Keywords
Erdheim Chester disease, tamponade, BRAF, interferon alpha
Abstract
Erdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosis and a very rare multisystemic disease of unknown aetiology, with skeletal involvement of the long bones and in more than 50% of cases with extraskeletal involvement. The disease was described in 1930 by the anatomopathologist Jakob Erdheim and his student William Chester. More than 500 cases have since been reported. We report the case of a 77-year-old Italian woman with ECD who was admitted to hospital for acute cardiac tamponade. The patient presented with simultaneous cutaneous, retro-orbital, skeletal, cerebral and cardiovascular manifestations and was successfully treated with corticosteroids followed by interferon.
References
Views: 1032
HTML downloads: 283
PDF downloads: 455
Untitled downloads: 0
Untitled downloads: 0
Figure 2 downloads: 0
Figure 1 downloads: 0
Published:
2016-11-28
Issue:
Vol. 3 No. 7
(view)