2017-01-26 16:19:26 UTC

A Rare Cause of Painful Skin Rash in Crohn’s Disease

Jan. 26, 2017

What's your diagnosis for this Gastro clinical image challenge?

Question: Gastroenterology's January 2017 issue features the case of a 29-year-old man with severe colonic Crohn's disease with perianal involvement was admitted with a 1-week history of painful, nonpruritic, erythematous skin rash associated with fever, chills, myalgias and arthralgias. The lesions started as red, violaceous papules and progressed to painful plaques and nodules with some of them ulcerating into larger lesions.

The patient also reported six to seven episodes per day of large-volume, watery, nonbloody diarrhea and lower abdominal cramps. He had been experiencing frequent flares of his Crohn's over the past year complicated by rectal abscess and anal fistula requiring surgical drainage and fistulotomy. He was recently started on azathioprine after a CD flare, after being on mesalamine for several years. He also reported recent exposure to stray cats but denied any scratches or bites. He failed outpatient therapy with trimethoprim/sulfamethoxazole before presentation.

Examination revealed multiple tender plaques, nodules, pustules and ulcerated lesions on erythematous bases in an asymmetrical distribution involving the chest, abdomen, back and extremities with sparing of the palms, soles and oral cavity (Figure). He also had ocular involvement with bilateral conjunctivitis and few nodular lesions on the eyelids. Laboratory testing showed leukocytosis (white blood cell count, 25,100 cells/μL; 90 percent neutrophils; 3 percent bands) and increased inflammatory markers (C-reactive protein, 90 mg/L; erythrocyte sedimentation rate, 208 mm/h) with mild acute kidney injury (creatinine, 1.4) and normal liver function tests.

Infectious workup (blood, urine, stool and wound cultures, Clostridium difficile DNA and hepatitis A, B, and C serologies) were negative. Immunoglobulin (IgG, IgA, IgM and IgG4) and complement (C3, C4) levels were within the normal range and the celiac panel was negative. Flexible sigmoidoscopy showed severe inflammation with ulcerations in sigmoid colon consistent with severe active Crohn's colitis and fistula in the anal canal without erythema or purulent drainage.

Based on the patient’s clinical presentation and imaging, what is the likely diagnosis?
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