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

Abstract

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.

European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I

“…165 A review of these ocular MMP scoring systems concluded that none met the need for a validated scoring system of the three parameters of inflammation, scarring and morbidity that cause progression in ocular MMP. 141 Only two out of the 11 systems available, measuring conjunctival scarring using different quantitative methods, have been validated for inter and intraobserver variability, and compared with another methodology. 157,163 A scoring system called the Cicatrising Conjunctivitis Assessment Tool 166 was developed to meet the requirements identified by Lee et al, and has been validated, by calculation of inter-and intra-observer levels of agreement, for reproducible scoring of the three functional categories of inflammation, scarring and morbidity.…”

“…Currently, no published validated scoring methodologies are available for multisite MMP, 141 although methodology for oral MMP has recently been validated 142 . The lack of an agreed unified disease severity score, or a means of combining site‐specific severity scores, has hindered interpretation of the few interventional studies in the literature 9 .…”

Validation of an Oral Disease Severity Score (ODSS) tool for use in oral mucous membrane pemphigoid

“…4 To date there have been no validated scoring methodologies for any site in MMP. 5 For ocular disease the most frequently used prospective scoring system is the Foster-Tauber tool, in which the presence of subconjunctival scarring, together with qualitative assessment of the extent of both forniceal foreshortening and symblepharon, are combined to create a four-stage alphanumeric measure of the extent of conjunctival scarring. 6 However, further ocular MMP scoring methodologies including assessments of the severity of inflammation using image-based grading and quantitative measurements of forniceal shortening are under evaluation.…”

“…In addition, to be valuable, a scoring tool should also be feasible and sensitive to change, and have external validity . To date there have been no validated scoring methodologies for any site in MMP . For ocular disease the most frequently used prospective scoring system is the Foster–Tauber tool, in which the presence of subconjunctival scarring, together with qualitative assessment of the extent of both forniceal foreshortening and symblepharon, are combined to create a four‐stage alphanumeric measure of the extent of conjunctival scarring .…”

A Review of Clinical Disease Scoring Systems for Cicatricial Diseases of the Conjunctiva

“…It describes the deficiencies of some of the previous grading tools, which focused mostly on ocular surface scarring caused by CCDs. The importance of scoring systems that make distinctions between disease “ activity ” and “ damage ,” is increasingly recognized ( 13 , 35 ). An overview of such scoring systems has thus been provided in this review, with the benefits and limitations of each system.…”