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Virtual Pediatric Hospital: Paediapaedia: Histiocytosis X, Letterer Siwe Paediapaedia: Musculoskeletal Diseases

Histiocytosis X, Eosinophilic Granuloma

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Seen in older children. Can present in vertebra with platyspondyly, so called vertebra plana.

Etiology/Pathophysiology:
Etiology unknown. Is a nondisseminated form of histiocytosis with localized proliferation of histiocytes. localized to the skeletal system. Good prognosis.

Pathology:
See an abnormal proliferation of histiocytes throughout the reticuloendothelial system.

Imaging Findings:
Usually monostotic. See a well defined intramedullary defect in tubular and flat bones without sclerosis around it and a circumferential cortical reaction without evidence of cortical destruction or soft tissue mass although if it gets big enough it can have sclerotic periosteal reaction around it. In the spine, it classically presents as vertebral body collapse (platyspondyly).

DDX:
Tumor like lesions of bone producing lytic defects with well defined margins:

References:
See References Chapter.

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