Eosinophilic Granuloma Wheeless

Description: Discussion: - includes a group of disorders characterized by a variety of tumor like lesions, which arise from from clonal proliferation of Langerhans-type histiocytes; - most common in children less than 12 years of age (but can occur in young adults); - etiology: - occurs as a result of metabolic defects in the reticuloendothelia system; - it is sometimes non painful, unless of course a fracture occurs; - hallmark is presence of an osseous lesion (70-90%) most frequently arising in skull and femur; - osseous lesions may produce rapidly destructive bone lesions; - soft tissue masses associated w/ skeletal lesions rarely are seen, - disease sub-types: - note that intermediate forms of the disease occur and individual disease presentations may change from one form to another; - Histiocytosis X; - Hand Schuller Christian disease - occurs in children> 3 yrs; - traid of skull lesions, exophthalmos, & diabetes insipidus; - a minority of patients will have wide spread viceral involvement; - cranial lesions are always present in this disease; - Letterer Siwe dz; - represents more acute manifestations of histiocytosis & generally has an age of onset of less than 3 yrs; - look for recurrent bacteremia, diffuse lymphadenopathy, & skin lesions; - disease is commonly fatal; - poor prognostic signs: - involvement at young age, rapid disease progression, organ involvement (eg pituitary, lung, hematopoietic, or liver involvement); - organ dysfunction carries an especially poor prognosis;