Hand-Schüler-Christian disease presenting with recurrent, bilateral, symmetrical mandibular lesions in an 8-year-old boy: report of a case.

INTRODUCTION

Hand-Schüller-Christian disease (HSC) is the unisystem multifocal form of Langerhans Cell Histiocytosis (LCH) and is primarily seen in infants and children.

CASE REPORT: An 8-year-old boy was referred for acute mandibular pain. His medical history included otic LCH and diabetes insipidus at age of 11 months. Intraorally, a pressure sensitive swelling, and radiographically, extensive bone loss were revealed on the area of lower second primary molars. The primary molars were extracted and histological examination confirmed the final diagnosis of HSC. The patient was treated with chemotherapy and the lesions decreased considerably. Two years later, a new swelling was recorded in the same area bilaterally. Biopsy confirmed recurrent HSC disease and the patient entered an alternative chemotherapy protocol. Six months later, improvement of the lesions was revealed.

CONCLUSIONS

Dentists can contribute to a timely and valid identification of HSC disease by correctly differentially diagnosing lesions of head and neck.