[POEMS syndrome revealed by a scleroderma-like skin thickening]

Abstract

Background: POEMS syndrome is a rare form of plasma-cell dyscrasia characterized by the various association of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes. The most frequent skin changes such as hyperpigmentation, hypertrichosis, scleroderma-like skin thickening and angiomas are not pathognomonic but nearly constant. However, they are rarely isolated.

Case report: A 57-year-old Caucasian woman presented with a 6 month history of skin thickening on both hands and feet, Raynaud's phenomenon and facial telangiectasias. Physical examination on presentation revealed hepatomegaly, signs of a sensorimotor peripheral neuropathy which was demyelinating in type on electrophysiological assessment, and ankle edemas. Initial laboratory 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 osteosclerotic or lytic lesion. A diagnosis of POEMS syndrome was made. The patient was treated with tamoxifen, methylprednisolone and plasmapheresis without improvement 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 the diagnosis, and initially suggestive of systemic scleroderma, confirmed histologically. 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.