Polyneuropathy with osteosclerotic myeloma — POEMS syndrome

A case report


A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old-man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.

This is a preview of subscription content, access via your institution.


  1. 1.

    Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (1980) Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, m-protein, and skin changes: the POEMS syndrome; report of two cases and a review of the literature. Medicine (Baltimore) 59:311–322

    Google Scholar 

  2. 2.

    Bitter MA, Komaiko W, Franklin WA (1985) Giant lymph node hyperplasia with osteoblastic bone lesion and the POEMS (Takatsuki's) syndrome. Cancer 56:188–194

    PubMed  Google Scholar 

  3. 3.

    Bolling JP, Brazis PW (1990) Optic disc swelling with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). Am J Ophthalmol 109:503–510

    PubMed  Google Scholar 

  4. 4.

    Bourdette DN, Rosenberg NL (1984) Infiltrative orbitopathy, optic disc edema, and POEMS. Neurology 34:532–533

    PubMed  Google Scholar 

  5. 5.

    Driedger H, Pruzanski W (1979) Plasma cell neoplasia with osteosclerotic lesions: a study of five cases and a review of the literature. Arch Intern Med 139:892–896

    PubMed  Google Scholar 

  6. 6.

    Driedger H, PruzanskiW (1980) Plasma cell neoplasia with peripheral polyneuropathy: a study of five cases and a review of the literature. Medicine (Baltimore) 59:301–310

    Google Scholar 

  7. 7.

    Ishikawa O, Nihei Y, Ishikawa H (1987) The skin changes of POEMS syndrome. Br J Dermatol 117:523–526

    PubMed  Google Scholar 

  8. 8.

    Iwashita H, Ohnishi A, Asada M, Kanazawa Y, Juroiwa Y (1977) Polyneuropathy, skin hyperpigmentation, edema and hypertrychosis in localised osteosclerotic myeloma. Neurology 27:675–681

    PubMed  Google Scholar 

  9. 9.

    Kelly JJ, Kyle RA, Miles JM, O'Brien PC, Dyck PJ (1981) The spectrum of peripheral neuropathy in myeloma. Neurology 31:31–34

    Google Scholar 

  10. 10.

    Kelly JJ, Kyle RA, Miles JM, Dyck PJ (1983) Osteosclerotic myeloma and peripheral neuropathy. Neurology 33:202–210

    PubMed  Google Scholar 

  11. 11.

    Kyle RA (1987) Osteosclerotic myeloma. In: Kelly JJ, Kyle RA, Latov N (eds) Polyneuropathies associated with plasma cell dyscrasia. Nijhoff, Boston Dordrecht Lancaster, pp 91–103

    Google Scholar 

  12. 12.

    Libeert P, Van Hoof A, Louwagie A, Marchau M, Dehaene I (1987) The POEMS-syndrome: an unusual form of plasma cell dyscrasia. Acta Clin Belg 42:188–192

    PubMed  Google Scholar 

  13. 13.

    Matsumine H (1985) Accelerated conversion of androgen to estrogen in plasma cell dyscrasia associated with polyneuropathy, anasarca, and skin pigmentation. N Engl J Med 313:1025–1026

    PubMed  Google Scholar 

  14. 14.

    Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, Tsubaki T, Igata A, Ozaki Y (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 34:712–720

    PubMed  Google Scholar 

  15. 15.

    Piette WW (1986) Myeloma, paraproteinemias, and the skin. Med Clin North Am 70:155–176

    PubMed  Google Scholar 

  16. 16.

    Reitan JB, Pape E, Fossa SD, Julsrud OJ, Slettens ON, Solheim ØP (1980) Osteosclerotic myeloma with polyneuropathy. Acta Med Scand 208:137–144

    PubMed  Google Scholar 

  17. 17.

    Resnick D, Greenway GD, Bardwick PA, Zvaifler NJ, Gill GN, Newman DR (1981) Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, m-protein, and skin changes: the POEMS syndrome: distinctive radiographic abnormalities. Radiology 140:17–22

    PubMed  Google Scholar 

  18. 18.

    Reulecke M, Dumas M, Meier C (1988) Specific antibody activity against neuroendocrine tissue in a case of POEMS syndrome with IgG gammopathy. Neurology 38:614–616

    PubMed  Google Scholar 

  19. 19.

    Shelley WB, Shelley ED (1987) The skin changes in the Crow-Fukase (POEMS) syndrome. Arch Dermatol 123:85–87

    PubMed  Google Scholar 

  20. 20.

    Takatsuki K, Sanada Z (1983) Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol 13:543–556

    PubMed  Google Scholar 

  21. 21.

    Waldenström JG, Adner A, Gydell K, Zettervall O (1978) Osteosclerotic “plasmocytoma” with polyneuropathy, hypertrichosis, and diabetes. Acta Med Scand 203:297–303

    PubMed  Google Scholar 

Download references

Author information



Corresponding author

Correspondence to Slobodan Apostolski.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Vidaković, A., Simić, P., Stojisavljević, N. et al. Polyneuropathy with osteosclerotic myeloma — POEMS syndrome. J Neurol 239, 49–52 (1992). https://doi.org/10.1007/BF00839213

Download citation

Key words

  • Myeloma
  • Osteosclerosis
  • Polyneuropathy
  • M protein
  • Haemangioma