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

Rashid, H., Lamberts, A., Diercks, G. F. H., Pas, H. H., Meijer, J. M., Bolling, M. C. & Horváth, B., Dec-2019, In : American journal of clinical dermatology. 20, 6, p. 847-861 15 p.

Research output: Contribution to journalReview articleAcademicpeer-review

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.

Original languageEnglish
Pages (from-to)847-861
Number of pages15
JournalAmerican journal of clinical dermatology
Volume20
Issue number6
Early online date16-Jul-2019
Publication statusPublished - Dec-2019

    Keywords

  • SERRATION PATTERN-ANALYSIS, LINKED-IMMUNOSORBENT-ASSAY, ANTI-DESMOGLEIN 1, PEMPHIGUS-VULGARIS, PARANEOPLASTIC PEMPHIGUS, DIRECT IMMUNOFLUORESCENCE, MULTIORGAN SYNDROME, LABORATORY DIAGNOSIS, DIFFERENTIAL-DIAGNOSIS, EUROPEAN ACADEMY

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