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Virtual Pediatric Hospital: Paediapaedia: Histiocytosis X, Hand-Schuller-Christian Paediapaedia: Musculoskeletal Diseases

Histiocytosis X, Hand-Schuller-Christian

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


Clinical Presentation:
Classic triad is diabetes insipidus, exophthalmos, and a skull lesion, but it is seen in only 10% of cases. It is a more chronic and widely disseminated type of histiocytosis that presents at 3-6 years old.

Etiology/Pathophysiology:
Etiology unknown. Due to abnormal proliferation of histiocytes throughout the reticuloendothelial system. Multi system organ involvement with 80% having skeletal lesions. Variable prognosis.

Pathology:
Not applicable

Imaging Findings:
On plain film more commonly have well defined medullary lesions with or without sclerotic margins that may have associated bone expansion, cortical destruction, periosteal reaction, soft tissue mass, and pathologic fracture.

Bone scan is useful in evaluating the disseminated form of the disease and the lesions are hot on it.

DDX:

References:
See References Chapter.

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