June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
In Vivo Confocal Microscopic Evaluation of Patients with Epidermolysis Bullosa to Demonstrate Severe Corneal Nerve Loss: A Case Series
Author Affiliations & Notes
  • Vicki Chen
    Ophthalmology, Tufts Medical Center, Boston, Massachusetts, United States
    Ophthalmology, Tufts University School of Medicine, Boston, Massachusetts, United States
  • Leyla Yavuz Saricay
    Ophthalmology, Tufts Medical Center, Boston, Massachusetts, United States
    Ophthalmology, Tufts University School of Medicine, Boston, Massachusetts, United States
  • Sarah Galinko
    Ophthalmology, Tufts Medical Center, Boston, Massachusetts, United States
    Ophthalmology, Tufts University School of Medicine, Boston, Massachusetts, United States
  • Pedram Hamrah
    Ophthalmology, Tufts Medical Center, Boston, Massachusetts, United States
    Ophthalmology, Tufts University School of Medicine, Boston, Massachusetts, United States
  • Footnotes
    Commercial Relationships   Vicki Chen Eliksa Therapeutics, Code C (Consultant/Contractor); Leyla Yavuz Saricay None; Sarah Galinko None; Pedram Hamrah Novartis, Code C (Consultant/Contractor), Kala, Code C (Consultant/Contractor), Dompe, Code C (Consultant/Contractor), Santen, Code C (Consultant/Contractor), Novartis, Code S (non-remunerative), Oyster Point, Code S (non-remunerative), Dompe, Code S (non-remunerative)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 3992 – A0272. doi:
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      Vicki Chen, Leyla Yavuz Saricay, Sarah Galinko, Pedram Hamrah; In Vivo Confocal Microscopic Evaluation of Patients with Epidermolysis Bullosa to Demonstrate Severe Corneal Nerve Loss: A Case Series. Invest. Ophthalmol. Vis. Sci. 2022;63(7):3992 – A0272.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Epidermolysis bullosa (EB) is a disease characterized by blistering, scarring, recurrent corneal erosions, and refractory corneal ulcers. Additionally, it presents with early-onset pain and irritation, which is not a usual presentation of disease with severe corneal nerve loss, such as neurotrophic keratopathy. Our aim is to illustrate severe corneal nerve loss using quantitative analysis by in vivo confocal microscopy (IVCM) images in EB patients

Methods : Case reports of five EB patients (ten eyes) who presented with ocular and systemic features of the disease, as confirmed by systemic evaluation and ocular examination. Records of best-corrected visual acuity (BCVA), ocular and periocular involvement, and comprehensive slit-lamp biomicroscopic evaluation, were summarized alongside IVCM findings

Results : The mean age of the patients were 13±3.08 (range 11-18) years. The mean total, main and branch corneal nerve density were 2,201.47 ±3,015.26 (range 0-8,646.64) µm/mm2, 965.39 ±1,388.38 (range 0-3,104.53) µm/mm2, and 1,236.09±1,893.41(range 0-5,542.11) µm/mm2 respectively. This degree of corneal nerve loss is consistent with repeated abrasions and ulcers, which is both the primary factor in EB and a risk factor for neurotrophic keratopathy.

Conclusions : Epidermolysis bullosa is a painful and vision-threatening condition, which causes a significant decline in both quantity and density of corneal nerves. The repeated cycle of abrasion and scarring could play a role in progressive corneal nerve loss, as seen in early-onset EB, but may also be part of the pathogenesis of the disease..

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

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