Langerhans' cell histiocytosis: report of a case with temporal localization

The authors report a case of Langerhans' cell histiocytosis (LCH) with temp oral localization and rapidly evolving initial clinical presentation in a 1 2-year-old boy. This disease of currently unknown etiology is actually cons idered a proliferative entity of cells with phenotypic characteristics of n ormal Langerhans' cells. An immunoregulation defect leading to abnormal mat uration and migration of Langerhans' cells might be the basis for LCH. Acco rding to the Hystiocyte Society diagnostic criteria, ATPase, S 100 and D-ma nnoxidase positivity in addition to typical hystopathologic findings are su fficient for diagnosis of LCH. Head and neck localization of LCH occurs in about 70% of cases; males are more frequently affected than females, age at presentation varies from a few months to 15 years. Presenting features, in itial diagnostic evaluation, differential diagnosis and treatment protocol of a unifocal monosystemic temporal bone localization of LCH are presented. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.