Urban legend series: mucous membrane pemphigoid

Abstract

Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune subepithelial blistering diseases affecting primarily mucous membranes showing marked degree of clinical and immunological variability. We investigated four controversial topics: (i) Does oral pemphigoid (OP) really exist as a separate entity? (ii) Is mucous membrane pemphigoid curable? (iii) What is the best therapeutic option for MMP? (iv) Does exclusive oral IgA dermatitis exist as a distinct entity from MMP? Results from extensive literature searches suggested that (i) it is still unclear whether patients with OP could be considered as a distinct subset of MMP with specific clinical and immunological features; (ii) it is uncertain whether treatment regimens that get MMP under control can be eliminated to allow patients to be in drug-free remission or they should be continuously administered in some capacities; (iii) there is a concerning paucity of good-quality trials on MMP and available recommendations are solely based on generally small patients' cohorts or case series. Some of the 2002 consensus experts' opinions should be possibly updated, particularly regarding the safety of sulfa drugs; (iv) we did not find any strong evidence to support an exclusive oral (and perhaps also mucosal) form of LAD as a separate entity.

Salivary IgA and IgG antibodies to bullous pemphigoid 180 noncollagenous domain 16a as diagnostic biomarkers in mucous membrane pemphigoid

“…In addition, other studies report reactivity against the C‐terminal domain of BP180 . Although no clear association between target antigens and a specific clinical phenotypes has been reported, some studies have suggested an association between ocular pemphigoid and the β4 integrin, while a possible association between oral disease and α6 integrin has been proposed …”

“…24,27,28 Although no clear association between target antigens and a specific clinical phenotypes has been reported, some studies have suggested an association between ocular pemphigoid and the b4 integrin, while a possible association between oral disease and a6 integrin has been proposed. [29][30][31][32][33][34] It is unclear why bullous pemphigoid (BP), a predominantly cutaneous disease, and MMP share the same target epitope, BP180-NC16a, yet have a different clinical phenotype and disease course. Differences may relate to additional epitopes on BP180 being targeted, additional antigens being targeted (e.g.…”

Mucous membrane pemphigoid and oral blistering diseases

“…There is limited evidence to guide therapy in MMP. 27,28 For mild to moderate oral disease, topical treatments may be used as mouthwashes (betamethasone 0.5 mg or fluticasone propionate 400 lg in ª 2019 British Association of Dermatologists 10 mL of water as a 3-min rinse) or pastes (clobetasol propionate in Orabase as a 50% mix) applied either directly or in a custom-made dental tray. Maintenance of good oral hygiene is paramount, as plaque may compound gingival inflammation.…”

Describing the gingival involvement in a sample of 182 Italian predominantly oral mucous membrane pemphigoid patients: A retrospective series

“…Neoplastic disorders (detailed at baseline) were: breast cancer (n=3), melanoma (1), lung cancer (1), and prostate cancer (2). Medications taken were principally anti-hypertensive (22.1%) and anxiolytics (7.9%).…”

“…There is an evident degree of variability in the clinical and immunological features of MMP, suggesting the existence of multiple phenotypic variants (2). From a diagnostic and therapeutic view, it might be of significant advantage to discriminate a clinical subset of MMP, termed oral pemphigoid, in which the disease is mainly limited to the oral cavity and patients have a better prognosis compared to other variants (3).…”