A 6-Year-Old Boy With Fever and Bone Swelling: Osmosis USMLE Study Question

March 06, 2020

Langerhans cell histiocytosis (LCH) is a neoproliferative disorder of Langerhans cells, the dendritic cells of the skin the mucosa. It often involves osteolytic bone lesions and invasion of multiple organs. There are tennis racket-shaped inclusions on blood smears, which are known as Birbeck granules, and these are pathognomonic for LCH. Children younger than three years are more likely to present with multiorgan disease, whereas older children and adults are more likely to have a slower course and present with single system disease.

The patient in this case has a subtype of LCH known as Hans-Schuller-Christian disease, and it's associated with a triad of lytic bone lesions (often in the cranium), diabetes insipidus from primary stalk infiltration, and exophthalmos.

Major Takeaway: LCH often involves osteolytic bone lesions and invasion of multiple organs. Birbeck granules are pathognomonic for LCH, and children younger than three years are more likely to present with multiorgan disease.

Read more about LCH.

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