Oral lesion as the initial presentation in the diagnosis of histiocytosis X: A case report with 16-month follow-up

Abstract

Introduction: Langerhans cell histiocytosis (LCH) is a rare neoplastic disease that comes from the abnormal proliferation of Langerhans cells (LCs). It presents with a broad clinical spectrum, varying from involvement of a single organ system to multisystem disease, and frequently affects children, although cases in adults have been reported. Oral manifestations of LCH are non-specific and may mimic common periodontal conditions, posing a diagnostic challenge for dental practitioners.

Case presentation: A 63-year-old female patient presented with complaints of periodontal problems. She was initially diagnosed with periodontitis, but symptoms persisted despite treatment, prompting a referral for further evaluation. Histopathological analysis, supported by immunochemistry (CD1a and S100 positivity), confirmed the diagnosis of LCH. She subsequently underwent systemic chemotherapy with vinblastine and corticosteroids.

Conclusion: LCH is a rare yet important differential diagnosis for patients presenting with persistent oral lesions, requiring a multidisciplinary approach for definitive diagnosis. Early recognition and treatment of LCH can prevent disease progression and complications.