Abstract
Histiocytoses are heterogeneous diseases characterized by the accumulation of histiocytes in various organs. The detection of somatic molecular alterations in some of these led to a new classification, published in 2016. In this review, we describe bone involvement of L-group histiocytosis (Langerhans-cell histiocytosis (LCH) and Erdheim-Chester disease (ECD)), and Destombes-Rosai-Dorfman disease (RDD). The osseous lesions of LCH consist of unilateral or multifocal, hypermetabolic lesions on bone C-computed tomography (CT)-scan or 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET), mainly affecting flat bones and vertebrae, and more rarely long bones, with a risk of fracture. ECD bone involvement is characterized by metaphyso-diaphyseal sclerotic, hypermetabolic lesions, present in more than 90 % of cases, and constituting an iconic feature of this disease. Limbs bone pain may be suggestive of ECD, and is sometimes the first sign of the disease. RDD is characterized by multifocal bone involvement of the limbs or spine, associated in typical forms with node involvement. Treatments options vary for these 3 types of histiocytoses, and range from abstention to chemotherapy in LCH, from immunomodulatory treatments to targeted therapies in ECD, and from abstention, corticosteroid therapy, immunosuppressive drugs, to chemotherapies in RDD.
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