Multidisciplinary approach in a case of Hand-Schüller-Christian disease with maxillary involvement

Langerhans cell histiocytosis (LCH) is a childhood pathology with a peak of incidence ranging from 1 to 4 years of age, though diagnosis is often made in adult age. LCH is clinically classified into three types: eosinophilic granuloma, Hand-Schuller-Christian disease and Abt-Letterer-Siwe disease. We report a case of Hand-Schüller-Christian disease with diabetes insipidus, skull and maxillary involvement in a 16-year-old boy referred to our observation for gradual increase in mobility of the teeth and subsequent gradual loss of the second premolars and the first molars of the upper jaw. Due to the extension of the lesion and the age of the patient, surgery, and chemotherapy was chosen as the more fit treatment according to the current protocol. The clinical and radiological evaluation at the end of the therapy and after 5 years showed complete remission. The absence of relapse has allowed to initiate a fixed orthodontic dental alignment treatment with a good response to orthodontic treatment despite the underlying disease. The present case exemplifies the importance of close multidisciplinary dental and medical collaboration including general dentistry, periodontology, oral medicine, oral and maxillofacial pathology, oral radiology, orthodontics and hematology-oncology for diagnosis, management, treatment monitoring, and decision-making.