Background: Kaposi's sarcoma (KS) is a vascular tumor arising in association with human herpesvirus-8 (HHV-8) infection, and different variants show different clinical presentations. Isolated intestinal KS in the background of Crohn's disease (CD) is exceedingly rare with only 3 cases reported in the English literature (from 1966 to 2016).
Case presentation: Herein, we report a case of intestinal KS in a 21-year-old HIV-negative Ethiopian male with a long-standing history of steroid therapy for his underlying IgA nephropathy. Recent gastrointestinal biopsies confirmed an additional diagnosis of CD. Despite the addition of Infliximab to his therapy, his CD remained refractory, and a laparoscopic-assisted ileocolectomy was performed to alleviate a partial small bowel obstruction. Examination of his terminal ileum demonstrated a polypoid mass with adjacent incidental ileal submucosal nodules. These nodules were composed of plump spindle cells with scattered mitoses and vascular channels with extravasated red blood cells. Intratumoral hyaline globules were also noted. Immunohistochemistry revealed HHV-8 positivity, confirming the histologic impression of KS.
Conclusions: Here we report the fourth case of KS in CD in an HIV-negative patient and only the third case of isolated intestinal KS in the setting of CD. A review of the literature suggests that attenuation of immunosuppressive therapy may be adequate management of iatrogenic KS in the absence of a systemic HHV-8 infection.
Keywords: Crohn’s disease; HIV; Kaposi sarcoma–associated herpesvirus; Kaposi’s sarcoma; inflammatory bowel disease; ulcerative colitis.