Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis

Christopher M. Lowther, Annie O. Morrison, Nicole M. Candelario, Sheva Khalafbeigi, Clay J. Cockerell

Research output: Contribution to journalArticlepeer-review

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients.

Original languageEnglish (US)
Pages (from-to)e140-e143
JournalAmerican Journal of Dermatopathology
Volume38
Issue number10
DOIs
StatePublished - Oct 1 2016

Keywords

  • atypical elastosis
  • cutaneous disease manifestations
  • cutaneous fibroelastosis
  • fibroelastosis
  • pleuroparenchymal fibroelastosis
  • PPFE
  • pulmonary fibrosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

Fingerprint Dive into the research topics of 'Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis'. Together they form a unique fingerprint.

Cite this