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Periumbilical perforating pseudoxanthoma elasticum.

Piyush Kumar1, Sushil S Savant, Rizwana Barkat

  • 1Dermatology, Katihar Medical College, Katihar, Bihar, India. docpiyush@gmail.com.

Dermatology Online Journal
|March 23, 2017
PubMed
Summary
This summary is machine-generated.

Periumbilical perforating pseudoxanthoma elasticum is a rare skin condition characterized by yellowish plaques and papules. Diagnosis involves histopathological examination of elastic fibers and inflammatory cells.

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Area of Science:

  • Dermatology
  • Pathology
  • Histopathology

Background:

  • Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissues.
  • Periumbilical involvement is an uncommon presentation of PXE.
  • The condition typically manifests in adulthood.

Observation:

  • A 50-year-old woman presented with a 2-year history of a yellowish plaque with keratotic papules in the periumbilical area.
  • Clinical presentation suggested a localized or unusual form of PXE.

Findings:

  • Histopathology of the plaque revealed abnormal, curled, and granular elastic fibers in the dermis.
  • Biopsies of papules showed degenerated elastic fibers and inflammation with perforation.
  • Clinicopathological correlation confirmed the diagnosis of periumbilical perforating pseudoxanthoma elasticum.

Implications:

  • This case highlights the importance of histopathological examination for diagnosing rare dermatological conditions.
  • Understanding the specific presentation can aid in early diagnosis and management of PXE variants.
  • Further research into the pathogenesis of localized perforating PXE may be warranted.