Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease.

Abstract

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.

• 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
• Journal: Practical neurology (Pract Neurol) (May 09 2022) ISSN: 1474-7766 [Electronic] England
• PMID: 35534197 (Publication Type: Journal Article)
• Copyright: © Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.