A unique case of polyostotic Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a relatively rare dendritic cell disorder with an unclear etiology. The incidence is 2:1 million and it is most commonly seen in children from 5 to 10 years of age. The diagnosis is confirmed by biopsy with treatment options varying from simple observation to systemic chemotherapy. The four-year event-free survival for patients with solitary bone disease approaches 90% compared with 58% of those with polyostotic bone disease.