Erdheim-Chester Disease

Abstract

P i c t u r e 1 . Ye l l o wi s h -b r o wn a n d s y mme t r i c a l l y d i s t r i b u t e d l e s i o n s o v e r b o t h u p p e r e y e l i d s a n d i n f r a o r b i t a l r e g i o n s . P i c t u r e 2 . Ab n o r ma l l y i n c r e a s e d r a d i o t r a c e r a c t i v i t y wa

Erdheim-Chester Disease of the Central Nervous System: New Manifestations of a Rare Disease

“…5 The clinical course varies widely, depending on the extent of disease, with some patients never developing symptoms and other patients succumbing after rapid disease progression. 2,6 Commonly reported causes of death in patients with ECD include renal failure, cardiomyopathy, and respiratory failure. 7 Neurologic involvement is encountered in fewer than 50% of patients with proved ECD, 8 with the most common locations including the HPA, brain parenchyma, orbits, meninges, and paranasal sinuses.…”