Appendiceal Perforation From Muscular Thinning in Limited Cutaneous Systemic Sclerosis: A Case Report

Systemic sclerosis (SSc) is a multi-system autoimmune disease characterized by vasculopathy and fibrosis. Gastrointestinal involvement is common; however, bowel perforation is rare and carries high morbidity. We report a 77-year-old woman with limited cutaneous SSc and primary biliary cirrhosis who presented with right lower abdominal pain and cutaneous necrosis. Imaging revealed a retroperitoneal abscess contiguous with the ileocecal region. Surgery demonstrated complete transection of the appendix at its base with preserved serosal color and marked bowel wall fragility, prompting ileocecal and partial transverse colectomies with functional end-to-end anastomoses. Histology revealed muscularis propria thinning and fibrosis extending to the terminal ileum and colon, and non-inflammatory thrombotic occlusion of small- to medium-sized vessels, consistent with SSc-related vasculopathy. Postoperatively, the patient developed anastomotic leakage due to muscular thinning and died on postoperative day 22.  This case illustrates that diffuse intestinal wall fragility in SSc may lead to perforation and anastomotic failure; thus, surgeons should minimize anastomoses and consider stoma creation in such patients.