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


METHODS
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.


CONCLUSIONS
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.
Toussaint
P
P
Service de Dermatologie, Hôpital Pellegrin, 33076 Bordeaux Cedex.
Sibaud
V
V
Labbe
L
L
Geniaux
M
M
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D010956:Plasmapheresis; D012595:Scleroderma, Systemic / Q000175:diagnosis*; D020845:Selective Estrogen Receptor Modulators / Q000627:therapeutic use; D012867:Skin / Q000473:pathology*; D013629:Tamoxifen / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D020845:Selective Estrogen Receptor Modulators; D013629:Tamoxifen; D008775:Methylprednisolone