Atypical jaw swelling in children: An unusual clinical spectrum of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH), also called histiocytosis X or eosinophilic granuloma, is a local or systemic unusual clonal proliferative disorder of Langerhans cells. It has a wide spectrum of clinical presentations and can occur in any age group with predominance in children and young adults. This article presents a rare case of LCH in a 2-year-old girl child which was provisionally diagnosed as an infection of the jaw bone of unknown etiology. Correlating the clinical, radiologic, and histologic features with immunohistochemical analysis aided in arriving at a definitive diagnosis of LCH. Oral manifestations being the earliest presentation seen in around 5%-75% of LCH cases emphasize the role of a dentist in the early detection of this lesion. A myriad of clinical spectra due to pathologic infiltration of Langerhans cells into various systems necessitates oral diagnosis to be followed by a full body scan to detect any systemic involvement. Advanced targeted therapies can improve the survival rate and quality of life in patients with LCH.