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Pseudoxanthoma elasticum. Case report

T H Chen1, F C Wei

  • 1Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Kaohsiung, Taiwan.

Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery
|December 23, 1998
PubMed
Summary
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Pseudoxanthoma elasticum (PXE) is a rare elastin disorder causing skin laxity. Surgical management, like rhytidectomy for cervical skin, can yield satisfactory cosmetic results in PXE patients.

Area of Science:

  • Dermatology
  • Medical Genetics
  • Ophthalmology

Background:

  • Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastin, leading to characteristic skin laxity and potential systemic manifestations.
  • While skin laxity is a common feature, surgical management options and outcomes for PXE are infrequently reported in medical literature.

Observation:

  • This report details a case of PXE with extensive skin involvement across the neck, axillae, breasts, abdomen, thighs, and perineum.
  • The patient also exhibited angioid streaks in the fundi, a known ophthalmological complication associated with PXE.

Findings:

  • A rhytidectomy procedure was performed to address significant laxity in the cervical skin.
  • The surgical intervention resulted in satisfactory cosmetic outcomes during a 13-month follow-up period.

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Implications:

  • This case highlights the potential benefits and successful application of rhytidectomy in managing cosmetic concerns arising from skin laxity in PXE.
  • Further review of published data aims to enhance understanding of PXE's genetic basis, histopathology, vascular and ophthalmological features, and surgical considerations.