Eye lesions of epidermolysis bullosa. Clinical features, management, and prognosis

Gans, L. A.

doi:10.1001/archderm.124.5.762

Cited by 13 publications

(8 citation statements)

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“…Reported frequencies of ocular complications were 12, 40, 4, and 51% in EBS, JEB, DDEB, and RDEB respectively. These findings were comparable to case series completed by Lin et al at Rockefeller University (New York) from 1986 to 1993 on 204 EB patients and by Gans at Washington University (New York) from 1979 to 1986 on 78 EB patients [ 23 , 24 ]. Two sequential case series from St Thomas’ Hospital (London) included the first reports of limbal broadening in DEB patients [ 21 , 22 ].…”

Section: Methodssupporting

confidence: 88%

“…Collectively termed Ocular Surface Disease (OSD), findings can include but are not limited to: blepharitis [ 23 ], lacrimal duct obstruction [ 24 , 25 ], blepharoconjunctivitis [ 19 , 21 , 23 ], symblepharon (Fig. 1 ) [ 15 – 17 , 21 ], ankyloblepharon [ 24 ], ectropion/entropion [ 24 , 25 , 28 ], corneal abrasions/erosions [ 14 , 17 , 19 – 21 , 23 – 25 , 28 ], pannus formation [ 22 , 24 , 25 ], keratopathy [ 17 , 25 , 28 , 29 ], and scarring [ 17 , 22 – 25 , 27 ]. Progressive visual impairment resulting in blindness has also been reported [ 27 , 30 , 31 ].…”

Section: Methodsmentioning

confidence: 99%

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A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

Lee

Tan

Radjenovic

et al. 2018

Orphanet J Rare Dis

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BackgroundEpidermolysis bullosa (EB) and autoimmune blistering diseases (AIBD) describe a group of rare chronic dermatoses characterized by cutaneous fragility and blistering. Although uncommon, significant ocular surface disease (OSD) may occur in both and require ophthalmological assessment. Disease scoring systems have a critical role in providing objective and accurate assessment of disease severity. The objectives of this report were, firstly, to document the prevalence and severity of ocular involvement in EB/AIBD. Secondly, to review and evaluate existing ocular and systemic scoring systems for EB/AIBD. Finally, to identify areas where further development of ocular specific tools in EB/AIBD could be pursued.MethodsA literature search was performed in October 2017 utilising Medline, Embase, and Scopus databases. The results were restricted by date of publication, between 01.01.1950 and 31.10.2017. The reference lists of these articles were then reviewed for additional relevant publications. Articles of all languages were included if an English translation was available. Articles were excluded if they were duplicates, had no reference to ocular involvement in EB/AIBD or described ocular involvement in other diseases.ResultsDescriptions of ocular involvement in EB/AIBD were identified in 88 peer-reviewed journal articles. Findings reported include but are not limited to: cicatrising conjunctivitis, meibomian gland dysfunction, dry eye disease, trichiasis, symblepharon, fornix fibrosis, keratopathy, ectropion/entropion, ankyloblepharon, corneal ulceration, visual impairment and blindness. Although scoring systems exist for assessment of OSD in mucous membrane pemphigoid, no such tools exist for the other AIBD subtypes or for EB. Several systemic scoring systems exist in the dermatological literature that are efficacious in grading overall EB/AIBD severity, but have limited inclusion of ocular features. To the best of our knowledge, there is no recognised or validated scoring systems which comprehensively stages or grades the spectrum of ocular manifestations in EB/AIBD.ConclusionsThere are a range of ocular complications documented in EB and AIBD. Development of a comprehensive ocular scoring system for EB/AIBD which incorporates the delineation between ‘activity’ and ‘damage’ would facilitate more objective patient assessment, improved longitudinal monitoring, comparison of intervention outcomes, and provide commonality for discussion of these patients due to the multidisciplinary nature of their care.

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Section: Methodssupporting

confidence: 88%

Section: Methodsmentioning

confidence: 99%

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

Lee

Tan

Radjenovic

et al. 2018

Orphanet J Rare Dis

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“…A variety of ocular findings have been reported in EB, 35,38 consistent with the many biochemical and ultrastructural similarities shared between skin and cornea, particularly at the level of the plasma membrane and the epithelialeconnective tissue basement membrane zone. 39 The most common of these are summarized in Table III.…”

Section: The External Eye Key Pointsmentioning

confidence: 52%

“…18 Since then, limited numbers of case reports [19][20][21][22][23][24][25][26][27][28][29][30][31][32] and case series [33][34][35][36][37] have confirmed that various types of pathology may arise within the external eye in some EB subtypes.…”

Section: The External Eye Key Pointsmentioning

confidence: 99%

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

243

168

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“…Symblepharon and corneal scarring have also been reported by other authors who studied eye lesions in 78 patients of various age with different forms of EB. 6 According to Tong et al, 7 recessive dystrophic EB typically features corneal abrasions, scarring, pannus, exposure keratitis secondary to lower lid ectropion, eyelid blisters, and conjunctival complications. Adhesions between the eyelid and the cornea may progress in the longer term and decrease visual acuity to the level of hand movements.…”

Section: Discussionmentioning

confidence: 99%

Amniotic Membrane Transplantation for Treatment of Symblepharon in a Patient With Recessive Dystrophic Epidermolysis Bullosa

Altan-Yaycıoğlu

Akova

Oto

2006

Cornea

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Eye lesions of epidermolysis bullosa. Clinical features, management, and prognosis

Cited by 13 publications

References 0 publications

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Amniotic Membrane Transplantation for Treatment of Symblepharon in a Patient With Recessive Dystrophic Epidermolysis Bullosa

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