Aggressive and atypical manifestations of Erdheim-Chester disease.

Abstract:

:Erdheim-Chester disease (ECD) is a disseminated non-Langerhans' cell histiocytosis with multisystem involvement, including characteristic sclerotic musculoskeletal lesions. We present the case of a 27-year-old woman with a fulminant course and atypical involvement by ECD manifesting as extensive cerebrovascular disease and lytic musculoskeletal lesions. This case represents an unusual and aggressive presentation of ECD owing to the patient's young age, the severity of the cerebrovascular involvement and the lytic osseous lesions.

journal_name

Clin Rheumatol

journal_title

Clinical rheumatology

authors

Lyders EM,Kaushik S,Perez-Berenguer J,Henry DA

doi

10.1007/s10067-003-0784-4

subject

Has Abstract

pub_date

2003-12-01 00:00:00

pages

464-6

issue

6

eissn

0770-3198

issn

1434-9949

journal_volume

22

pub_type

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