S2291 Severe Cutaneous Lesions in Crohn's Disease Responsive to Combination Therapy, Ustekinumab and Tofacitinib

Abstract

INTRODUCTION: Severe skin manifestations of inflammatory bowel disease specifically Crohn’s Disease can be debilitating, painful, lead to a decreased quality of life, high risk of infection and are often exceedingly difficult to treat. We present a case report of a patient with severe cutaneous granulomatous lesions in Crohn’s Disease who had failed numerous treatment regimens and was successfully treated with combination therapy, Ustekinumab and Tofacitinib. CASE DESCRIPTION/METHODS: A 40-year-old African American male with past medical history of Rheumatoid Arthritis, Uveitis, Non-Alcoholic Fatty Liver Disease, Granulomatous Dermatitis with Vasculitis and Crohn’s Disease (CD) presented to clinic for follow up. His CD involved large and small intestine and was diagnosed by colonoscopy when he was 6 years old. His course had been complicated by perianal fistulae, lymphopenia, cutaneous lesions, bowel resection, colostomy, total proctocolectomy and end ileostomy. Physical exam revealed bowel sounds present, abdomen soft, nontender. Right lower quadrant ileostomy, abdominal scarring. No lymphadenopathy and healing scars in perineum, gluteal cleft, groin, and bilateral thighs. Lab results revealed sedimentation rate (ESR) of 14, C-Reactive Protein 1.10, WBC 10, Hemoglobin/Hematocrit 14.6/44.4 and Platelets of 265. Since being diagnosed the patient’s treatment regimens have consisted of Infliximab, Adalimumab, Azathioprine and Prednisone. Most recently treatment consisted of Azathioprine and Ustekinumab for over a year with continued flares and minimal skin lesion improvement. Following the diagnosis of Granulomatous Dermatitis with Vasculitis via biopsy and two abnormal Dihydrorhodamine tests (DHR) there was high concern for Chronic Granulomatous Disease (CGD). Whole Exome Sequencing was completed for confirmation. Pending CGD results given recent literature data the decision was made to discontinue Azathioprine and begin Tofacitinib along with Ustekinumab. Patient had significant overall improvement and substantial healing of his granulomatous rash. DISCUSSION: This patient with cutaneous Crohn’s Disease had extensive disease burden with numerous prior failed treatments and demonstrated significant improvement on combination therapy with Ustekinumab and Tofacitinib. The decision was made to treat with JAK inhibitor and IL-12/IL-23 combination given recent favorable literature results as well as, the concern patient might also have CGD along with severe CD.Figure 1.: Extensive perineum cutaneous lesions prior to treatment with combination therapy.Figure 2.: Substantial improvement in perineum lesions following treatment with Ustekinumab and Tofacitinib.