Abstract
Relevance. Pemphigus vulgaris is a rare autoimmune bullous disease characterised by the formation of intraepithelial blisters and erosions. Often, the oral mucous membrane is the first and, for quite some time, the only area affected by this disease. The oral manifestation of the disease is often misdiagnosed as erythema multiforme, candidiasis, recurrent herpes simplex infection or common diseases, which results in errors in diagnosis, late referral to a dermatologist, and inappropriate treatment. Timely recognition of the lesion will affect the prognosis and quality of life of the patient.
Aim. To generalise clinical manifestations of the oral cavity affected by pemphigus vulgaris, to analyse errors made when diagnosing at the stage of primary diagnosis, and to develop practical recommendations on the order of actions for physicians when suspected of pemphigus vulgaris.
Materials and methods. A prospective clinical, descriptive analysis of 42 patients diagnosed with pemphigus vulgaris, who were treated at the dental medical centre, Bogomolets National Medical University, was conducted. The observation period covered 2018-2025. Clinical, cytological (Tzanck smear), and serological methods (ELISA for anti-Dsg1 and anti-Dsg3 antibodies) were used. The quality of life in patients was measured using of the SF-36v2 and DLQI questionnaires. Statistical analysis was performed using of the t-test, the Mann-Whitney test, and Spearman’s correlation analysis (p < 0.05).
Results. Primary symptoms manifested as lesions of the oral mucous membrane in 83.3% of patients. In these cases, the differential diagnosis was difficult. The duration of the period between the first symptom and the diagnosis was 3.5 ± 0.6 months on average and was shorter in women than in men. At the time of referral, all patients had received incorrect diagnoses (candidiasis, erythema multiforme, recurrent herpes simplex infection) and inappropriate treatment. During the primary examination, acantholytic cells were detected by cytological examination in 47.6% of smears. When examining repeat cytological smear, it was found that acantholytic cells were present in 100% of cases. Serological testing confirmed the presence of anti-Dsg3 antibodies in all patients and anti-Dsg1 antibodies in 41.7%, which is consistent with the phenotype of the isolated and generalised forms. It was found that there was a direct correlation between the time of diagnostic uncertainty and the decline in quality of life (ρ = 0.62, p < 0.01).
Practical significance. Prompt identification of solitary lesions on the oral mucosa together with the simultaneous use of cytological and serological techniques minimises the time for establishing the correct diagnosis, helps differentiate between the various types of pemphigus vulgaris and prevents further evolution of the disease. The suggested pattern of clinical actions can serve as a working guideline to dentists and otolaryngologists to facilitate the timely and accurate detection of disease and the timely referral of patients to the corresponding specialists.
Conclusions. Oral manifestations of pemphigus vulgaris can manifest clinically as other pathologies and often lead to misdiagnosis and delayed diagnosis. Tzanck smear test is a very simple diagnostic tool and does not require special equipment, but this method should be performed together with serological research for a more accurate diagnosis of the disease, and as a result, better prognosis and higher quality of life of patients.