Background
Eosinophilic Granuloma (80%)
Localized benign form
Isolated to bone
Hand-Schuller-Christian disease (15-20%)
Skull lesions
Exopthalmos
Diabetes Insipidus
Letterer-Siwe disease (>10%)
Disseminated lesions involving multiple visceral organs
Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow–derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells,
including T-cells,
Reticulum cells
The bones which are the most commonly
involved are the skull,
the pelvis,
and the diaphysis of long bones.
The systemic condition includes Hand-Schüller-...
Findings and procedure details
Important points for the paediatrician and Paediatric radiologist regarding bone LCH include the following:
_ LCH should be considered in all young patients whopresent with a skull mass,
jaw pain,
swelling and/or
loose teeth,
chronic ear drainage,
with dermatitis of the auricular canal,
mastoiditis and cholesteatoma
or proptosis,
swelling and redness of the eyelid.
The classical radiologic finding is a punched-out lytic lesion in bone,
but some LCH lesions can resemble an
aggressive bone sarcoma with destruction of bone and periosteal elevation [40].
This is...
Conclusion
This exhibit illustrates the spectrum of Imagng features of LCH in its typical atypical location
References
Ardavin C.
Origin,
precursors and differentiation of mouse dendritic cells.
Nature Reviews Immunology 2003; 3: 582-590.
Caldemeyer KS,
Parks ET,
Mirowski GW.
Langerhans cell histiocytosis.
Journal of the American Academy of Dermatology 2001; 44: 509-511.
Egeler RM,
D’Angio GJ.
Langerhans cell histiocytosis.
The Journal of Pediatrics 1995; 127: 1-11.
Howarth,
DM,
Gilchrist GS,
Mullan BP,
Wiseman GA,
Edmonson JH,
Schomberg PJ.
Langerhans cell histiocytosis: Diagnosis,
natural history,
management,
and outcome.
Cancer 1999; 85: 2278-2290.
Kilborn TN,
Teh J,
Goodman TR.
Paediatric manifestations of Langerhans cell...