The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, 
and skin changes) syndrome is a rare variant of plasma cell dyscrasia with 
multisystemic manifestations. We present 4 cases with arterial symptoms typical 
of acute arterial obliteration (AAO) and review 9 similar cases in the 
literature. The clinical course of AAO was unusual and particularly severe when 
affecting the lower limbs; recurrent events required amputations. As 
demonstrated by angiographic and histologic studies, thrombotic and atheromatous 
lesions were the main pathologic features of AAO. Atherosclerotic risk factors 
were absent or moderate in 3 of our cases, and no cause of thrombosis other than 
the POEMS syndrome was found. A high production of cytokines was found in all 
cases, with elevated serum levels of interleukin-1 beta (9/9 samples), 
interleukin-6 (7/9 samples), and tumor necrosis factor-alpha (6/9 samples). We 
suggest that arterial manifestations should be added to the spectrum of 
manifestations of the POEMS syndrome. Cytokines may mediate the POEMS 
syndrome-associated AAO, as previously proposed for the other systemic 
manifestations of this disorder.