An unusual manifestation of Langerhans cell histiocytosis in the gastrointestinal tract

Rev Esp Enferm Dig. 2024 Jan 29. doi: 10.17235/reed.2024.10152/2023. Online ahead of print.

Abstract

Langerhans cell histiocytosis (LCH) is a monoclonal proliferative disease that can affect multiple organs. It is a rare disorder, and children are the most commonly affected. Its classification depends on whether the disease is localized (usually bone or skin) or systemic. We present the case of a 49-year-old woman with a previous diagnosis of LCH in 2018 with only cutaneous involvement, managed with topical corticosteroids. After developing hypertransaminasemia, a PET-CT scan was performed, showing dissemination of the disease with bone, hepatosplenic and gynecological involvement. In addition, hypermetabolic lesions were described in the cecum with ileocecal adenopathies. A colonoscopy was performed showing in the cecum and ascending colon multiple sessile polyps of 5-10mm with central fibrin-coated erosions, from which biopsies were obtained. Histology revealed typical features of LCH with positive staining for CD1A, S100 and Langerin.