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POEMS Syndrome Clinical Presentation

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

History

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
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Physical

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]
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Causes

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.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Acknowledgements

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.

References
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