Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity.

Case report: A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss. Periodontal therapy was performed with no significant improvement. An incisional biopsy revealed Langerhans cells and positive reaction to S-100 and CD1, and the patient was diagnosed with LCH. The patient underwent systemic chemotherapy with vinca alkaloids and corticosteroids. Regression of gingival lesions, as well as significant decrease in mobility of the remaining teeth and severity of pain, was achieved during 12 months of follow-up.

Conclusion: The rarity and variable system involvement of LCH necessitate a multidisciplinary approach be carried out for accurate diagnosis, effective treatment, and an uneventful follow-up. Awareness of oral manifestations of LCH may aid clinicians greatly in reducing morbidity and mortality associated with this debilitating condition.

Figure 1

(a) Intraoral view of the patient after extraction of teeth with severe mobility, prior to initiation of chemotherapy. Extensive gingival lesions are observed on the palatal surface of the maxilla. (b) Intraoral view of the patient after extraction of teeth with severe mobility, prior to initiation of chemotherapy. Diffuse, erythematous, and ulcerated gingival hyperplasia is observed in the mandible. (c) Panoramic radiograph of the patient at initial presentation. Generalized alveolar bone loss is observed. (d) Axial sections of the computed tomography scan obtained at initial presentation. Diffuse involvement of both maxilla and the mandible is observed.

Figure 2

Infiltration of the edematous stroma by polygonal cells with eosinophilic cytoplasm, oval nuclei with longitudinal grooves resembling coffee beans named as Langerhans cells, and eosinophils are observed. (a) Haematoxylin and eosin, ×200 magnification. (b) Haematoxylin and eosin, ×400 magnification. (c) Langerhans cells were highlighted immunohistochemically by S-100, ×200 magnification. (d) Langerhans cells were highlighted immunohistochemically by CD1a, ×200 magnification.

Figure 3

(a) Intraoral view of the patient 12 months after initiation of chemotherapy. Significant regression of gingival lesions is observed on the palatal surface of the maxilla. (b) Intraoral view of the patient 12 months after initiation of chemotherapy. Significant regression of gingival lesions is observed in the mandible. (c) Panoramic radiograph of the patient 12 months after initiation of chemotherapy. Periradicular bone levels of the remaining teeth are maintained except for mandibular left premolar and molar teeth, around which minimal progression of the alveolar bone loss is observed.