The Patient With Pyoderma Gangrenosum

The Patient With Pyoderma Gangrenosum

Maria T. Abreu, MDChief, Division of Gastroenterology

University of Miami Miller School of MedicineMiami, Florida

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Audience Question 1

Correct answer: b, 6%

What percentage of patients with IBD have dermatologic manifestations?

IBD, inflammatory bowel disease.

A. 1%

B. 6%

C. 20%

D. 50%

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Audience Question 1

Correct answer: b, 6%

What percentage of patients with IBD have dermatologic manifestations?

IBD, inflammatory bowel disease.

25%

23%

25%

27% A. 1%

B. 6%

C. 20%

D. 50%

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Audience Question 2

With which of the following therapies would you initiate treatment in a patient with PG?

6-MP, 6-mercaptopurine; AZA, azathioprine;PG, pyoderma gangrenosum; TNF, tumor necrosis factor.

A. Corticosteroids

B. Anti-TNF therapy

C. 6-MP/AZA

D. Topical tacrolimus

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Audience Question 2

With which of the following therapies would you initiate treatment in a patient with PG?

6-MP, 6-mercaptopurine; AZA, azathioprine;PG, pyoderma gangrenosum; TNF, tumor necrosis factor.

21%

23%

26%

29% A. Corticosteroids

B. Anti-TNF therapy

C. 6-MP/AZA

D. Topical tacrolimus

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Case Study: Linda

• 25-year-old female with severe colonic CD complicated by perianal disease

• No response to 6-MP or IFX

• Undergoes a diverting ileostomy due to severe perianal disease

• Develops PG around the ileostomy

• Moving the ileostomy site leads to recurrence of the PG

CD, Crohn’s disease; IFX, infliximab.

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LindaPeristomal PG

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Extraintestinal Manifestations of IBD

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Dermatologic Manifestations of IBD

•5.8% of patients with IBD had ≥1 skin manifestation (cohort of >2,000) – PG (0.75%)– Erythema nodosum (4%)– Psoriasis (up to 10%)– Cutaneous CD– Numerous other disorders (uncommon)

• Sweet’s syndrome• Epidermolysis bullosa acquisita

Farhi D, et al. Medicine (Baltimore). 2008;87:281-293.

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PGEpidemiology

• 5% of patients with UC; less common in CD– Usually pancolitis

• Pustular lesion– Evolves to an ulcer with

undermining violaceous borders

• “Pathergy” worsens with trauma

• Clinical course may be independent of IBD

• Painful

• Correlations– Black African origin

(P=0.003)– Familial history of UC

(P=0.0005)– Pancolitis (P=0.03)– Permanent stoma (P=0.002)– Eye involvement (P=0.001)– Erythema nodosum

(P<0.0001)

UC, ulcerative colitis.Farhi D, et al. Medicine (Baltimore). 2008;87:281-293.

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PGClinical Features

• Location– Lower extremity most

common– Peristomal: differentiate

from fistulas – Anywhere

• Biopsy– No pathognomonic

features

• Exclude superinfection

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Early PG

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Early PG (cont’d)

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Multiple PG LesionsMid-Stage

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Advanced PG

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Advanced PG (cont’d)

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Peristomal PG

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PGTreatment Options

•Treating extraintestinal manifestations should be part of treating the underlying CD

•Variety of options, not much data– Topical steroids/topical tacrolimus– Anti-TNF therapy– Corticosteroids– Cyclosporine or oral tacrolimus acutely– 6-MP or AZA may be needed for chronic lesions– Thalidomide

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PG HealingPre- and Post-Treatment

Pretreatment Post-Treatment

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PG HealingIdentifying Remission

No Improvement Remission

Brooklyn TN, et al. Gut. 2006;55:505-509.

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Treatment of PGIFX

a P=0.025. Brooklyn TN, et al. Gut. 2006;55:505-509.

Placebo (n=17)

IFX 5 mg/kg (n=13)

Open-label IFX 5 mg/kg (n=29)

Imp

rove

men

t at

Wee

k 2,

%

0

20

40

60

80

6

46

a

Res

ult

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%

RemissionImprovement

0

20

40

60

8069

21

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Treatment of PGCyclosporine

•11 patients with steroid-refractory PG

• IV cyclosporine administered – 4 mg/kg per day – 7 to 22 days

IV, intravenous.Friedman S, et al. Inflamm Bowel Dis. 2001;7:1-7.

•All patients experienced closure of PG– Mean time to response, 4.5 days– Mean time to closure, 1.4 months

•9 patients able to discontinue steroids

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Summary

•Extraintestinal manifestations of IBD generally mirror disease activity and may respond to treatment of underlying disease

•PG may respond to biologics and immunomodulators

•Certain skin diseases may result from treatment– Psoriasis from anti-TNF therapy– Squamous cell cancers

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LindaNext Steps

•Started on natalizumab

•No PG response

•Reversal of stoma considered