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    Drugs & Diseases > Dermatology

    POEMS Syndrome Clinical Presentation

    Updated: Sep 17, 2020
    • 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. [20]

    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). [21]

    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. [22]

    • 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. [23, 24]

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