Unexpected finding of encapsulating peritoneal sclerosis: mind the cocoon

Here, we present a fatal case of a man in his 40s with encapsulating peritoneal sclerosis (EPS). In retrospect, a spot diagnosis on the abdominal CT scan. The patient presented with progressive abdominal complaints of pain and vomiting over the last 2 months. He had a history of therapy-refractory sarcoidosis. A CT scan was performed, and loculated ascites was noted. The patient was admitted with suspected spontaneous peritoneal peritonitis but did not improve on antibiotics. Data from the microbiology, pathology and radiology investigations lead to no diagnosis. Eventually, laparoscopy revealed the diagnosis of EPS. Treatment with tamoxifen to counteract fibrosis and a pulse dose of methylprednisolone was started. Unfortunately, the patient developed a bowel perforation and died. In retrospect, the radiological sign of a cocoon encasing the small bowels should have been a clue to an early diagnosis.