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POEMS syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.1,2⇓ Although no accepted set of criteria is available for the diagnosis of POEMS syndrome, most authors agree that at least three of the features summarized in the acronym need to be fulfilled for the diagnosis.2 Although the etiology is still unclear, vascular factors,3,4⇓ proinflammatory cytokines such as tumor necrosis factor alpha (TNF-α) and interleukin 6 (IL-6),5 and paraprotein specificity for neuroendocrine tissue6 have been suggested to play a causative role. We report an associated inflammatory myopathy in a patient with POEMS syndrome.
Case report.
A 59-year-old man was admitted to our hospital because of progressive weakness of his arms and legs and thickening of the skin of both hands during the previous 6 months. He had also noted a brownish coloration of the skin, and had fluctuating difficulties in breathing and swallowing. Physical examination revealed bilateral exophthalmos, an enlarged thyroid gland, gynecomastia, and a marked hyperpigmentation of the skin with sclerodermatoform changes especially of the elbows, hands, and mouth. His face displayed telangiectasis and small angiomas. Additionally he had bilateral edema of the lower legs. He did not have papilledema, lymphadenopathy, or organomegaly. Neurologic examination displayed proximal weakness (Medical Research Council [MRC] grade 2) and atrophy with reduced tendon reflexes of the legs. He was unable to elevate his arms above his head or to lift himself from a chair without help. The motility of his hands and fingers was severely impaired by sclerodermatoform skin changes. Dorsiflexion of the feet was weak (MRC grade 4). Sensory examination revealed mildly (wrist) to …
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