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POEMS syndrome - Skin
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POEMS syndrome - Skin

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Contributors: Nneka I. Comfere MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

POEMS syndrome is a multisystem disorder associated with underlying plasma cell dyscrasia. POEMS is a syndrome of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes and sclerotic bone changes. Skin changes seen in POEMS include hyperpigmentation, hypertrichosis, skin thickening, acrocyanosis, white nails, dependent rubor, clubbing, and hemangiomas. Glomeruloid hemangiomas (usually multiple) are relatively specific lesions. Frequent lymphadenopathy with the changes of Castleman disease is present in about one-quarter of cases. Pulmonary changes including hypertension are frequent.

Criteria used for diagnosis include the following.

Mandatory major criteria:
  • Polyneuropathy that is frequently demyelinating (100%)
  • Monoclonal plasma cell proliferation (100%) – usually IgG, almost always lambda chain
Other major criteria include:
  • Castleman disease (angiofollicular lymph node hyperplasia)
  • Marked elevation of serum vascular endothelial growth factor (VEGF) levels
  • Extravascular volume overload (ascites, pleural effusions)
  • Endocrinopathy that may have affected several glands (67% to 84%)
  • Bone lesions – usually sclerotic (27% to 97%)
  • Multiple skin changes (detailed below) (68% to 89%)
  • Papilledema (29% to 64%)
  • Thrombocytosis (54% to 88%)
For more information, see OMIM.

Codes

ICD10CM:
G62.9 – Polyneuropathy, unspecified

SNOMEDCT:
79268002 – POEMS syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

See glomeruloid hemangioma for a complete histopathologic differential diagnosis of that lesion.

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 03/29/2017
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POEMS syndrome - Skin
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POEMS syndrome : Hepatomegaly, Ascites, Gynecomastia, Lymphadenopathy, Muscle weakness, Neuropathy peripheral, Numbness, Papilledema, Thickened skin, Peripheral leg edema, PLT increased, Hyperpigmentation, Prolactin elevated
Clinical image of POEMS syndrome
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