S1056 Clinical Characteristics of Metastatic Crohn’s Disease, a Rare Cutaneous Extra-Intestinal Manifestation: A Systematic Review

Introduction: Metastatic Crohn’s disease (MCD) is a rare cutaneous extra-intestinal manifestation (EIM) mimicking crohn's disease (CD). Literature concerning MCD is limited to case reports and small case series. We performed a systematic review of biopsy proven MCD cases to characterize the clinical features and risk factors of this entity. Methods: We searched Medline, Embase, Cochrane Central, and Web of Science from inception through April 2021 for English publications describing MCD. Studies were screened by four independent reviewers who then extracted study data. A skin biopsy revealing histologic features consistent with MCD was required for inclusion. Case series without individual level data were excluded. Missing data was not imputed. We summarized data with medians, ranges, and percentages. Results: In 321 included cases, MCD occurred at median of 28 years, mostly in females (65%) and within a median of 2 years (range -11; 43) from CD diagnosis. About 27% of cases occurred within 3 months of CD diagnosis. MCD lesions mostly involved genital (68%) and gluteal (18%) areas, but were multifocal in 75% of cases. Perianal MCD was present in 53/165 (32%) of patients, 29% had prior IBD-related surgeries. Most patients (59%) had non-stricturing, non-penetrating disease, especially in pediatric patients, and 61% were on no IBD therapy at time of MCD diagnosis. Peristomal MCD was present in 10% of ostomized patients. Genital lesions were predominantly characterized by ulceration and erythematous induration, non-genital areas typically were ulcers, nodules and plaques. Endoscopic CD assessment when available was active in 70% of cases, and interestingly, IBD was either not investigated or not found on endoscopy in 38/303 (12.5%). Additional EIMs were present in 13% of patients. Treatment characteristics for MCD are summarized in Table 1. Hospitalization was required in 18% of patients. Biologic (76/299; 25%) and non-biologic immunosuppressive therapies (90/299; 30%) were relatively common MCD treatments. CD long-term therapy changes occurred in 60/227 (26%) patients. MCD recurrence rate was 26%. Conclusion: MCD is rare and occurrence in genital and gluteal areas makes it a challenging diagnosis for clinicians. MCD can occur in children and adults, tends to occur early in the course of IBD, seems to parallel active CD, and typically triggers medical therapy changes to control both diseases. Table 1. - Treatment and Outcomes of MCD Therapies N (%) Steroids and any type of steroid Received treatment (%) 206 (68.9) Local 51 (25.5) PO 103 (51.5) IV (with any other form) 9 (4.5) Local + PO 37 (18.5) Median number of steroid days (Range) 36.00 [14.00, 240.00] Other treatments Colchicine 1 (0.3) Dapsone 3 (0.9) Oral retinoid 1 (0.3) Indomethacin or NSAIDs 2 (0.6) Use of antibiotics as MCD-directed therapy 103 (34.4) Tacrolimus 18 (5.6) Mesalazine 27 (8.4) Sulfasalazine 27 (8.4) Hyperbaric oxygen 3 (0.9) Azathioprine 66 (20.6) Other immunomodulator 8 (2.5) Cyclosporine 10 (3.1) Methotrexate 18 (5.6) Thalidomide 4 (1.2) Adalimumab 27 (8.4) Infliximab 51 (15.9) Ustekinumab 9 (2.8) Other biologic 7 (2.2) Surgical debridement (%) 44 (15.1) Outcomes of treatment (%) Lesion resolved 123 (56.7) Among those with biologic therapy 29 (52.7) Among those with non-biologic immunomodulators 32 (47.1) Among both biologics and non-biologic immunomodulators 14 (58.3) With neither (corticosteroids allowed) 76 (64.4) Lesion did not resolve 22 (10.1) Among those with biologic therapy 4 (7.3) Among those with non-biologic immunomodulators 9 (13.2) Among both biologics and non-biologic immunomodulators 2 (8.3) With neither (corticosteroids allowed) 11 (9.3) Spontaneous recovery without treatment 3 (1.4) Lesions improved but did not resolve 69 (31.8) Among those with biologic therapy 22 (40) Among those with non-biologic immunomodulators 26 (38.2) Among both biologics and non-biologic immunomodulators 8 (33.3) With neither (corticosteroids allowed) 29 (24.6) Recurrence (%) 57 (25.2) Among those with biologic therapy 12 (19) Among those with non-biologic immunomodulators 19 (25.7) Among both biologics and non-biologic immunomodulators 8 (28.6) With neither (corticosteroids allowed) 34 (29.1) Reason for recurrence (%) During Steroid taper 12 (22.2) After non-steroid therapy stopped 12 (22.2) After steroid therapy stopped 15 (27.8) IBD re-flare 2 (3.7) MCD not treated on first presentation 13 (24.1)