Langerhans Histiocytosis in a Child – Diagnosed by Oral Manifestations

Abstract

Langerhans Histiocytosis (LCH) is a rare reactive and proliferative disease of histiocytes with unknown etiology, characterized by excessive proliferation of histiocytes called Langerhans cells. It occurs mainly in children but occurrence in adults has also been reported. It manifests as punched out lesions in the skull, maxilla, mandible, sternum and other flat bones and causes rapid resorption of the alveolar bone leading to floating teeth appearance in the radiographs. This disease manifests initially in the oral cavity in most of the cases and can be diagnosed by careful clinical and radiological examination. Here, we present a case of LCH in a child which was diagnosed by a swelling in the mandibular region.

Langerhans Cell Histiocytosis in a 5-Year-Old Girl: A Case Report and Review of Literature

“…The chronic and disseminated form of LCH termed as the Hand-Schuller-Christian syndrome consists of skeletal and extraskeletal lesions usually affecting children The Letterer-Siwe syndrome is a disseminated acute or subacute form of LCH, that is, most often fatal because of extensive skeletal and extraskeletal lesions; this form usually affects infants and children below 3 years of age. [3,4] The present case represents monostotic eosinophilic granuloma of the mandible.…”

“…[2] Since the proliferating histiocytes involved in LCH are phenotypically similar to that of the Langerhans cells found in the normal mucosa and skin, the condition was named as LCH. [3,6] Various hypotheses have been proposed explaining the etiology of LCH. The presence of inclusion bodies resembling the Birbeck granules of Langerhans cells classifies LCH as a proliferative disorder of the Langerhans cells or their marrow derivatives.…”

“…The accumulation of the pathological Langerhans cells causes infiltration and destruction of the local tissues. [3] The incidence of LCH is relatively low with an estimation of 2-5 cases per million per year. [1] LCH mainly comprises of three morphologically similar lesions, namely eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe syndrome classified by Lichtenstein in the year 1953.…”

“…[1] LCH mainly comprises of three morphologically similar lesions, namely eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe syndrome classified by Lichtenstein in the year 1953. [3,4] The cause of this disease is not clearly known; however, there is evidence that the disorder is a presentation of an immunological variation. [5] In this article, we report a case of LCH involving the mandible with an overt swelling of one side of the face which was diagnosed with the help of imaging modalities and histopathological investigation.…”

Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

“…The rate peak is among children aged 5 to 15 ( 8 ). LCH is more common in males compared to females ( 9 ). LCH can involve a single lesion or multiple organs such as bone, skin, lung, spleen and liver ( 5 , 10 ).…”