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Papular elastorrhexis: clinical perspectives.

Engin Sezer1, Emel Öztürk1, Durmaz Sedef Şahin1

  • 1Department of Dermatology, Acibadem University School of Medicine, Istanbul, Turkey, engin.sezer@acibadem.com.tr.

Clinical, Cosmetic and Investigational Dermatology
|November 23, 2018
PubMed
Summary
This summary is machine-generated.

Papular elastorrhexis (PE) is a rare skin condition causing small, hypopigmented papules. This subtle disorder may be underdiagnosed due to its asymptomatic nature and resemblance to other skin conditions.

Keywords:
connective tissueelastic fiberspapular elastorrhexis

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Area of Science:

  • Dermatology
  • Genetics
  • Pathology

Background:

  • Papular elastorrhexis (PE) is a rare genetic disorder affecting elastic fibers in the skin.
  • Characterized by small, asymptomatic, hypopigmented papules, PE presents symmetrically on the trunk and limbs.
  • The condition typically manifests in the first or second decade of life.

Purpose of the Study:

  • To review the clinical and histopathological features of papular elastorrhexis.
  • To discuss the differential diagnosis of PE, highlighting its potential for underdiagnosis.
  • To explore current understanding and treatment challenges for this rare elastic fiber disorder.

Main Methods:

  • Literature review of reported cases of papular elastorrhexis.
  • Analysis of clinical presentations and histopathological findings.
  • Comparison with similar dermatological conditions for differential diagnosis.

Main Results:

  • 33 cases of PE reported, suggesting potential underestimation due to subtle presentation.
  • Clinical features include monomorphous, hypopigmented papules on the trunk and limbs.
  • Differential diagnosis is broad, including acne scars, pseudoxanthoma elasticum, and other elastolytic disorders.

Conclusions:

  • Papular elastorrhexis is a rare but distinct elastic fiber disorder.
  • Its subtle and asymptomatic nature can lead to misdiagnosis or underdiagnosis.
  • Currently, no definitive curative treatment exists, and management remains a challenge.