Aggressive and atypical manifestations of Erdheim-Chester disease.
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.
Lyders
Eric M
EM
Medical College of Virginia Hospital, Virginia Commonwealth University, Richmond, VA 23298-0615, USA.
Kaushik
Shaifali
S
Perez-Berenguer
Juan
J
Henry
Daniel A
DA
D000328:Adult; D001707:Biopsy, Needle; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000175:diagnosis*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D010014:Osteolysis / Q000209:etiology* / Q000473:pathology; D035583:Rare Diseases; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed