POEMS syndrome disclosed by a scleroderma-like skin thickening.

Background. POEMS syndrome is a rare Form of plasma-cell dyscrasia characte rized by the various association of Polyneuropathy, Organomegaly, Endocrino pathy, Monoclonal component and Skin changes. The most frequent skin change s such as hyperpigmentation, hypertrichosis, scleroderma-like skin thickeni ng and angiomas are not pathognomonic but nearly constant. However, they ar e rarely isolated. Case report. A 57-year-old Caucasian woman presented with a 6 month history of skin thickening on both hands and feel, Raynaud's phenomenon and facial telangiectasias. Physical examination on presentation revealed hepatomegal y, signs of a sensorimotor peripheral neuropathy which was demyelinating in type on electrophysiological assessment, and ankle edemas. Initial laborat ory investigations revealed a platelet count of 900 000/mm(3), a monoclonal IgG lambda gammapathy. Plasma-cells were slightly increased (10 p. 100 of marrow elements) and full skeletal radiographs showed no focal osteosclerot ic or lytic lesion. A diagnosis of POEMS syndrome was made. The patient was treated with tamoxifene, methylprednisolone and plasmapheresis without imp rovement in polyneuropathy or in skin changes. Discussion. Our patient satisfied the criteria for POEMS syndrome. The most typical feature here was the scleroderma-like skin change, which has been recognized by other authors. But, in the present case, Raynaud's phenomenon , skin thickening and facial telangiectasias were present 6 months before t he diagnosis, and initially suggestive of systemic scleroderma, confirmed h istologically. The pathogenesis of POEMS syndrome might be regarded as the result of a marked activation of the proinflammatory cytokine network, but an increase in serum Vascular Endothelial Growth Factor (VEGF) levels could well account for other manifestations such as skin thickening.