Abstract
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children. The objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare our case with previously reported series. We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and also an indurated swelling of posterior face of the left shoulder on pathological fracture. A cerebro-orbital MRI performed at home showed an extra-conical process with extension at the temporal fossa. The patient, then, underwent an open-air biopsy of the temporal mass and the pathological analysis returned to favor Langerhans cell histiocytosis. The diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The child was treated by chemotherapy with a good outcome. Our results concord with literature. The prognosis is different and better in children with unifocal bone disease than those with a multifocal disease with a survival rate of 65% - 100%.
Highlights
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children
We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and an indurated swelling of posterior face of the left shoulder on pathological fracture
We report the case of a young 4-year-old Moroccan patient with a history of otorrhea and recurrent otalgia treated by antibiotics without improvement
Summary
Langerhans cell histiocytosis (LCH), previously called histiocytosis X, was first described by Alfred Hand, Jr. in 1893 and described a spectrum of proliferation. Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells. It is characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone. LCH usually occurs in young children with a peak at the age of 1 - 4 years. The spheno-temporal localization is rare in children [2]. The temporal bone invasion is approximately 15% to 60% of cases [3]. That usually delayed the diagnosis [4]
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