“ Formerly known as histiocytosis X, this is a disease of immune regulation resulting in excessive histiocytes and granuloma formation . It ...
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Formerly known as histiocytosis X, this is a disease of immune regulation resulting in excessive histiocytes and granuloma formation. It is more common in children and causes varying effects from eosinophilic granuloma (single/few bones affected), Hand-Schuller-Christian disease (children aged 1-5 years, multiple bones and extra-skeletal involvement) and the most severe Letterer-Siwe disease (multisystem disease, children aged <2 years and often fatal).
PLAIN FILM
• 50% of bone lesions occur in the skull, mandible, ribs or pelvis.
• Lucent lesion with periostitis (appear aggressive early on).
• Look for endosteal scalloping and soft tissue nodules.
• Vertebra plana —LCH is the most common cause in children.
• Lesions may resolve completely or appear sclerotic.”
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