Langerhans Cell Granulomatosis

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