Indian Journal of Dental Research

: 2019  |  Volume : 30  |  Issue : 1  |  Page : 157-

The langerhans cell histiocytosis in oral and maxillofacial area

Konstantinos Natsis1, Maria Piagkou2,  
1 Department of Anatomy and Surgical Anatomy, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece
2 Department of Anatomy and Surgical Anatomy, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

Correspondence Address:
Maria Piagkou
Department of Anatomy and Surgical Anatomy, School of Medicine, National and Kapodistrian University of Athens, Athens,

How to cite this article:
Natsis K, Piagkou M. The langerhans cell histiocytosis in oral and maxillofacial area.Indian J Dent Res 2019;30:157-157

How to cite this URL:
Natsis K, Piagkou M. The langerhans cell histiocytosis in oral and maxillofacial area. Indian J Dent Res [serial online] 2019 [cited 2020 Jul 14 ];30:157-157
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We would like to highlight some interest points of the recently published paper by Divya.[1] The Langerhans cell histiocytosis (LCH) earlier known as “histiocytosis X” includes three variants: The eosinophilic granuloma (EG) (unifocal LCH with a solitary bone lesion), the Hand–Schuller–Christian (multifocal LCH with the triad-skull lesions, exophthalmos and diabetes insipidus) and the Letterer-Siwe (fulminant LCH with multiple organ involvement) disease.[2] The LCH is a rare disorder, mostly affecting infants and children with a wide range of clinical manifestations from self-limiting to life threatening. Children under 2 years of age suffer more commonly from the multisystem disease.[3]

The etiology of LCH remains unknown and is still uncertain whether the disease is a neoplastic or a reactive disorder resulting from dysregulation of the immune system.[4] Emphasis should be given on diagnostic standards. The point that must be clarified is that the biopsy is necessary, and the definitely diagnostic confirmation is obtained after the morphologic identification of Langerhans cells and the positive immunohistochemical staining with S-100 and CD1a and/or CD207.[5]

In children, single or multiple bones are usually affected, and the unifocal involvement (EG) is more common than the multifocal disease.[4] A predilection exists for the flat bones (skull, ribs and pelvis). The calvarium (parietal and frontal bones) is affected more often than the skull base (temporal bone).[6] In jaws, the severe alveolar bone destruction may appear as radiolucency on the orthopantomograph with the characteristic “floating teeth” image.[1] In the long bones, the femur, humerus and tibia are affected in order of frequency.[2]

Conventional radiography remains the most effective diagnostic tool for children suspected to have LCH, despite the fact that some skeletal areas may be difficult to assess due to the bone's superposition (skull base, scapula and spine) or the gas presence in the intestine (pelvis) and even though that the lytic lesions become apparent only after 30-50% of the bone density loss. After the diagnosis of the osteolytic lesion, computed tomography, or magnetic resonance imaging may be necessary to assess the degree of trabecular and cortical bone destruction or the soft tissue infiltration. Finally, caution should be given on the variability of the radiographic features in children with LCH that may mimic Ewing sarcoma or osteomyelitis. The LCH of skeletal involvement in children is very similar to that seen in adults with multiple myelomas (destructive lesions in the axial skeleton).[6]

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