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Year : 2019  |  Volume : 30  |  Issue : 1  |  Page : 157
The langerhans cell histiocytosis in oral and maxillofacial area

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

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Date of Web Publication20-Mar-2019

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

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 2021 Jun 14];30:157. Available from:


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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Conflicts of interest

There are no conflicts of interest.

   References Top

Divya KS. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation. Indian J Dent Res 2014;25:228-30.  Back to cited text no. 1
[PUBMED]  [Full text]  
Stull MA, Kransdorf MJ, Devaney KO. Langerhans cell histiocytosis of bone. Radiographics 1992;12:801-23.  Back to cited text no. 2
Martin A, Macmillan S, Murphy D, Carachi R. Langerhans cell histiocytosis: 23 years' paediatric experience highlights severe long-term sequelae. Scott Med J 2014.  Back to cited text no. 3
Weitzman S, Egeler RM. Langerhans cell histiocytosis: Update for the pediatrician. Curr Opin Pediatr 2008;20:23-9.  Back to cited text no. 4
Satter EK, High WA. Langerhans cell histiocytosis: A review of the current recommendations of the Histiocyte Society. Pediatr Dermatol 2008;25:291-5.  Back to cited text no. 5
Khung S, Budzik JF, Amzallag-Bellenger E, Lambilliote A, Soto Ares G, Cotten A, et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging 2013;4:569-79.  Back to cited text no. 6

Correspondence Address:
Maria Piagkou
Department of Anatomy and Surgical Anatomy, School of Medicine, National and Kapodistrian University of Athens, Athens,
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.254522

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