Abstract
Langerhans cell histiocytosis is a rare disease consistent in its histologic characteristics, but variable in both phenotypic presentation and prognosis. Classically, a soft tissue mass with adjacent bony destruction is often present that shows a histologic monoclonal proliferation of cells positive for CD1a, S100, and langerin (CD207). Pathognomonic Birbeck granules may be seen on transmission electron microscopy. In this review, the diagnosis, etiologies, treatments, and, most importantly, the classification schemes are discussed.
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Zachary D. Pearce, Adam S. Hassan, and Hakan Demirci declare that they have no conflict of interest. No human studies were carried out by the authors for this article. No animal studies were carried out by the authors for this article.
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Pearce, Z.D., Demirci, H., Hassan, A.S. (2015). Langerhans Cell Histiocytosis. In: Demirci, H. (eds) Orbital Inflammatory Diseases and Their Differential Diagnosis. Essentials in Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46528-8_8
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DOI: https://doi.org/10.1007/978-3-662-46528-8_8
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