Eye involvement in autoimmune blistering diseases

Ekong, Anthony; Foster, C. Stephen; Roque, Manolette R.

doi:10.1016/s0738-081x(00)00182-6

Cited by 14 publications

(11 citation statements)

References 73 publications

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“…3 Though ocular involvement is rare, a few reports of early cicatricial changes exist in literature. 4,[7][8][9] In the current series, all five cases presented with advanced disease and extensive conjunctival fibrotic changes, which were more severe in the inferior bulbar and palpebral conjunctiva. Corneal changes were also more localized to the inferior cornea suggesting a degree of exposure keratopathy, due to the inferior cicatricial changes, contributing to the disease process.…”

Section: Discussionmentioning

confidence: 51%

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

et al. 2021

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Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Data regarding the clinical features, treatment administered, and outcomes was collected. Five cases (n=10 eyes) of bullous pemphigoid disease with ocular involvement were included. All eyes had significant cicatricial conjunctival changes in the form of symblephara, inferior forniceal shortening, and tarsal conjunctival scarring. Conjunctival granulomas were present in 3/10 eyes. Corneal involvement in the form of punctate keratitis was present in all eyes while 4/10 eyes had an epithelial defect as well. The management of these cases involved topical therapy with corticosteroids and lubricants (n=10 eyes) while pulse doses of intravenous methyl prednisolone were administered in 5/5 cases. Pulse intravenous cyclophosphamide was supplemented in 2/5 cases. Adequate control of the disease was noted in 3/5 cases while one case had a recalcitrant form of the disease and developed a dermalised ocular surface in both eyes. The last patient was lost to follow up during the course of therapy. Bullous pemphigoid can present with an aggressive form of cicatrizing conjunctivitis similar to other variants of autoimmune blistering disorders and must be considered as a differential in cases presenting with ocular cicatricial disease. Long-term intensive immunosuppression is required for the management of these cases to preserve the visual function and the integrity of the globe.

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Section: Discussionmentioning

confidence: 51%

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

et al. 2021

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“…The patient used to avoid gluten in his diet but patients with LAD tend to not respond to a diet free of gluten as in other dermatological diseases [2, 12]. …”

Section: Discussionmentioning

confidence: 99%

Ocular Involvement and Blindness Secondary to Linear IgA Dermatosis

Ramos-Castellón

Ortiz-Nieva

Fresán

et al. 2010

Journal of Ophthalmology

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A 43-year-old man with linear immunoglobulin A (IgA) dermatosis associated with gluten intolerance presented with progressive vision loss, pain and photosensitivity in both eyes. His visual acuity was light perception (LP) in both eyes. A physical examination revealed bullous, papular lesions with erythematous borders in periocular tissues, limbs, and thorax. Slit-lamp examination showed conjunctival hyperemia, fibrosis, corneal opacification, and vascularization with epithelial defects. Immunofluorescent skin and corneal surface biopsy studies showed linear IgA deposits. The patient was treated with keratolimbal allogenic transplantation and cryopreserved amniotic membrane in the right eye. Regardless of the treatment he persisted with torpid evolution developing retinal and choroidal detachments. After these events he was started on intravenous immune globulin (IVIG) and showed very slight improvement in ocular surface. These types of blistering diseases are rare in the eye. Even when adequate local treatment is given, systemic treatment is mandatory and ocular prognosis can be unsatisfactory.

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“…Bullous pemphigoid (BP) is the most common AIBD, affecting proteins BP180 and BP230 – however, extra-cutaneous membranes are only occasionally involved in BP and there is limited literature on ocular involvement [ 5 , 86 , 87 ]. Conjunctival scarring has been reported as occurring infrequently [ 50 ].…”

Section: Methodsmentioning

confidence: 99%

“…Documentation of ocular manifestations in anti-p200 pemphigoid, lichen pemphigoides, and pemphigus gestationis have been limited due to their rarity [ 43 ]. Lastly, ocular involvement in dermatitis herpetiformis is rare, confined to peri-orbital areas, and rarely results in cicatrising changes [ 86 ].…”

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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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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Eye involvement in autoimmune blistering diseases

Cited by 14 publications

References 73 publications

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

Ocular Involvement and Blindness Secondary to Linear IgA Dermatosis

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

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