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Brendon William Hungchow Lee, Jeremy Tan, Melissa Radjenovic, Lien Tat, Dedee F Murrell, Minas T Coroneo; Prospective study on the ocular manifestations in Epidermolysis Bullosa and Autoimmune Blistering Diseases.. Invest. Ophthalmol. Vis. Sci. 2018;59(9):156.
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© ARVO (1962-2015); The Authors (2016-present)
Inherited Epidermolysis Bullosa (EB) and Autoimmune Blistering Diseases (AIBD) are a heterogenous group of blistering dermatoses associated with ocular surface complications. We aimed to prospectively study the range, frequency, and severity of ocular manifestations in an Australasian cohort of patients with EB and AIBD.
Prospective, cross-sectional, and observational study design. 38 patients with a immunohistochemical and clinical diagnosis of EB or AIBD were recruited from dermatology practices, as well as the Australasian EB and AIBD registries. Patients with varying extents of ocular involvement were included. Exclusion criteria consisted of other local or systemic causes of cicatricial conjunctivitis such as Stevens-Johnson syndrome/Toxic epidermal necrolysis, previous chemical injury, graft-versus-host disease etc. Participants underwent a comprehensive ophthalmic review including history of ocular symptoms, ocular surface disease index (OSDI) questionnaire, slit-lamp and tear-film examination.
A total of 76 eyes of 38 patients (57.9% female), aged 2-88, were examined. Thirty-six patients (94.7%) complained of at least one ocular surface disease symptom, with median OSDI score of 16.6 (0-100 range) and 25% of patients graded with severe disease. Visual acuity was at least 20/20 in 57.9% of patients while the remaining patients had 20/60 or better.Ocular signs included blepharitis (48.6%, n=37), conjunctival/limbal hyperaemia (48.6%, n=37), conjunctival/corneal scarring (19.7%, n=15), symblepharon (7.9%, n=6), trichiasis (5.3%, n=4), and ectropion/entropion (3.9%, n=3).On tear-film assessment, 33 patients (94.3%) had a reduced tear break-up time (<8 seconds) and 89.7% an abnormal Schirmer test (<15mm). Furthermore, 51.4% exhibited significant corneal staining and tear osmolarity was elevated in 42.4% of patients (>308mOsm/L).
A high prevalence of DED was found on patient-reported symptoms, OSDI and objective assessment. This is a novel finding in inherited EB patients. Ophthalmic evaluation of ocular surface disease should be a vital component in the multidisciplinary management for these patients to prevent long-term sequelae.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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