Double Subclavian Steal Syndrome As Initial Manifestation of Erdheim-Chester Disease: A Case Report.

Erdheim-Chester disease (ECD) is a histiocytosis that infiltrates all organs, but especially large arteries such as the aorta and its branches, which become sheathed and lead to multiple stenoses or even occlusions. Subclavian steal syndrome (SSS) has not been reported in ECD. A 68-year-old female was diagnosed with ECD due to the typical sheathing of the aorta and its major branches. Five years previously, asymptomatic SSS due to stenosis of the left subclavian artery was incidentally diagnosed. In the following years, occlusion of the subclavian and left vertebral artery, and stenosis of basilar artery and right middle cerebral artery occurred. The abnormal cerebral perfusion had consequences on the perfusion of the left upper extremity and was presumably responsible for falls. Basilar and middle cerebral artery stenosis is rare in ECD and vertebral artery occlusion and double subclavian steal have not been reported in ECD. This case is the first to show that the initial manifestation of ECD can be unilateral SSS and that subclavian artery occlusion can even lead to double SSS. Patients with SSS should undergo a thorough diagnostic evaluation to detect rare causes of SSS, such as ECD.