Liquid-based cytology for differentiating two cases of pemphigus vulgaris from oral squamous cell carcinoma.

Pemphigus vulgaris (PV) is a rare autoimmune disease characterized by blisters on the skin and mucous membrane. Since it often appears in the oral mucosa first, it may be diagnosed by oral mucosal cytology. Although the cytologic finding is characterized by acantholytic cells, that is, Tzanck cells, it is important to distinguish PV from neoplastic lesions of the oral mucosal epithelium, including differentiation from atypical parabasal/basal cells, which appear in squamous cell carcinoma (SCC). In this study, we examined the cellular findings in two cases of PV and a case of well-differentiated SCC with loss of epithelial cell cohesion. The samples were prepared using liquid-based cytology, which showed small round-shaped and deeply stained atypical, orangeophilic keratinocytes not only in SCC but also in PV, which made differentiation between the two difficult. However, Tzanck cells found in PV differ from the deep atypical parabasal/basal cells of SCC, suggesting that the cell outline is indistinct and small protrusions and brush-like structures are observed. This feature of Tzanck cells may be useful in cytological judgment.

A clinical and demographic analysis of oral pemphigus vulgaris: A retrospective cross-sectional study from 2001 to 2021.

Background and Aims: Pemphigus vulgaris is an autoimmune vesiculobullous mucocutaneous disorder with life-threatening consequences. Early detection and adequate care are crucial for a good prognosis. This study aimed to determine the demographic data, clinical features, and the prognosis of patients with oral pemphigus vulgaris. Materials and Methods: From 2001 to 2021, all diagnosed oral pemphigus vulgaris cases were extracted. Each patient's demographic and clinical data were gathered. Patients were called via phone to assess the prognosis, treatment type, and specialty of the physician who provided the diagnosis and therapy. Results: The majority of the patients had only oral lesions with higher prevalence in female who also expressed severe pain than male. Only 14 of 29 patients responded phone calls. Except for one, all patients were in active disease. More than half of those respondents said pemphigus negatively affects social behavior and food intake. Correct diagnosis and treatment were decided by dermatology, oral medicine, and maxillofacial surgery specialists. Conclusion: Oral pemphigus vulgaris was prevalent in females. Severe pain was common in females and older people. Even with effective therapy, the prognosis was poor. Medical and dental professionals had little knowledge of pemphigus vulgaris. Patients frequently report poor quality of life.

Immunocytometric Analysis of Oral Pemphigus vulgaris Patients after Treatment with Rituximab as Adjuvant.

Background: B-cell depletion therapy was demonstrated to be a valid and safe alternative as an adjuvant in oral-pharyngeal pemphigus vulgaris (OPV) patients. We aimed to assess its effects on anti-desmoglein (Dsg) 1 and 3 and leukocytes subsets profile in these patients' population. Methods and Materials: We evaluated the immunologic profile of 10 OPV patients treated with RTX as adjuvant by using the ELISA testing for anti-Dsg-1 and -3 titers and the immunophenotyping for B and T-cell lymphocyte subpopulations and compared them with the PDAI score for clinical remission. Results: A significant difference in medians between baseline, end of RTX therapy, and 6 months after RTX therapy was observed in Dsg-3 titer (p < 0.001), in the CD8 (p = 0.009), and CD20 counts (p < 0.001). Multiple comparisons after Bonferroni adjustment confirmed such significant differences mainly between baseline and the end of RTX therapy and baseline and 6 months after RTX therapy. Only the anti-Dsg-3 titer at the end of RTX therapy demonstrated a slight positive correlation with the PDAI score at baseline (p = 0.046, r = 0.652). Conclusions: B-cell depletion adjuvant therapy in OPV patients demonstrated a significant impact on anti-Dsg-3 titer and B and T-cell lymphocyte subpopulations profile.

Immune-Mediated Desquamative Gingivitis and Optical Coherence Tomography Diagnostic Patterns: Clinical Implication from a Systematic Review.

Desquamative Gingivitis (DG) comprises heterogeneous clinical manifestations of numerous immune-mediated muco-cutaneous diseases. Optical Coherence Tomography (OCT) has been proposed as a valuable diagnostic support even if, to date, there are no standardized OCT-diagnostic patterns applicable to DGs. A systematic review was performed to detect existing data on in vivo OCT diagnostic patterns of the most common immune-mediated DGs (i.e., pemphigus vulgaris, mucous membrane pemphigoid and oral lichen planus). It has been found that OCT exhibits specific patterns that address the diagnosis of DG by pemphigus vulgaris (i.e., intraepithelial unilocular blister, reduced epithelial thickness, presence of acantholytic cells in the blister) and by mucous membrane pemphigoid (i.e., subepithelial multilocular blister, presence of inflammatory infiltrate), but not by oral lichen planus. These patterns could offer an attractive diagnostic OCT framework to support the clinical preliminary assessment and monitoring of these complex pathological conditions.

The use of rituximab as an adjuvant in the treatment of oral pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris patients with exclusive oral involvement (OPV) treated with conventional immunosuppressive therapy may be non-responders or experience severe side effects and/or relapses. In such cases, rituximab could be used as an adjuvant in recalcitrant OPV patients. METHODS: A retrospective single-center study on patients with oral pemphigus vulgaris treated with RTX at a dose of 375 mg/m2 was performed, evaluating the complete clinical and immunological remission, side effects of RTX, and possible correlation between anti-desmoglein (Dsg) 3 antibodies and clinical remission. RESULTS: We treated 10 OPV patients, of which 60% had a moderate and 40% mild disease severity before therapy with RTX. Complete clinical remission (CCR) was achieved in 100% of OPV patients, of which 20% developed side effects and 20% experienced a relapse in a mean time of 15.2 ± 10.2 weeks. The mean time for CCR was achieved in 19.8 ± 10.3 weeks, whereas the duration of the CCR consisted in 37.4 ± 33.5 weeks. OPV patients underwent a mean follow-up of 57.2 ± 37.7 weeks. In all patients, the mean of pemphigus disease area index (PDAI) decreased from 20.3 ± 14.1 to 0.4 ± 0.0, whereas the mean Dsg3 value dropped from 157.1 ± 40.6 to 67.0 ± 26.6 after therapy with RTX. However, no correlation was found between PDAI and anti-Dsg3 antibodies before and after therapy with RTX (P > .05). CONCLUSIONS: RTX represents a valid and safe alternative as an adjuvant in OPV patients with low rate of relapses and side effects.

Oral pemphigus vulgaris: Liquid-based cytological findings and pitfalls.

Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease characterized by the formation of suprabasal cleavage and acantholysis. As this disease almost always affects the oral mucosa, conventional cytological smears of oral lesions can be used for the initial diagnosis of PV. We report two cases of PV that were initially diagnosed based on cytological smears of an oral sample. As atypical squamous cells were present even in the liquid-based cytological (LBC) smears of the oral lesion in these two cases, this ultimately led to the misinterpretation of squamous cell carcinoma. These findings demonstrate that cytological mimicry of oral PV can occur in malignant cases when there is an absence of appropriate clinical information.

Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study.

OBJECTIVES: Few studies have compared oral mucous membrane pemphigoid (MMP) and pemphigus vulgaris (PV). Descriptive analysis of oral features, extent of extra-oral involvement, and management outcomes were performed. SUBJECTS AND METHODS: Patients with PV and MMP, the latter with exclusive oral involvement at first presentation, were included. RESULTS: There were 26 MMP (46%) and 31 PV (54%) patients. Desquamative gingivitis was evident in 84% of MMP cases compared to 28% of PV cases (P < 0.05). Non-gingival lesions were noted in 6% of MMP cases compared to 55% of PV cases (P < 0.01). Management of MMP consisted of only topical corticosteroids in 88% of cases while 12% of cases required concomitant systemic therapy. All PV cases (100%) required systemic therapy. No patients with MMP developed scarring or ocular lesions, and one patient (4%) developed cutaneous lesions. Five PV cases (16%) had oral cavity involvement only with three (60%) developing pharyngeal involvement and two (40%) developing cutaneous lesions on follow-up. CONCLUSION: Oral MMP presents primarily as desquamative gingivitis, infrequently involving extragingival sites, and is highly amenable to topical therapy, while PV is a systemic mucocutaneous disease with extensive non-gingival oral lesions that almost always requires systemic therapy.

Pénfigo vulgar oral: reporte de un caso clínico / Oral penphigus vulgaris: report of a clinical case

El pénfigo vulgar es el tipo más común de un grupo padecimientos crónicos autoinmunes identificados por la presencia de lesiones ampulosas situadas en las mucosas y piel. El pénfigo vulgar oral (PVO) se caracteriza por la presencia de ampollas localizadas en las encías, paladar blando, carrillos, pero cualquier sitio de la cavidad oral puede ser afectado. Estas lesiones se presentan primero en la cavidad oral y meses después en la piel, por lo que su diagnóstico temprano y oportuno es vital para el pronóstico. El propósito de este trabajo es presentar esta condición clínica en una persona adulta mayor.

Pemphigus vulgaris is the most common of a group of chronic autoim-mune conditions characterized by the presence of mucosal and dermal blisters. In the case of oral pemphigus vulgaris (OPV), these are typi-cally found on the gums, soft palate, and cheeks, though anywhere in the oral cavity can be affected. These lesions appear first in the oral cavity and then, months later, on the skin. Therefore, early diagnosis is crucial for prognosis. The aim of this paper is to present a case report of this condition in an older adul.