Langerhans cell histiocytosis: Current concepts in dentistry and case report

Abstract

Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease.

Key words: Children, dental management, histiocytosis, Langerhans cells.

Figure 1

Frontal and lateral radiograph of the skull showing a punched-out lesion in the frontal and temporal bone (arrows). A 3-dimensional CAD view of cranial bones of the same patient demonstrating osteolytic lesions (arrows).

Figure 2

Extraoral view.

Figure 3

a) Frontal intraoral view; b) Intraoral view of maxillary arch; c) Intraoral view of mandibular arch.