Raro caso di istiocitosi multifocaler a cellule di Langerhans. Case Report.

INTRODUCTION: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue. It often affects children and young men. Histological and immunohistochemistry analysis are essential to obtain the correct diagnosis. CASE REPORT: A 29 years-old Caucasian man is referred to the Oral Surgery Unit of George Eastman Hospital of “Sapienza” – University of Rome with third degree mobility of teeth belonging to second, third and sixth sexstant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left mandible and on the left maxilla. The presence of osteolytic lesions were confirmed by a subsequent CT scan. Incisional biopsy of the lesion with histological and immunohistochemistry analysis revealed Birbeck bodies, S100 protein and CD1a antigen positive markers. According to these findings the final diagnosis of multifocal Langerhans cell histiocytosis of the oral cavity was made. The patient was sent to the Hematology department of Umberto I Teaching Hospital of “Sapienza” – University of Rome for the appropriate treatment. DISCUSSION: A brief literature review conducted by the authors reveals that Langerhans cell histiocytosis is common in children and that in about the 50% of cases it presents itself as a unifocal lesion involving the mandibular bone. Other findings in the most recent literature suggest that LCH can also have clinical appearance of hypertrophic gingivitis, premature tooth loss and oral ulcers; root resorption and tooth displacement are considered more uncommon clinical presentations. The present case of rare multifocal LCH only involving oral hard tissues shows the strong importance of better investigate, with appropriate additional exams, initial shifty symptoms that could lead to a misdiagnosis. CONCLUSIONS: In this case the absence of oral manifestations except teeth mobility showed the importance to better investigate unusual findings such as third degree teeth mobility in a young patient with no history of periodontitis performing radiograph examinations and histological and immunohistochemistry analysis. The correct integration of the clinical and radiographic data allows the clinician to reach the final diagnosis. Early diagnosis of LCH is important to improve the patient's prognosis and quality of life.