POEMS syndrome complicated by extensive arterial thromboses.
POEMS syndrome--characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes--is a rare multi-systemic disease. Its skin change is often like that of connective tissue diseases such as scleroderma. Although clinical manifestations of POEMS syndrome are known to be diverse, arterial or venous thrombosis is a less-recognised feature. We report a 41-year-old man with scleroderma-like skin change who was initially negative for monoclonal protein, but finally confirmed as having POEMS syndrome. During the disease course, he was complicated by extensive arterial thromboses including stroke. This case evokes the need to include POEMS syndrome in the differential diagnoses in patients with scleroderma-like skin change and to repeat measurements of monoclonal protein to confirm its diagnosis. A POEMS patient with extensive arterial thromboses may have a catastrophic disease course, requiring earlier diagnosis and more aggressive treatment.
Lee
Mi Ra
MR
Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, 1198 Kuwol-dong, Namdong-gu, Incheon, 405-760, South Korea.
Choi
Hyo Jin
HJ
Lee
Eun Bong
EB
Baek
Han Joo
HJ
D000328:Adult; D001158:Arteries / Q000473:pathology*; D001921:Brain / Q000473:pathology; D002544:Cerebral Infarction / Q000175:diagnosis / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010026:Osteosclerosis / Q000175:diagnosis / Q000000981:diagnostic imaging; D016878:POEMS Syndrome / Q000175:diagnosis*; D012595:Scleroderma, Systemic / Q000175:diagnosis; D020521:Stroke / Q000175:diagnosis; D013927:Thrombosis / Q000150:complications* / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed / Q000379:methods