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Related Experiment Videos

POEMS in childhood.

Sonya Marina1, Valentina Broshtilova

  • 1Department of Dermatology and Venereology, Faculty of Medicine, Medical University, Sofia, Bulgaria.

Pediatric Dermatology
|May 3, 2006
PubMed
Summary

This case study highlights a rare multisystemic disorder in a 17-year-old boy, characterized by diabetes, malabsorption, and distinctive skin changes. Early recognition and potential cyclophosphamide therapy are crucial for managing this complex condition.

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Area of Science:

  • Endocrinology
  • Dermatology
  • Genetics

Background:

  • Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome is a rare multisystemic disorder.
  • Its pathogenesis is obscure, with no clear hereditary pattern observed.

Observation:

  • A 17-year-old male presented with a 3-year history of diabetes mellitus, malabsorption, and skin changes (induration, hyperpigmentation, hypertrichosis) on thighs, abdomen, and scrotum.
  • Physical examination revealed hypogonadism, hepatomegaly, gynecomastia, growth retardation, and ankle edema.

Findings:

  • The patient exhibited characteristic skin manifestations and a constellation of symptoms suggestive of POEMS syndrome.
  • Neuropathy and plasma cell dyscrasia were notably absent in this case.
  • Elevated vascular endothelial growth factor (VEGF) is implicated in the microangiopathy and multiorgan deterioration seen in POEMS syndrome.

Implications:

  • This case underscores the importance of recognizing POEMS syndrome based on its diverse clinical presentation, even without overt neuropathy.
  • Cyclophosphamide cytostatic therapy shows potential benefit in managing the multiorgan deterioration associated with this rare disorder.
  • Further research into the pathogenesis of POEMS syndrome is warranted to develop targeted therapies.

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