Oral Langerhans cell histiocytosis in Malaysian children: a 40-year experience

Background.  Oral Langerhans cell histiocytosis is generally seen in children.
Objective.  To determine the clinicopathological features of oral LCH in Malaysian paediatric patients.
Methods.  A retrospective study was carried out to determine the clinicopathological features of Langerhans cell histiocytosis (LCH), Letterer–Siwe disease, Hand–Schuller–Christian disease, eosinophilic granuloma, and histiocytosis X occurring in the oral cavity in children, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2007.
Result.  There were 17 cases (eight girls and nine boys) with age ranging from 1 to 7 years. There were ten Malays, four Chinese, two Indians, and one of other ethnicity. Thirteen cases presented as gingival swellings with six of these cases accompanied with mobility of the teeth. Nine cases involved the mandible, two in the maxilla, and two cases in both the maxilla and mandible. The radiographic findings were mentioned only in nine cases with presence of bony erosion or destruction of the jaw bones. Four cases had punched-out radiolucencies of the skull. The patients also had other systemic signs and symptoms: skin lesions ( n  = 5), hepatosplenomegaly ( n  = 2), prolonged fever ( n  = 2), diabetes insipidus ( n  = 1), and exophthalmos ( n  = 1). Two cases were known cases of systemic LCH.
Conclusion.  The histopathologic features of LCH are easily recognized; however, with the development of immunostaining, the use of CD1a helps in confirming the diagnosis.