A case report of an Erdheim-Chester patient focused on pain management

Abstract

Introduction: Erdheim-Chester disease (ECD) is a rare and difficult-to-treat non-Langerhans cell histiocytosis characterized by the excessive production and accumulation of histiocytes. This study reports a case of ECD, emphasizing both its diagnosis, assessment and treatment of the pain associated with the disease.

Case Report: Six years ago, a 39-year-old male patient presented with generalized pain of moderate intensity in the lower limbs that involved periods of greater intensity associated with ambulation. The diagnosis of histiocytosis associated with panhypopituitarism and adrenal insufficiency was proposed. For a specific diagnosis, a bone lesion biopsy was performed, revealing the presence of histiocytic proliferation that was CD1 negative, S100 protein positive, and CD68 negative. Therefore, the diagnosis of non-Langerhans histiocytosis known as ECD was confirmed. During the two years that followed, the patient presented with severe bone pain, particularly in the lower limbs and cranial vault, and the pain subsided to a certain extent with the use of tramadol and paracetamol. Because of the pain, the patient was unable to walk and became bedridden As the patient remained in severe pain, even after the administration of morphine, the opioid was changed from morphine (60mg/day) to oxycodone (30mg/day) for a convenient dosing schedule; furthermore, the oxycodone dosage was scheduled to increase to 40mg/day that same week. The patient experienced significant pain reduction, requiring rescue analgesia only once or twice a week.

Conclusion: To the best of our knowledge, this is the first case report on the characterization and treatment of pain specific to ECD, and we highlight that the patient had a good response to treatment.