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

Histiocytosis X, Letterer Siwe

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


Clinical Presentation:
Onset in first year. Have seborrheic rash, otitis media, lymphadenopathy, hepatosplenomegaly.

Etiology/Pathophysiology:
Etiology unknown. Due to abnormal proliferation of histiocytes throughout the reticuloendothelial system. Any organ system may be involved. This is the acute disseminated form involving the bone marrow, lymphatics, and solid organs. There may be no skeletal lesions. Prognosis is poor.

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

Imaging Findings:
May have generalized osteopenia with decreased cortical thickness if the disease is in a disseminated form.

DDX:

References:
See References Chapter.

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