OrthoNet

Langerhans Cell Granulomatosis

- eosinophilic granuloma - solitary lesion in long bones, pelvis, ribs & vertebrae of young kids (5-10 yrs)
- nonneoplastic proliferation of Langerhans cells associated with eosinophils & chronic inflammatory cells
- Hand-Schuller-Christian disease - systemic process involving multiple bones (esp skull) as well as liver, lungs, spleen & skin
- Letterer-Siwe disease - skeletal lesions with aggressive clinical course due to widespread involvement of skin, viscera & lymphatics

Signs & Symptoms:
- localized pain & swelling

Xrays:
- in long bones: - metaphyseal or diaphyseal lesions with endosteal scalloping
- local periosteal reaction if cortical destruction
- spinal lesions - vertebra plana (2o compression of vertebral body)

Histology:
- Langerhan’s cells - vesicular, indented nucleus with small, centrally placed nucleolus & abundant pale, granular eosinophilic cytoplasm
- arranged in sheets or clusters & surrounded by eosinophils, various chronic inflam’y cells & macrophages
- electron microscope - ‘Birbeck granules’ = racquet-shaped cytoplasmic structures

Treatment:
- depends on location of lesion
- intralesional steroid injection vs curettage & bone grafting