POEMS Syndrome Clinical Presentation

Updated: Jun 13, 2018
  • Author: Joanna L Chan, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

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

  • Retinal artery occlusion [4]

Next:

Physical Examination

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. [10]

  • Other authors suggest that patients with POEMS syndrome who had ischemic stroke had elevated fibrinogen levels in the absence of increased acute-phase reactants. [15] 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. [16]

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). [17]

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. [14]

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. [18]

  • 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. [19, 20]

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