罗赛-多夫曼病
组织细胞
埃尔德海姆-切斯特病
组织细胞增多症
医学
疾病
病理
神经学
朗格汉斯细胞组织细胞增多症
皮肤病科
精神科
作者
Antonia S Carroll,Carolynne M Doherty,Julian Blake,Stephen Hunt,Chandrashekar Hoskote,Feargal McNicholl,Rahul Phadke,Oonagh Sheehy,Fion Bremner,Shirley D’Sa,Christopher McNamara,Mary Reilly
出处
期刊:Practical Neurology
[BMJ]
日期:2022-05-09
卷期号:22 (5): 396-406
标识
DOI:10.1136/practneurol-2022-003398
摘要
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.
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