left necrotic

The

case of this patient

rather

unusual

course

derma

gangrenosum.

demonstrated

of colitis with The

patient

had

a

pyotwo

FIG. 2. Excised abdominal-wall ulcer, 15 X 20 cm in diameter, with normal tissue included in the margins.

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Fro. 8. Above, Abdominalwall defect; below, split-thickness skin graft over abdominalwall defect.

separate episodes of pyoderma during her chronic colitis, of which the first resolved with steroids and azulfidine. T h e second episode, however, occurred while the patient was taking these drugs. Colectomy was thought to be necessary because of the fulminating colitis and pyoderma gangrenosum; however, the large abdominal-wall ulcerating lesion prohibited transabdominal operation. Wide, deep, total excision of the ulcerating lesion, with a 3-cm margin

of normal surrounding tissue and delayed split-thickness skin waft, proved successful. This procedure enabled colectomy shortly thereafter. When associated with colitis, pyoderma gangrenosum is thought to occur only with idiopathic ulcerative colitis. This patient's original diagnosis was ulcerative colitis; however, with the development of a rectovaginal fistula, Crohn's disease should have been suspected. T h e m!croscopic studies of

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cosal and transmural colitis represent the ends of one spectrum of disease. Summary

Fro. 4. Abdominal wall two months after skin graft and one month after total colectomy and ileostomy.

A case of pyoderma gangrenosum of the abdominal wall occurring in a patient with histologically proven Crohn's disease of the colon is described. An aggressive surgical approach (wide excision with delayed skin grafting) resulted in rapid healing of the abdominal wall. Subsequent colectomy through the previously involved abdominal site was accomplished without complication. References

the colon confirmed the diagnosis as Crohn's disease. A h h o u g h pyoderma gangrenosum is thought to occur exclusively in patients who have idiopathic ulcerative colitis, this case represents the second recording of pyoderma gangrenosum in a patient with Crohn's disease. 3 Indeed, this clinical problem lends credence to the notion that mu-

1. Braverman IM: Skin Signs of Systemic Disease. Philadelphia, W. B. Saunders Company, 1970, p 331 2. Spiro HM: Clinical Gastroenterology. London, Collier-MacMillan Limited, 1970, p 659 3. Verbov JL: The skin in patients with Crohn's disease and ulcerative colitis. Trans St Johns Hosp Dermatol Soc 59: 30, 1973 4. Vokurka V, Micanek B: Pyoderma in the course of ulcerative colitis. Gastroenterologia 99: 311, 1963