BACKGROUND: Calciphylaxis is a syndrome consisting of vascular calcification, 
thrombosis, and skin necrosis. The syndrome develops often in chronic 
hemodialysis patients. However, there have been several case reports on 
calciphylaxis in patients with POEMS (polyneuropathy, organomegaly, 
endocrinopathy, M-protein, and skin changes) syndrome, a systemic disease 
associated with plasma cell dyscrasia and upregulation of vascular endothelial 
growth factor (VEGF).
METHODS: In 76 POEMS patients and 86 age- and gender-matched disease controls, 
we studied abnormal small vessel calcification by computed tomography (CT) of 
the soft tissues. Clinical and laboratory profiles were compared between POEMS 
patients with and without calciphylaxis. Histological examination was performed 
in six autopsy cases.
RESULTS: Small vessel calcification on CT was found in 17 % of POEMS patients 
and in none of the controls (P < 0.001). Autopsy confirmed calciphylaxis in 2 
(33 %) patients. Among POEMS patients, higher disease activity, including more 
severe neuropathy and ascites, higher serum levels of interleukin-6, and lower 
serum albumin levels, was associated with the development of calciphylaxis. 
Serum levels of creatinine, calcium, and phosphate were not related to the 
presence of calciphylaxis.
CONCLUSIONS: Calciphylaxis is often present in patients with POEMS syndrome. 
Upregulation of multiple inflammatory cytokines such as VEGF and interleukin-6 
may contribute to the development of calciphylaxis, by entirely different 
mechanism from that in chronic dialysis. POEMS syndrome should be recognized as 
a potential cause of calciphylaxis.