The diagnosis of LCH can be challenging. A pathological diagnosis is needed for confirmation. Langerhans cells are present to varying degrees within individual specimens. Other inflammatory cells are also present. Over time, the primary site may show fibrosis predominating the lesion. The presence of the Langerhans cell on microscopy suggests the diagnosis, but immunohistochemical staining for S-100 or CD1a is needed to confirm the diagnosis. The presence of Birbeck granules, which can only be identified via EM, can also confirm the diagnosis. Once the diagnosis is made, a comprehensive evaluation, including a skeletal survey and basic laboratory evaluation, are important (2,3).

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