Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed.We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD.
Aim Significant heterogeneity exists in the recommendations for the diagnosis and management of acute lower gastrointestinal bleeding (LGIB). This study aims to demonstrate the contemporary management strategies adopted by a busy tertiary hospital. Methods We conducted a single-centre retrospective review of adults presenting to our institution with LGIB between January and March 2021. Demographic data, observations, laboratory results, endoscopy and radiology reports, operation notes and discharge summaries were collected for each patient via a retrospectively maintained registry. Results 23 patients were included. Mean age was 66.6 years with a mean initial haemoglobin of 10.9 g/dL (Range 4.1–15.5g/dL). 17% of patients were in circulatory shock on presentation. 70% of patients presented with haematochezia. 47.8% of patients were on either an anticoagulant or anti-platelet agent at presentation. The transfusion index in this cohort was 30%. 26.1% of patients had a re-bleed during their admission. The diagnostic yield of cross-sectional imaging amongst 11 patients was 27.3% with no correlation with haemodynamic status or active bleeding. 47.8% of patients underwent endoscopy and 27% of patients who underwent an endoscopic procedure required endoscopic haemostasis. Flexible sigmoidoscopy was the commonest endoscopic modality (55%), followed by gastroscopy (27%) and colonoscopy (18%). No patients required surgical or radiological intervention. No cause of bleeding was identified in 26% of patients. Conclusion This study confirms the variation in diagnosis and management of LGIB. Despite access to onsite interventional radiology, endoscopy remained the predominant diagnostic and therapeutic modality. Routine cross-sectional imaging may not be justified given its suboptimal diagnostic yield.
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