Oral Lesions in Autoimmune Bullous Diseases: An Overview of Clinical Characteristics and Diagnostic Algorithm

Abstract

Autoimmune bullous diseases are a group of chronic inflammatory disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques in the skin and mucosa, leading to intra-epithelial or subepithelial blistering. The oral mucosa is frequently affected in these diseases, in particular, in mucous membrane pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus. The clinical symptoms are heterogeneous and may present with erythema, blisters, erosions, and ulcers localized anywhere on the oral mucosa, and lead to severe complaints for the patients including pain, dysphagia, and foetor. Therefore, a quick and proper diagnosis with adequate treatment is needed. Clinical presentations of autoimmune bullous diseases often overlap and diagnosis cannot be made based on clinical features alone. Immunodiagnostic tests are of great importance in differentiating between the different diseases. Direct immunofluorescence microscopy shows depositions of autoantibodies along the epithelial basement membrane zone in mucous membrane pemphigoid subtypes, or depositions on the epithelial cell surface in pemphigus variants. Additional immunoserological tests are useful to discriminate between the different subtypes of pemphigoid, and are essential to differentiate between pemphigus and paraneoplastic pemphigus. This review gives an overview of the clinical characteristics of oral lesions and the diagnostic procedures in autoimmune blistering diseases, and provides a diagnostic algorithm for daily practice.

Clinico-Pathological Profile and Outcomes of 45 Cases of Plasma Cell Gingivitis

“…For a proper diagnosis, it is necessary to complete the diagnostic algorithm with histological and immunological data on the tissue (DIF) and in the sera (ELISA, immunoblotting). Histological examination of incisional biopsy stained by hematoxylin-eosin, the first step of the diagnostic workflow, shows the level of the bulla, intraepithelial or subepithelial, and the type of inflammatory infiltrate [ 43 ] followed by the DIF and ELISA test for desmoglein 1 and 3, BP180, and 230 [ 44 ]. In our bullous, but also in erythematous/erosive cases, histological analysis showed only subepithelial detachment; DIF resulted positive for Igs-A and Igs-G in the stromal area and intercellular deposits of Igs-G in 4 specimens.…”

Risk Factors for Mucosal Involvement in Bullous Pemphigoid and the Possible Mechanism: A Review

“…In addition, circulating autoantibodies are usually absent or present at low titers in patients with MMP ( 19 ). Besides the NC16A domain of BP180, other antigens in MMP include the C-terminal domain of BP180, laminin 332, p200, type VII collagen, and α6β4 integrin ( 20 ). However, the diagnosis of MMP with generalized blisters and of BP with extensive mucosal involvement remains challenging especially in patients with circulating anti-BP180 autoantibodies.…”

Saliva and Oral Diseases

“…Histopathology, in some cases with direct immunofluorescence, remains the gold standard for the diagnosis of oral disease. In fact, it is often necessary to perform a biopsy to confirm the diagnosis of bullous diseases (together with DIF) [47], Sjögren's syndrome [48], and for all lesions suspected for malignancy [49].…”