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Atrophic Papulosis.

Marie Robert1, Arnaud Hot1

  • 1Department of Clinical Immunology and Internal Medicine, University of Lyon 1, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.

Dermatology (Basel, Switzerland)
|November 10, 2022
PubMed
Summary
This summary is machine-generated.

Atrophic papulosis (AP) is a rare vasculopathy with unclear causes. Eculizumab offers promising treatment for the malignant form, potentially improving outcomes for patients with gastrointestinal involvement.

Keywords:
DermatopathologyImmunologySkin inflammationVasculitis

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Area of Science:

  • Vascular Medicine
  • Dermatology
  • Pathology

Background:

  • Atrophic papulosis (AP) is a rare obliterating vasculopathy with poorly understood etiology and pathophysiology.
  • Current treatments for AP are empirical, and the malignant form carries a poor prognosis.

Purpose of the Study:

  • To elucidate the underlying pathogenesis of Atrophic Papulosis.
  • To review current understanding of benign and malignant forms of AP.
  • To discuss treatment strategies for AP, including novel therapeutic approaches.

Main Methods:

  • Literature review of Atrophic Papulosis pathogenesis.
  • Analysis of clinical presentation and diagnostic criteria for AP.
  • Evaluation of treatment outcomes for various therapeutic interventions.

Main Results:

  • AP pathogenesis involves vasculopathy, coagulopathy, and endothelial dysfunction.
  • Benign AP is skin-limited, while malignant AP can lead to systemic involvement and high mortality (over 65%).
  • Gastrointestinal and central nervous system infarctions are common causes of death in malignant AP.

Conclusions:

  • Eculizumab, a complement inhibitor, shows efficacy in malignant AP with gastrointestinal involvement.
  • Combination therapy with eculizumab and treprostinil may prevent relapse in malignant AP.
  • Further research is needed to clarify AP pathogenesis and optimize empirical treatments.