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Elastosis Perforans Serpiginosa: a D-penicillamine induced dermatoses in a patient with Wilson's disease

Swagatika Samal1, Mukund Sable1

  • 1All India Institute of Medical Sciences, Department of Pathology. Bhubaneswar, Orissa, India.

Autopsy & Case Reports
|December 21, 2020
PubMed

Insights

Long-term D-penicillamine treatment for Wilson's disease can cause skin issues. This case highlights D-penicillamine-induced elastosis perforans serpiginosa, a rare adverse reaction.

Area of Science:

  • Dermatology
  • Pharmacology

Background:

  • Wilson's disease is a genetic disorder of copper metabolism.
  • D-penicillamine is a chelating agent used to treat Wilson's disease.
  • Long-term D-penicillamine use is linked to various adverse reactions.

Observation:

  • A 28-year-old male with Wilson's disease presented with a six-month history of a raised, violaceous, serpiginous neck lesion.
  • The patient also developed two small papules with central umbilication.
  • Skin biopsy revealed transepidermal perforating channels and increased irregular elastic fibers.

Findings:

  • Histopathological findings were consistent with elastosis perforans serpiginosa.
  • Verhoeff's-van Gieson staining highlighted abnormal elastic fibers.
  • The findings suggest a diagnosis of D-penicillamine-induced elastosis perforans serpiginosa.

Implications:

  • This case underscores the potential for D-penicillamine to induce rare dermatological side effects.
  • Awareness of this adverse reaction is crucial for managing Wilson's disease patients.
  • Early recognition and diagnosis can guide treatment adjustments.
Keywords:
BiopsyElastic tissueSkin Diseases

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