EADV Releases Guidelines on Diagnosis, Assessment of Mucous Membrane Pemphigoid

The European Academy of Dermatology and Venereology (EADV) has published new consensus-based European guidelines on the diagnosis and assessment of mucous membrane pemphigoid (MMP), with recommendations made on the clinical definition of MMP, disease assessment, and outcome scores. The guidelines were published in the Journal of the European Academy of Dermatology and Venereology.

Clinical Definition of MMP

The EADV guideline committee defines MMP as “a group of chronic, autoimmune subepithelial blistering disease [that] is predominantly affecting the mucous membranes.” The guideline suggests MMP should be considered a disease phenotype that is shared by a heterogenous group of blistering conditions, with antibodies that target varying autoantigens.

Although several different terminologies have been applied to describe the various types of MMP, the guideline committee wrote that MMP represents the most appropriate nomenclature for disease in patients with more than 1 affected mucous membrane. The guideline recommends that terms such as “ocular (monosite) MMP” or “oral (monosite) MMP” should be used for patients with MMP who have single-site involvement.

Outcome Measurements

According to the guideline committee, there is currently no published validated scoring methodologies for multisite MMP, but there is a recently validated methodology for oral MMP. As such, there remains no available agreed upon unified disease severity score or valid methods of combining site-specific severity scores. Ultimately, this has limited the interpretation of the relatively few interventional studies in the medical literature. The EADV writing group noted that the MMP Disease Area Index (MMPDAI), which was developed in 2012, has been proposed for use in mild MMP and was designed to be used primarily by dermatologists.

Another tool proposed for use in MMP, the Autotimer Bullous Skin Disorder Intensity Score (ABSIS), is based on the percentage of body surface area involvement and degree of activity/healing observed in the site, according to the guideline authors. Although the ABSIS has reportedly been validated for pemphigus vulgaris, the guideline authors note that this tool has not been validated for MMP. The EADV guideline committee, however, recommends using MMPDAI or ABSIS scoring tools for clinical studies in MMP.

The committee pointed out that the Oral Disease Severity Score (ODSS) has also been advocated as a comprehensive scoring system for oral lesions of lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris. According to the guideline authors, the ODSS has previously been independently shown to be a sensitive and reliable tool for assessing oral MMP. The guidelines therefore recommend application of the ODSS for clinical studies and daily practice focused on monocyte and predominantly oral MMP.

Another scoring system termed the Cicatrising Conjunctivitis Assessment Tool was developed and validated for reproducible scoring of 3 functional categories of scarring, inflammation, and morbidity. The guideline authors wrote that while the validation of the disease assessment tool was performed on patients with MMP, it is still applicable to all cases of cicatrizing conjunctivitis. Regardless, the guideline committee recommends the Cicatrising Conjunctivitis Assessment Tool for disease assessment in ocular MMP.

The guideline authors explain that these scoring systems do not address otorhinolaryngological MMP sufficiently, particularly in their ability to assess disease progression in the hypopharynx or larynx. Aside from the Cicatrising Conjunctivitis Assessment Tool, these scoring systems have reportedly not been assessed for the necessary inter- and intra-observer reproducibility of scarring, inflammation, and morbidity. The guideline committee recommends the validation of an otorhinolaryngological version of the MMPDAI scoring tools to address current gaps in MMP assessment.

Patient-reported outcome measurements (PROMs) are also important tools for diagnosis and treatment of MMP, the guideline authors wrote. Quality of life (QoL), an increasingly recognized PROM, can help clinicians gain a better understanding of patient care, evaluate treatment efficacy, and monitor the course of disease. The guideline authors noted that the QoL burden is independent of clinical severity and objective disease burden.

Questionnaires and psychometric tools that assess QoL in autoimmune bullous disease (AIBD) include those that are generic, skin specific, as well as disease specific. Tools that have been used to assess QoL in AIBD studies include the Medical Outcome Study 36-item Short Form (SF-36), Activities of Daily Living (ADLs), 12-item General Health Questionnaire (GHQ-12), Hospital Anxiety and Depression scale (HADS), Clinical Depression Questionnaire (CDQ), Beck Depression Inventory (BDI), and The Work Productivity and Activity Impairment Questionnaire–Specific Health Problem (WPAIQ-SHP).

Other dermatology-specific instruments that assess QoL in AIBD include the Dermatology Life Quality Index (DLQI), Dermatology QoL Scales, Dermatology Specific QoL Instrument, Itchy QOL and Skindex-29. In addition, the guideline authors pointed out that the Oral Health Impact Profile (OHIP) represents the most commonly used generic instrument in oral medicine. The first validated specific QoL measure, the Chronic Oral Mucosal Disease Questionnaire (COMDQ), may be also be used to evaluate chronic conditions affected the oral mucosa.

In addition, the validated 17-item AIBD QoL (ABQOL) questionnaire represents the only disease-specific tool for use in patients with AIBD. In contrast, the Treatment of AIBD QoL (TABQOL) is the first validated patient-centered system to provide quantitative assessment of therapy-specific impact on QoL in patients with AIBD. The guideline committee therefore recommends consideration of the generic, SF-36, DLQI, oral – COMDQ, OHIP, AIBD – ABQoL, and TABQoL PROMS in patients with MMP.

Disclosure: Disclosure: Several study authors declared affiliations with the biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.  

Reference

Rashid H, Lamberts A, Borradori L, et al. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I. J Eur Acad Dermatol Venereol. 2021;35(9):1750-1764. doi:10.1111/jdv.17397