July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Prospective study of the ocular manifestations in Epidermolysis Bullosa and Autoimmune Blistering Diseases identifies Dry Eye Disease
Author Affiliations & Notes
  • Brendon William Hung Chow Lee
    Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
    Department of Ophthalmology, Prince of Wales Hospital, Sydney, New South Wales, Australia
  • JEREMY TAN
    Department of Ophthalmology, Prince of Wales Hospital, Sydney, New South Wales, Australia
  • Melissa Radjenovic
    Department of Dermatology, St George Hospital, Sydney, New South Wales, Australia
  • Lien Tat
    Department of Ophthalmology, Prince of Wales Hospital, Sydney, New South Wales, Australia
  • Dedee F Murrell
    Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
    Department of Dermatology, St George Hospital, Sydney, New South Wales, Australia
  • Minas T Coroneo
    Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
    Department of Ophthalmology, Prince of Wales Hospital, Sydney, New South Wales, Australia
  • Footnotes
    Commercial Relationships   Brendon Lee, None; JEREMY TAN, None; Melissa Radjenovic, None; Lien Tat, None; Dedee Murrell, None; Minas Coroneo, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4707. doi:
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      Brendon William Hung Chow Lee, JEREMY TAN, Melissa Radjenovic, Lien Tat, Dedee F Murrell, Minas T Coroneo; Prospective study of the ocular manifestations in Epidermolysis Bullosa and Autoimmune Blistering Diseases identifies Dry Eye Disease. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4707.

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

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Abstract

Purpose : Inherited Epidermolysis Bullosa (EB) and Autoimmune Blistering Diseases (AIBD) are a heterogenous group of blistering dermatoses associated with ocular surface manifestations. This study examined the range, frequency, and severity of ocular signs in an Australasian cohort of patients with EB and AIBD.

Methods : Prospective, cross-sectional, and observational study design. Sixty-one patients with an immunohistochemical and clinical diagnosis of EB or AIBD were recruited from a dermatology subspecialty centre, and the Australasian EB and AIBD patient registries. Exclusion criteria consisted of other local or systemic causes of cicatricial conjunctivitis such as Stevens-Johnson syndrome/Toxic epidermal necrolysis, graft-versus-host disease, infection and chemical injuries.

Participants underwent a comprehensive ophthalmic review, which included an ocular and medical history, ocular surface disease index (OSDI) questionnaire, slit-lamp examination and tear-film assessment.

Results : A total of 122 eyes of 61 patients (54.1% female), aged 2-88 years old, were examined. Fifty-seven patients (93.4%) reported at least one ocular surface disease symptom. The mean OSDI score was 13.63 (0-100 range) with 24.6% of patients graded as having moderate to severe disease. Visual acuity was at least 20/20 in 42.6% of participants while the remaining were 20/60 or better.

On slit lamp examination, common ocular signs identified included blepharitis (50.8%, n=62), conjunctival/limbal hyperaemia (36.1%, n=44), conjunctival/corneal scarring (20.5%, n=25), limbal broadening (17.2%), symblepharon (8.2%, n=10), trichiasis (5.7%, n=7), and ectropion/entropion (3.3%, n=4).

On tear-film assessment, 95.1% of patients tested had a reduced tear break-up time (<8 seconds) and 92.4% an abnormal Schirmer test (<15mm). Furthermore, 57.9% exhibited significant corneal staining and tear osmolarity was elevated in 59.4% of patients (>308mOsm/L).

Conclusions : This study found a high prevalence of dry eye disease on patient-reported symptoms, OSDI and objective assessment tools. This is novel in inherited EB patients and adds to recent findings in AIBD. Ophthalmic evaluation of ocular surface disease should be a vital component in the multidisciplinary management of patients with blistering dermatoses to prevent long-term sequelae.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

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