We report a case of a 64-year-old man with POEMS (polyneuropathy, organomegaly, 
endocrinopathy, monoclonal gammopathy and skin changes) syndrome that had been 
previously misdiagnosed as systemic sclerosis. He had typical symptoms of POEMS 
syndrome, however, the existence of skin sclerosis, contracture of fingers and 
pigmentation were similar to that of systemic sclerosis. Ten patients, including 
the patient discussed in this case, visited our department between 1990 and 
2011. Among them, five patients had skin sclerosis. Therefore, we compared skin 
lesions and clinical/laboratory features of POEMS syndrome and systemic 
sclerosis in an attempt to distinguish these disorders. Regarding the cutaneous 
and laboratory findings, the existence of hemangioma or hypertrichosis is 
indicative of POEMS syndrome. By contrast, the existence of systemic 
sclerosis-specific autoantibodies, nail fold bleeding, digital ulcer/digital 
pitting scar or telangiectasia is highly suggestive of systemic sclerosis. To 
our knowledge, this is the first report to discuss in detail the differentiation 
between POEMS syndrome and systemic sclerosis.