Abstract
Ustekinumab (UST) is an effective treatment for Crohn’s disease (CD). Here we present two cases of leukocytoclastic vasculitis (LCV) in CD patients after UST induction therapy with a review of the literature.
Patient #1: A 26 year old woman with a 14 year history of Crohn’s ileocolitis. She was previously treated with 6-mercaptopurine, infliximab, and vedolizumab without durable response. She ultimately underwent a left hemicolectomy due to development of a sigmoid stricture and was started on UST postoperatively. Thirty-six days after her initial UST intravenous (IV) infusion of 390 milligrams (mg) she developed new partially blanching, erythematous, non-tender, non-pruritic macules and papules over the right medial thigh, which later evolved into palpable purpura involving both lower extremities (Figure 1A). She reported no gastrointestinal (GI) or other symptoms. White blood cell (WBC) count and metabolic panel were normal. Antinuclear antibody (ANA) titer was 1:80. Perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) were positive. C-reactive protein (CRP) was elevated to 41.5 mg/liter (L). One lesion was biopsied, and pathology findings were consistent with LCV (Figure 1B). She was started on colchicine 0.6 mg daily with improvement in her rash and has received her second dose of UST without further complications.
Patient #2: A 29 year old woman with a 6 year history of Crohn’s ileocolitis. Her prior treatments include budesonide, mesalamine, and infliximab. While on infliximab, she developed jejunal and ileal ulcers on video capsule endoscopy. Infliximab was discontinued, and UST was started. Three months following initial IV induction therapy of UST, she developed pink purpuric papules and hemorrhagic vesicles involving her bilateral shins, dorsal feet, calves, and bilateral extensor forearms. The appearance of the rash was consistent with LCV (Figure 2). She was started on prednisone at 60 mg daily for 1 week with taper resulting in complete resolution of her rash. UST was continued with no recurrence. WBC count and metabolic panel were normal. ANCA was negative, and ANA titer was 1:80. CRP was elevated to 13.2 mg/L.
Literature review revealed only one prior published case report of a patient with inflammatory bowel disease developing LCV after administration of UST. In this case, UST was discontinued since re-administration caused the rash to return. Here we have two patients who developed LCV soon after initiating UST therapy, although after treatment with prednisone/colchicine, there was no recurrence with continuing UST. It is possible to develop LCV in association with CD alone; however, these patients had longstanding disease without occurrence of LCV until being started on UST. Moreover, patient #1, was in remission from a Crohn’s disease standpoint at the time of UST initiation and onset of the rash.
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