Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations
2009
Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and
abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic
manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms
develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schuller-Christian disease (disseminated
chronic form) and eosinophilic granuloma (localized chronic form).
LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions
accompanied by adenopathies and/or periodontal lesions, presenting gingival inflammation, bleeding, recession,
necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnoses include
advanced periodontal disease or a periapical process of dental or periodontal origin.
The odontologist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing
routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind
that these may be the first or only signs of LCH.
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