Medscape is available in 5 Language Editions – Choose your Edition here.


POEMS Syndrome Clinical Presentation

  • Author: Joanna L Chan, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Oct 22, 2015


Presenting symptoms vary based on the organ systems involved. Frequent presentations include the following:

  • Symmetric distal weakness and paresthesia
  • Painful diarrhea
  • Peripheral edema

Patients may also report the following:

  • Impotence
  • Gynecomastia
  • Amenorrhea
  • Shortness of breath
  • Hypertrichosis
  • Hyperpigmentation
  • Hyperhidrosis
  • Raynaud phenomenon
  • Loss of function because of skin tightening
  • Edema
  • Diarrhea
  • Rarely, bone pain and fractures


Skin manifestations are as follows:

  • Diffuse hyperpigmentation and lower extremity edema are seen in more than 90% of patients.
  • Hypertrichosis is seen in 80% of patients and can be diffuse but is usually most pronounced over the face, the limbs, and the chest.
  • Skin thickening with sclerodermoid changes and tightening, which can limit function, is seen in approximately 77% of patients.
  • Angiomas are often present and may progress rapidly.
  • Nail changes are noted, with whitening of the proximal nails and possible clubbing.
  • Many other skin changes have also been observed in persons with POEMS syndrome; these changes include alopecia, flushing, ichthyosis, Sweetlike lesions, and vasculitis.
  • Mucous membrane involvement has not been reported.

Neurologic manifestations are as follows:

  • Motor deficiency follows decreased perception of pinprick and vibration sensation.
  • Both motor and sensory deficits are reported and are characterized by distal, symmetric, and progressive involvement associated with gradual proximal spread.
  • Some loss of temperature sensation and nociception has been reported.
  • Deep tendon reflexes are diminished, but cranial nerve examination results are normal.

Cardiovascular manifestations are as follows:

  • Rarely, macroangiopathy affecting coronary and lower limb arteries may be associated with POEMS syndrome, which has resulted in reported cases of cerebrovascular manifestations in the absence of vascular risk factors.
  • Acute ischemic stroke has been reported rarely in patients with POEMS syndrome, and one case series of such patients described a particular association with end artery border-zone infarctions and elevated fibrinogen levels, which may be implicated in the pathogenesis of cerebrovascular events.[7]
  • Other authors suggest that patients with POEMS syndrome who had ischemic stroke had elevated fibrinogen levels in the absence of increased acute-phase reactants.[10] This counterpoint suggests that increased fibrinogen may be more a consequence of the chronic release of proinflammatory cytokines characteristic of POEMS syndrome and of the inflammatory response following a stroke, rather than to its actual pathogenesis.
  • A 2015 article describes a case of cardiac amyloidosis in a patient with POEMS syndrome, suggesting another potential associated risk and method for diagnosis of the syndrome.[11]

Endocrine manifestations are as follows:

  • In a series of 170 patients, 84% had a recognized endocrinopathy, the most common of which was hypogonadism associated with depressed testosterone levels, gynecomastia, or elevated prolactin levels.
  • Other abnormalities included hypothyroidism, abnormal glucose metabolism, adrenal insufficiency, hypocalcemia
  • Many patients may exhibit evidence of multiple endocrinopathies in the 4 major endocrine axes (ie, gonadal, thyroid, adrenal, and glucose).[12]

Pulmonary manifestations are as follows:

  • Approximately a quarter of patients will develop respiratory symptoms within 2 years of diagnosis.
  • Patients may develop restrictive lung disease, pulmonary hypertension, respiratory muscle weakness, and an isolated diminished diffusing capacity.
  • Radiographic findings such as pleural effusions and diaphragm elevation may be seen in nearly a quarter of patients.[13]

Other extracutaneous manifestations are as follows:

  • Hepatomegaly, splenomegaly, and lymphadenopathy occur in patients with organomegaly.
  • Patients with endocrine involvement may have gynecomastia.
  • Strokes have been reported rarely in patients with POEMS syndrome, which is postulated to result from an intracranial vasculopathic process.[14]
  • Patients with POEMS syndrome have been shown to have bilateral optic disc edema and bilateral cystoid macular edema. Authors suggest that these findings may reflect increased vascular permeability.[15, 16]


All cases of POEMS syndrome are associated with a plasma cell disorder. The syndrome has been seen in association with osteosclerotic myeloma, monoclonal gammopathy of unknown significance, and Waldenström macroglobulinemia, but not with classic multiple myeloma. The reason plasma cells in some dyscrasias produce factors that cause POEMS syndrome while others do not is not clear. The mechanism by which plasma cells lead to POEMS syndrome is not understood; however, elevations in IL-1beta, IL-6, TNF-alpha, and VEGF levels have been implicated.

Contributor Information and Disclosures

Joanna L Chan, MD Mohs Fellow, California Skin Institute

Joanna L Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.


Matthew N Kubicki Perelman School of Medicine, University of Pennsylvania

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

Wingfield Rehmus, MD, MPH Dermatologist, BC Children's Hospital, Vancouver, British Columbia

Wingfield Rehmus, MD, MPH is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Valeant Canada<br/> Received honoraria from Valeant Canada for advisory board; Received honoraria from Pierre Fabre for advisory board; Received honoraria from Mustella for advisory board; Received honoraria from Abbvie for advisory board.


Alexa F Boer Kimball, MD, MPH Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital

Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

  1. Ofran Y, Yishay O, Elinav E, Eran E. POEMS syndrome: failure of newly suggested diagnostic criteria to anticipate the development of the syndrome. Am J Hematol. 2005 Aug. 79(4):316-8. [Medline].

  2. Morizane R, Sasamura H, Minakuchi H, Takae Y, Kikuchi H, Yoshiya N, et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol. 2008 May. 80(5):452-5. [Medline].

  3. Min JH, Hong YH, Lee KW. Electrophysiological features of patients with POEMS syndrome. Clin Neurophysiol. 2005 Apr. 116(4):965-8. [Medline].

  4. Pavord SR, Murphy PT, Mitchell VE. POEMS syndrome and Waldenström's macroglobulinaemia. J Clin Pathol. 1996 Feb. 49(2):181-2. [Medline].

  5. Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet. 1996 Mar 9. 347(9002):702. [Medline].

  6. Kastritis E, Terpos E, Anagnostopoulos A, Xilouri I, Dimopoulos MA. Angiogenetic factors and biochemical markers of bone metabolism in POEMS syndrome treated with high-dose therapy and autologous stem cell support. Clin Lymphoma Myeloma. 2006 Jul. 7(1):73-6. [Medline].

  7. Koike H, Iijima M, Mori K, Yamamoto M, Hattori N, Watanabe H, et al. Neuropathic pain correlates with myelinated fiber loss and cytokine profile in POEMS syndrome. J Neurol Neurosurg Psychiatry. 2008 Apr 3. [Medline].

  8. Lanza C, Misericordia M, Fabrizzi G. POEMS syndrome in a 15-year-old boy: radiological findings. Pediatr Radiol. 2007 Nov. 37(11):1147-50. [Medline].

  9. Sevketoglu E, Hatipoglu S, Ayan I, Dogan O, Salihoglu B. Case report: POEMS syndrome in childhood. J Pediatr Hematol Oncol. 2008 Mar. 30(3):235-8. [Medline].

  10. Miralles GD, O'Fallon JR, Talley NJ. Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med. 1992 Dec 31. 327(27):1919-23. [Medline].

  11. Pei G, Yang D, Sun J, Luo Y, Yan J, Chen Y. Cardiac Involvement in a Patient With POEMS Syndrome Detected Using Cardiac Magnetic Resonance Imaging. Int Heart J. 2015 Sep 29. 56 (5):571-3. [Medline].

  12. Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD. Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc. 2007 Jul. 82(7):836-42. [Medline].

  13. Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A. Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest. 2008 Apr. 133(4):969-74. [Medline].

  14. Garcia T, Dafer R, Hocker S, Schneck M, Barton K, Biller J. Recurrent strokes in two patients with POEMS syndrome and Castleman's disease. J Stroke Cerebrovasc Dis. 2007 Nov-Dec. 16(6):278-84. [Medline].

  15. Wiaux C, Landau K, Borruat FX. Unusual cause of bilateral optic disc swelling: POEMS syndrome. Klin Monatsbl Augenheilkd. 2007 Apr. 224(4):334-6. [Medline].

  16. Chong DY, Comer GM, Trobe JD. Optic disc edema, cystoid macular edema, and elevated vascular endothelial growth factor in a patient with POEMS syndrome. J Neuroophthalmol. 2007 Sep. 27(3):180-3. [Medline].

  17. Bourgeault E, Dahl A, Thibeault MM, Dupéré A, Drolet AM, Mathieu J. POEMS Syndrome Complicated by Extensive Calciphylaxis: A Remarkable Recovery. J Cutan Med Surg. 2015 May-Jun. 19 (3):309-12. [Medline].

  18. Hudnall SD, Chen T, Brown K, Angel T, Schwartz MR, Tyring SK. Human herpesvirus-8-positive microvenular hemangioma in POEMS syndrome. Arch Pathol Lab Med. 2003 Aug. 127(8):1034-6. [Medline].

  19. Papo T, Soubrier M, Marcelin AG, Calvez V, Wechsler B, Huraux JM, et al. Human herpesvirus 8 infection, Castleman's disease and POEMS syndrome. Br J Haematol. 1999 Mar. 104(4):932-3. [Medline].

  20. Obermoser G, Larcher C, Sheldon JA, Sepp N, Zelger B. Absence of human herpesvirus-8 in glomeruloid haemangiomas associated with POEMS syndrome and Castleman's disease. Br J Dermatol. 2003 Jun. 148(6):1276-8. [Medline].

  21. Colaco SM, Miller T, Ruben BS, Fogarty PF, Fox LP. IgM-lambda paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome. J Am Acad Dermatol. 2008 Apr. 58(4):671-5. [Medline].

  22. Dao LN, Hanson CA, Dispenzieri A, Morice WG, Kurtin PJ, Hoyer JD. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid and myeloid findings in 87 patients. Blood. 2011 Mar 8. [Medline].

  23. Li J, Zhang W, Jiao L, Duan MH, Guan HZ, Zhu WG, et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood. 2011 Mar 10. [Medline].

  24. Ganti AK, Pipinos I, Culcea E, Armitage JO, Tarantolo S. Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. Am J Hematol. 2005 Jul. 79(3):206-10. [Medline].

  25. Imai H, Kusuhara S, Nakanishi Y, Teraoka Escaño MF, Yamamoto H, Tsukahara Y, et al. A case of POEMS syndrome with cystoid macular edema. Am J Ophthalmol. 2005 Mar. 139(3):563-6. [Medline].

  26. Giglia F, Chiapparini L, Fariselli L, Barbui T, Ciano C, Scarlato M. POEMS syndrome: relapse after successful autologous peripheral blood stem cell transplantation. Neuromuscul Disord. 2007 Dec. 17(11-12):980-2. [Medline].

  27. Nakaseko C. Autologous stem cell transplantation for POEMS syndrome. Clin Lymphoma Myeloma Leuk. 2014 Feb. 14 (1):21-3. [Medline].

  28. Dispenzieri A, Lacy MQ, Hayman SR, Kumar SK, Buadi F, Dingli D, et al. Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome. Eur J Haematol. 2008 May. 80(5):397-406. [Medline].

  29. Kojima H, Katsuoka Y, Katsura Y, Suzuki S, Suzukawa K, Hasegawa Y, et al. Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. Int J Hematol. 2006 Aug. 84(2):182-5. [Medline].

  30. Sinisalo M, Hietaharju A, Sauranen J, Wirta O. Thalidomide in POEMS syndrome: case report. Am J Hematol. 2004 May. 76(1):66-8. [Medline].

  31. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood. 2005 Aug 1. 106(3):1135. [Medline].

  32. Dietrich PY, Duchosal MA. Bevacizumab therapy before autologous stem-cell transplantation for POEMS syndrome. Ann Oncol. 2008 Mar. 19(3):595. [Medline].

  33. Kanai K, Kuwabara S, Misawa S, Hattori T. Failure of treatment with anti-VEGF monoclonal antibody for long-standing POEMS syndrome. Intern Med. 2007. 46(6):311-3. [Medline].

  34. Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome. Blood. 2006 Jun 15. 107(12):4972-3; author reply 4973-4. [Medline].

  35. Dispenzieri A, Klein CJ, Mauermann ML. Lenalidomide therapy in a patient with POEMS syndrome. Blood. 2007 Aug 1. 110(3):1075-6. [Medline].

  36. Sethi S, Tageja N, Arabi H, Penumetcha R. Lenalidomide Therapy in a Rare Case of POEMS Syndrome with Kappa Restriction. South Med J. 2009 Sep 4. [Medline].

  37. Jaccard A, Danielou-Lazareth A, Karlin L, Choquet S, Frenzel L, Garderet L. A Prospective Phase II Trial of Lenalidomide and Dexamethasone (Len-Dex) in POEMS Syndrome. Clinical Lymphoma Myeloma and Leukemia. 2015 Sep 27. 15(3):e57. [Full Text].

  38. Sanada S, Ookawara S, Karube H, Shindo T, Goto T, Nakamichi T, et al. Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation. Am J Kidney Dis. 2006 Apr. 47(4):672-9. [Medline].

  39. Authier FJ, Belec L, Levy Y, Lefaucheur JP, Defer GL, Degos JD, et al. All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflammatory cytokines. Arthritis Rheum. 1996 Aug. 39(8):1423-6. [Medline].

  40. Tang X, Shi X, Sun A, et al. Successful bortezomib-based treatment in POEMS syndrome. Eur J Haematol. 2009 Aug 6. [Medline].

  41. Kaygusuz I, Tezcan H, Cetiner M, Kocakaya O, Uzay A, Bayik M. Bortezomib: A New Therapeutic Option for POEMS Syndrome. Eur J Haematol. 2009 Sep 3. [Medline].

  42. Jouve P, Humbert M, Chauveheid MP, Jaïs X, Papo T. POEMS syndrome-related pulmonary hypertension is steroid-responsive. Respir Med. 2007 Feb. 101(2):353-5. [Medline].

  43. Rached S, Athanazio RA, Dias SA Jr, Jardim C, Souza R. Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome. J Bras Pneumol. 2009 Aug. 35(8):804-8. [Medline].

All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.