91桃色

Abstract

Langerhans cell histiocytosis (LCH) is a group of three rare diseases (annual incidence less than 4/1000.000 H) with increasing aggressiveness respectively: Eosinophilic granuloma (unifocal solitary osteolytic lesion), Hand-Schuller-Christian disease (multifocal lesion) and Letterer-Siwe disease (the most aggressive, disseminated disease with systemic manifestations). Th e bone is most commonly involved with a predilection for the skull. Some cases of temporal bone localizations have been described in the literature. We report a case of an 18-month child presenting a LCH of the temporal bone misdiagnosed at the beginning as osteoarthritis. In fact, the child presented a retro-auricular swelling with infl ammatory characters, fever and otorrhea. Th e exploration before surgery revealed besides the extensive temporal lesion, hematologic perturbations, a splenomegaly and a hepatomegaly. Th e histopathology confi rmed the diagnosis of a LCH. We describe the diagnosis challenges, the surgical diffi culties and the therapeutic response of the child.

Biography