Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes 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.PMID:35534197 | DOI:10.1136/practneurol-2022-003398

Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research