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Pseudoxanthoma elasticum: a dermal elastosis with surgical implications

E N Kaplan, E Y Henjyoji

    Plastic and Reconstructive Surgery
    |November 1, 1976
    PubMed
    Summary
    This summary is machine-generated.

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    Pseudoxanthoma elasticum is a rare genetic disorder affecting connective tissue. This case report details a patient with pseudoxanthoma elasticum and discusses its surgical management.

    Area of Science:

    • Dermatology
    • Medical Genetics

    Background:

    • Pseudoxanthoma elasticum (PXE) is a rare, inherited disorder of connective tissue characterized by the progressive calcification and fragmentation of elastic fibers.
    • PXE affects multiple organ systems, primarily the skin, eyes, and cardiovascular system, leading to significant morbidity.

    Observation:

    • This report presents a case of pseudoxanthoma elasticum in a patient with redundant skin.
    • The patient's clinical presentation and diagnostic considerations are discussed.

    Findings:

    • Surgical excision of redundant skin from the axilla and thigh was performed.
    • The histopathological findings confirmed the diagnosis of pseudoxanthoma elasticum.

    Implications:

    • This case highlights the importance of considering PXE in the differential diagnosis of skin laxity and related conditions.

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  • Surgical intervention can be a viable option for managing symptomatic skin manifestations of PXE.
  • Further research into the long-term outcomes of surgical management for PXE is warranted.