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Langerhans Cell Disease - A Case Report

Introduction: Langerhans Cell Disease (LCD), formerly known as Histiocytosis X, is a rare disorder characterized by a proliferation of cells exhibiting phenotypic characteristics of langerhans cells. The etiology and pathogenesis of this disease is unknown. The disease is categorized according to the extent of involved organs. Oral manifestations may be the first and the only sign of the disease. A number of signs can be clinically observed such as periodontal involvement, oral ulcers, gingival bleeding, mobility and premature exfoliation of the teeth or gingival enlargement. In this paper, a case of langerhans cell disease with oral manifestations has been presented. Results: A 6-year old boy was referred with a chief complaint of progressive luxation of posterior teeth. Intraoral examination revealed gingival enlargement with teeth loosening in posterior areas of the jaws. Our clinical diagnosis was Langerhans cell disease. In panoramic radiography, multiple well-defined radiolucencies were observed in both maxilla and mandible. Skull X-ray was normal. Histopathologic examination and SI00 protein staining confirmed our diagnosis. The patient was referred to pediatric oncologist and underwent chemotherapy. In follow up examinations we noticed regression of gingival lesions. Chemotherapy was stopped after one year of treatment. Conclusion: Since early diagnosis and treatment of this disease is important in the prognosis, dentists should be aware of oral manifestations and differential diagnosis of this disease. In patients in whom the only manifestation of LCD is oral involvement, early diagnosis and referring of the patient by dental practitioners for proper management is very important.