An overlooked case of mandibular histiocytosis: Case report and review of the literature

Atypical Langerhans Histiocytosis in Children: About 2 Cases

“…The Histiocyte Society proposes a classification based on the age, the number and topography of lesions [4]: Type 1: unifocal involvement Type 2: Multi-focal involvement without liver, splenic, pulmonary or spinal cord injury, and aged 2 years and over. Type 3: Multi-focal involvement with at least one impairment of the following four organs:liver, spleen, lung, marrow, or less than 2 years old.…”

“…We distinguish multiple clinical entities with a common histological substratum, localized eosinophilic granuloma (isolated benign lesion), Letterer-Siwe disease (rapidly progressive multi-organ involvement ) and Hand Schuller's disease. Christian (progressive condition with diabetes insipidus, intracranial bone deficiencies and exophthalmos) and transition forms which associates multiples lesions and locations [4].…”

“…The treatment of LCH is still depends on the extent and the severity of disease at diagnosis. The LCH Study Group adopted a stratification system, which separated LCH patients into two major categories [4,9]: 1. 'Single-system' LCH: subdivided further into single and multiple sites.…”

“…The Histiocyte Society considers an initial 6-week course of therapy with vinblastine and prednisone to be effective treatment with minimal toxicity and is therefore suggested for all patients with MS-LCH; regardless of risk organ involvement [4,10].…”