Conflict of interest: none declared.
Skin sclerosis as a manifestation of POEMS syndrome
Article first published online: 23 JUN 2012
© 2012 Japanese Dermatological Association
The Journal of Dermatology
Volume 39, Issue 11, pages 922–926, November 2012
How to Cite
HASEGAWA, M., ORITO, H., YAMAMOTO, K., MATSUSHITA, T., HAMAGUCHI, Y., FUJIMOTO, M. and TAKEHARA, K. (2012), Skin sclerosis as a manifestation of POEMS syndrome. The Journal of Dermatology, 39: 922–926. doi: 10.1111/j.1346-8138.2012.01609.x
- Issue published online: 29 OCT 2012
- Article first published online: 23 JUN 2012
- Received 11 April 2012; accepted 16 May 2012.
- Crow–Fukase syndrome;
- POEMS syndrome;
- skin sclerosis;
- systemic sclerosis
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.