Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 15, 2009
Langerhans Cell Histiocytosis
- Langerhans cell histiocytosis is an idiopathic reticuloendothelial proliferative disorder involving the Langerhans cell, an immature dendritic cell of bone marrow origin. It describes a spectrum of disease ranging from isolated eosinophilic granuloma to disseminated Letterer-Siwe disease.
- Disease can occur at any age but most frequently develops in children less than 4 years old.
- Orbital LCH frequently occurs in the supratemporal frontal bone as stem cell precursors of Langerhans cells are located in hematopoetic bone marrow in the frontal bone. As the frontal sinus develops, the marrow space is crowded into the lateral orbital roof.
- Orbital LCH may include significant juxtaneural or intracranial extension.
- Children with evidence for orbital LCH should undergo work-up for more generalized disease.
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