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Plasma Cell Dyscrasia with Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, and Skin Changes
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1980
Year
Plasma Cell DyscrasiaPathologySkin HyperpigmentationDermatologyOsteoporosisOrthopaedic SurgeryCsf ProteinBone DiseaseSurgical PathologyNeuropathologyConnective Tissue DiseaseAutoimmune DiseaseHistopathologySkin ChangesDermatopathologySclerodermaGeneral PathologyM ProteinWound HealingMedicineEndocrine Disease
Two patients with plasma cell dyscrasias, manifested by osteosclerotic bone lesions and small amounts of M protein, and a complicating multi-system disorder are described. Their features of severe sensory-motor polyneuropathy, organomegaly, endocrine dysfunction, anasarca, elevated CSF protein, and skin hyperpigmentation are similar to a clinical syndrome reported primarily in Japanese men. Two previously unrecognized findings--hyperprolactinemia and an unusual radiographic abnormality of fluffy, spiculated bony proliferation--may facilitate recognition of the syndrome. The relationship of these various manifestations to the plasma cell dyscrasia is unknown, but a number of possibilities are discussed.