Langerhans cell histiocytosis oral manifestation: A case report

Abstract

Introduction and importance: Langerhans cell histiocytosis is a rare disorder characterized by the proliferation of Langerhans cells in various tissues. While it commonly affects bones, skin, and other organs, oral manifestations are less frequent but can be diagnostically challenging.

Case presentation: we present a clinical case of LCH with an oral manifestation in a 14-year-old adolescent. The patient presented with progressive jaw pain for approximately two months.

Clinical: Intraoral examination revealed vestibular swelling at the mandibular angle, with painful vestibular palpation. However, the adjacent teeth to the lesion were not mobile, and the vitality test was positive. Radiographic examination showed an inhomogeneous hypodense osteolytic lesion at the left mandibular angle. Histopathological analysis confirmed the diagnosis of LCH with diffuse infiltration of Langerhans cells in the gingival tissue. The patient was referred to Hospital in Marseille and therapeutic abstention is chosen, clinical and radiological follow-up is established every 6 months for 5 years. At 6 weeks, a significant decrease in the lesion and an osteoperiosteal reconstruction phenomenon are observed. After one year, the lesion has spontaneously resolved. Clinical discussion: The disease activity score allows defining the severity and activity of the disease and determine the treatment. The clinical presentation of Langerhans cell histiocytosis is highly variable. The severe form of the disease occurs before the age of 2 and is characterized by multi-system involvement and neurodegenerative involvement.

Conclusion: The lesion resolved spontaneously after the biopsy was performed. Prompt diagnosis and multidisciplinary management are crucial for successful treatment outcomes in such cases. This case highlights the importance of recognizing oral manifestations of LCH in dental practice for early diagnosis and appropriate management.

Keywords: Case report; Langerhans cell histiocytosis; Mandible; Oral surgery.

Fig. 1

Photograph of the exobuccal examination.

Fig. 2

Photograph of the endobuccal examination.

Fig. 3

Cone beam computed tomography, axial mandibular sections.

Fig. 4

Histological section of the biopsy from the left mandibular angle.

Fig. 5

Immunohistochemistry staining with toluidine blue for cellular markers CD1a (A), immunohistochemistry staining for cellular markers CD207 (B).

Fig. 6

Cone beam computed tomography, axial mandibular sections at 6 months of follow up (A), cone beam computed tomography, axial mandibular sections at one year of follow up (B).