부산대학교 도서관

The histiocytoses are a group of rare disorders characterised by the accumulation of neoplastic or non- neoplastic activated histiocytes in various tissues. Phenotypes vary widely from cutaneous lesions or lymphadenopathy that regress spontaneously to disseminated disease with poor prognosis. Neurological symptoms can be a presenting feature or appear during the course of disease. We present a challenging diagnostic and management case of Rosai- Dorfman- Destombes disease in a 48- year- old woman with a relapsing, partially steroid- responsive syndrome comprising patchy, non- length- dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen- activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai- Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim- Chester disease. [ABSTRACT FROM AUTHOR]