Mandibular manifestation of Langerhans cell histiocytosis in children

Abstract

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. In this article, we report a case with LCH. The history, radiological appearance, histopathology and treatment options of the patient were discussed.

Atypical Langerhans Histiocytosis in Children: About 2 Cases

“…Local complications may include pathological fracture, ocular compression and dental instability, functional outcomes are usually limited [6].…”

Mandibular Langerhans cell histiocytosis in an adult

“…More frequent is in male than in female. The reported ratio ranges from 2:1 to 4:1 (5). A skeletal lesion of LCH can occur in any bone.…”

“…Also it is referred as the disease that is most often fatal. This form usually affects infant and children younger than 3 year age (5).…”

“…The disease is characteristic by a pathologic proliferation of Langerhans cell (LCH) with accumulation of eosinofi lic granulocytes, histiocytes and macrophags (3,5). The diagnosis of LCH can be confi rmed by the presence of Birbeck granules or by immunohistochemical staging with CD-1a and S-100 protein (2,6,7,9,11).…”

“…Morphologically, LCH comprises three similar lesions. Eosinophilic granuloma, Hand-Schuller-Christians syndrome and Abl-Letterer-Siwe syndrome (5,10). The exact aetiology of LCH is still unknown.…”