LANGERHANS CELL HISTIOCYTOSIS ASSOCIATED WITH ERDHEIN-CHESTER'S DISEASE: A RARE OCCURRENCE

Abstract

Histiocytosis, Rosai-Dorfman, and Erdhein-Chester are rare diseases caused by infiltration of histiocytes that can involve 1 or multiple organs. Diagnosis is made based on imaging and biopsy of the lesions. The immunohistochemistry profile in Langerhans cell histiocytosis includes positivity to S-100 and CD1-A, whereas in Erdhein-Chester disease, lesions are positive to CD68 and negative to S-100 and CD1-A. A 60-year-old Black female patient whose main complaint was tooth loss reported recent dental implant placement and retroperitoneal fibrosis treated with mycophenolate mofetil. On intraoral examination she had dental mobility, ulcerated lesions, and alveolar bone loss. An incisional biopsy was performed for which the diagnosis was Langerhans cell histiocytosis proven positive to S-100 and CD1-A. Due to the report of retroperitoneal fibrosis, the patient was referred to hematology, which confirmed the association between Langerhans cell histiocytosis and Erdhein-Chester disease. The patient is currently under follow-up by the hematology team.