Peripapillary comet lesions and comet rain in PXE-related retinopathy

  • Vittoria Murro
  • Dario Pasquale Mucciolo
  • Andrea Sodi
  • Federica Boraldi
  • Daniela Quaglino
  • Gianni Virgili
  • Stanislao Rizzo
Retinal Disorders



To study peripapillary comet lesions (PCL) in Italian patients affected with pseudoxanthoma elasticum (PXE).


Retrospective review of fundoscopic and swept-source (SS) optical coherence tomography (OCT) images of patients with PXE examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Careggi Teaching Hospital of Florence from 2012 to 2017.


From 148 eyes of 74 patients affected with PXE, we identified 24 eyes of 14 patients (11 were female) with a mean age of 39 years (range, 20–58 years) characterized by peripapillary comet lesions. Of these 24 eyes, 15 eyes (of 10 patients) were characterized by comet rain. The smallest comet lesion at the OCT examination appeared as a focal roundish hyper-reflective alteration at the level of the outer retinal segments and RPE-Bruch’s membrane complex; the larger lesions appeared as circular and ovoid structures with hyper-reflective borders in the outer nuclear layer.


The comet lesion formation process involves the outer layers of the retina and RPE/Bruch’s membrane complex. It consists of a degenerative/rearrangement process of the photoreceptors which occurs in an area of focal altered RPE/Bruch’s membrane resembling the outer retinal tubulation.


PXE Comet lesion Comet rain Peripapillary comet lesion OCT ORT Outer retinal tubulation 



Authors gratefully acknowledge the collaborative support of PXE Italia Onlus and the technical contribution of Sonia Costa.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (name of institute/committee) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

For this type of study, formal consent is not required.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Surgery and Translational Medicine, Eye ClinicUniversity of FlorenceFlorenceItaly
  2. 2.Department of Life SciencesUniversity of Modena and Reggio EmiliaModenaItaly

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