Langerhans histiocytosis is a non- malignant proliferative disease that involves Langerhans dendritic cells. It is an orphan disease mainly affecting children and young adults. Its etiology still remains unknown, its clinical aspect quite broad. We report a case of multifocal pauci sign involvement in a 5-year-old child having affected the humerus, femur and left frontal bone. The diagnosis was confirmed by histological examination. The evolution was favorable after surgical treatment associated with polychemotherapy. Based on this observation, we will review the literature to focus on the clinical, histological, radiological, therapeutic and progressive aspects of this orphan disease.
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Gomes et al. (2024) studied this question.
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