Skin changes in POEMS syndrome.

Correspondence: Giuseppe Longo, M.D., Department of Medical Sciences Section of Internal Medicine, University of Modena, via del Pozzo 71, 41100 Modena, Italy. Phone: international +39-059-422782 – Fax: international +39-059-424549 – E-mail: [email protected] The so-called POEMS syndrome is a multisystemic disorder characterized by the association of polyneuropathy, organomegaly, endocrinopathy and skin changes. These disorders seem to be secondary to a plasma cell dyscrasia leading to production of a monoclonal component. Several other signs can occur, e.g. anasarca, pyrexia, finger clubbing, sweating and hematologic disorders. The syndrome has been described mainly in Asians, although some Caucasian cases have been reported.1,2 We report the images of skin changes which appeared in a 39-year-old Caucasian male; all the other above mentioned signs were also present. The diagnosis of POEMS syndrome was established in April 1987. The patient developed a progressive peripheral polyneuropathy with demyelination, hepatosplenomegaly, sclerotic bone lesions, scleroderma and IgGl monoclonal gammopathy. Bone marrow examination showed about 9% plasma cells of apparently normal morphology. Two years later the patient had a myocardial infarction with transient thrombocytosis together with papilledema. A consistent improvement was obtained with plasmapheresis, chemotherapy (melphalan) and high dose prednisone. After a further three years severe polyneuropathy reappeared, with symmetrical motor and sensory deficiencies in the limbs, peripheral edema, pleural effusion, hypogonadism and hypothyroidism. Treatment with melphalan, cyclophosphamide and plasmapheresis together with prednisone was not very effective. Since September 1997 the patient has had rapid, progressive appearance of multiple skin angiomas (50-60, several tuberous), together with dermal fibrosis with sclerodactylia, pleural and myocardial effusions, pyrexia and capillary leak syndrome. A slight improvement was obtained using high dose dexamethasone and this has been maintained up to now with intermittent dexamethasone treatment.