Pemphigus vulgaris and mucous membrane pemphigoid: A systematic review of clinical manifestations, diagnosis, and treatment.

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are mucocutaneous autoimmune diseases characterized by blistering lesions of mucous membranes and skin, with very similar clinical manifestations. This study aimed to systematically review the literature on the clinical and demographic profile, diagnostic methods, and treatment of patients with pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP). Studies describing cases of PV and MMP diagnosed by direct immunofluorescence that exhibited intraoral manifestations were included. Thirty-two articles were included, with 18 studies on PV and 15 on MMP, corresponding to 50 and 123 cases diagnosed as PV and MMP, respectively. Most patients with PV (64 %) and MMP (81.3 %) were women in the fifth and sixth decade of life, respectively. The mouth was the primary site of involvement both in PV (71.4 %) and in MMP (91 %). The cheek mucosa and gingiva were the most frequently affected intraoral sites in PV (30 %) and MMP (64.2 %), respectively. Direct immunofluorescence was positive for IgG in all cases of the two conditions. The treatment of choice was systemic corticosteroid therapy for patients with PV (50 %) and topical treatment for patients with MMP (53.7 %). Differences in intraoral site predilection, extraoral involvement, and the results of diagnostic tests allow us to trace the clinical, demographic, and diagnostic profile of PV and MMP that contributes to differential diagnosis and therapeutic management.

Ocular involvement in oral vesiculobullous diseases: A review on reported cases in the literature.

Introduction: Vesiculobullous disorders are a group of autoimmune diseases manifesting as chronic ulcers in the oral cavity. Ocular involvement may accompany oral ulcers and cause various problems for patients. This review summarizes the data regarding ocular involvement in patients with oral vesiculobullous. Methods: Web of Science, Scopus, PubMed/MEDLINE, and Embase electronic databases were searched according to related keywords. Finally, 58 articles were included, all of which were case reports or series. Characteristics such as the age and sex of patients, location and type of oral lesion, type of ophthalmic injury, the interval between oral and ocular lesion, and treatment of oral and ocular disorders were summarized in tables. Results: Eye involvement was 1.6 times more prevalent in women, and most patients were between 30 and 60 years old (67.4 %). Pemphigus vulgaris accounted for almost half of the cases (48.4 %), though lichen planus is more prevalent in the general population. The most frequently affected oral site was the buccal mucosa (17.5 %), and oral ulcers usually presented as erythema, erosion, or inflammation (22.7 %). Conjunctivitis was the most common type of eye involvement (18.4 %), and ophthalmic lesions regularly appeared 12-60 months after the development of oral lesions (30.1 %). Blindness was reported in only one case. Corticosteroids and immunosuppressives were the most frequent oral and ocular lesion therapies. Conclusion: Considering the serious burdens of any ocular injury, monitoring the ocular health of patients with oral vesiculobullous diseases is highly recommended in high-risk cases, especially middle-aged women with oral pemphigus vulgaris.

Optimal biopsy site for the diagnosis of oral pemphigus vulgaris and mucous membrane pemphigoid: a systematic review and meta-analysis.

The aim of this study was to critically evaluate the diagnostic yields of direct immunofluorescence (DIF) analysis on perilesional and normal-appearing mucosa biopsy samples, to determine the optimal biopsy site for patients presenting with oral pemphigus vulgaris (PV) or mucous membrane pemphigoid (MMP). Electronic databases and article bibliographies were searched in December 2022. The primary outcome was the rate of DIF positivity. Of 374 records identified after the elimination of duplicates, 21 studies with 1027 samples were ultimately included. Meta-analysis revealed a pooled DIF positivity rate of 99.6% (95% confidence interval (CI) 97.4-100.0%, I2 = 0%) for PV and 92.6% (95% CI 87.9-96.5%, I2 = 44%) for MMP for biopsies from perilesional sites, and of 95.4% (95% CI 88.6-99.5%, I2 = 0%) for PV and 94.1% (95% CI 86.5-99.2%, I2 = 42%) for MMP for biopsies from normal-appearing sites. For MMP, there was no significant difference in the rate of DIF positivity between the two biopsy sites (odds ratio 1.91, 95% CI 0.91-4.01, I2 = 0%). The results suggest that the perilesional mucosa remains the optimal biopsy site for DIF diagnosis of oral PV, while the normal-appearing mucosa biopsy is optimal for oral MMP.

Laryngeal Mucous Membrane Pemphigoid as an Immune-related Adverse Effect of Pembrolizumab Treatment.

Pembrolizumab is an immune checkpoint inhibitor (ICI) approved for multiple indications in a variety of malignancies. Although generally well tolerated, the potential for significant adverse effects, specifically immune related adverse effects (irAEs) needs to be taken into consideration. Several cases of bullous pemphigoid have been reported as a cutaneous adverse effect of ICIs since 2015, and there are recent reports of mucous membrane pemphigoid (MMP). We present the case of an 84-year-old male with metastatic urothelial carcinoma on treatment with pembrolizumab, who developed laryngeal mucous membrane pemphigoid as an irAE. The diagnosis was based on patient's clinical history and serologic testing, and supported by symptomatic improvement after ICI discontinuation and immunosuppression. Pembrolizumab-induced MMP is a newly described and infrequent irAE, requiring early suspicion and close monitoring for its diagnosis and management.

Platelet-rich fibrin a new approach in management of persistent oral ulcers in blistering skin diseases.

The present study designed to evaluate the healing power of platelet-rich fibrin (PRF) in terms of pain control and mucosal repair. A randomised, controlled, pilot clinical trial was conducted on 16 patients randomly distributed with 1:1 allocation ratio into two groups. The treatment group received PRF minced and mixed with orabase and the control group received clobetasol propionate 0.05% mixed with orabase. Pain reduction was evaluated as primary outcome along with mucositis healing as secondary outcome. A statistically significant difference in pain reduction was observed between the two groups (p ≤ 0.05). The clinical results at Day 7 has shown that PRF group had 100% pain reduction while, CP group had 32.5% reduction from base line. PRF offered superior clinical results providing rapid pain alleviation and accelerated ulcer healing compared to corticosteroids.

Photobiomodulation of gingival lesions resulting from autoimmune diseases: systematic review and meta-analysis.

OBJECTIVES: To evaluate the effects of photobiomodulation (PBM) in gingival lesions resulting from autoimmune diseases; to compare PBM and topical corticosteroid (CS) treatment; and to assess PBM outcome over time of follow-up. MATERIALS AND METHODS: A comprehensive electronic search was performed in four electronic databases. Treatment effects were measured through visual analog scale of pain (VAS) and clinical evolution of lesion (Thongprasom scale for oral lichen planus (OLP)). Meta-analysis was performed to compare PBM with topical corticosteroid treatment and to evaluate PBM effect over time of follow-up. RESULTS: Seventeen studies were included in this review, of which six were used for the meta-analysis. Meta-analysis results showed no significant differences between PBM and topical CS in pain reduction at baseline (MD = 0.20, 95% CI = - 0.92, 1.32, p = 0.72) and 60-day follow-up (MD = 0.63, 95% CI = - 3.93, 5.19, p = 0.79); however, VAS showed significant pain reduction when compared before and after PBM at 30-day (MD = - 3.52, 95% CI = - 5.40, - 1.64, p = 0.0002) and 60-day (MD = - 5.04, 95% CI = - 5.86, - 4.22, p < 0.00001) follow-up. Thongprasom clinical scale for OLP also showed significant improvement at 30-day follow-up (MD = - 2.50, 95% CI = - 2.92, - 2.08, p < 0.00001) after PBM. CONCLUSION: PBM led to significant reduction of pain and clinical scores of the lesions, not having shown significant differences when compared to topical CS. CLINICAL RELEVANCE: PBM has been used in the treatment of autoimmune gingival lesions, but so far there is little strong evidence to support its use.

Oral Pemphigoid Recalcitrant Lesion Treated with PRGF Infiltration. Case Report.

Mucous membrane pemphigoid (MMP) is a heterogeneous group of chronic autoimmune subepithelial blistering diseases. Oral involvement is present in almost all patients, may represent the onset of the disease, and causes different degrees of pain, dysphagia, soreness, and bleeding. Treatment is based on systemic and/or oral corticoids, or other immunosuppressants. Occasionally, oral lesions can show a poor response to standard treatments. We present the case of a 61-year-old female patient with a painful extensive MMP oral ulcerative lesion recalcitrant to previous systemic azathioprine and local triamcinolone treatment, which was successfully treated in a novel way using PRGF infiltrations as adjuvant. After four weekly infiltrations, pain was reduced from 10 to 0 in a VAS and the lesion was completely healed. The patient continued with a low dose maintenance immunosuppressive treatment (prednisone 5 mg/day PO), and after 13 months of follow-up, there was no relapse of the lesion and no side effects. Although future research is necessary to confirm these observations, PRGF could be a useful adjuvant for the management of extensive mucous membrane pemphigoid oral lesions.

A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.

Desenlaces en pacientes operados con queratoprótesis boston tipo 1 en un centro oftalmológico especializado en la ciudad de Medellín / Outcomes in patients with boston type 1 keratoprosthesis in a specialized center in Medellín

Objetivo: reportar las características y descenlaces de pacientes con queratoprótesis Boston tipo 1. Diseño: estudio observacional descriptivo de corte transversal. Método: se incluyeron los pacientes operados con queratoprótesis Boston tipo 1 residentes en Colombia, mayores de 17 años, sin queratoprótesis previas y que tuviera más de 6 meses de seguimiento, para un total de 39 ojos de 39 pacientes. La cirugía se indicó en los casos con o sin queratoplastia previa que tuvieran agudeza visual (AV) igual o inferior a cuenta dedos y déficit de limbo bilateral o vascularización corneal profunda en más de 3 cuadrantes. Se recolectó y analizó la información de la historia clínica en forma retrospectiva y prospectiva. Las variables principales fueron: agudeza visual, retención del dispositivo y complicaciones. Se realizó un análisis univariado presentando los resultados en frecuencias absolutas y porcentajes para las variables categóricas y para las variables cuantitativas como promedio y desviación estándar (DE) si tienen distribución normal o como mediana y rango intercuartil si no tienen distribución normal. Resultados: La mediana de seguimiento fue de 53.5 meses (rango 6 meses a 10 años). La mayoría de pacientes (66.7%) habían tenido previamente queratoplastia penetrante. El diagnóstico de base fue en 53.8% condiciones no inflamatorias yen 46.2% condiciones inflamatorias. La AV preoperatoria en todos los ojos estaba entre percepción de luz y cuenta dedos; posterior a la cirugía el 97.4% tuvieron mejoría de la AV. Para el fi nal del seguimiento la AV permaneció mejor que la preoperatoria en 53.8%, fue igual a esta en 17.94% y peor que esta en 28.2%. Hubo necrosis periprostética en 28.2%, y 38.5% tuvieron recambio del dispositivo. Al final del seguimiento 89.7% de los pacientes tenían una queratoprótesis in-situ. Conclusión: la queratoprótesis Boston tipo 1 es una opción viable para mejorar la AV en pacientes con trasplantes de córnea fallidos o en quienes se prevé alta tasa de fallo o rechazo de este. En la gran mayoría de los pacientes se logra mejoría inicial significativa de la AV, la cual se mantiene al final del seguimiento en más de la mitad de ellos. Refinamientos progresivos en la técnica quirúrgica y el manejo postoperatorio disminuirán las complicaciones que llevan a la perdida visual progresiva

Purpose: to report characteristics and outcomes in patients with Boston type 1 keratoprosthesis. Design: observational descriptive cross-sectional study. Method: Patients with Boston type 1 queratoprosthesis who were living in Colombia, 17 years or older, without previous keratoprosthesis and with more than 6 months follow up were included, for a fi nal of 39 eyes from 39 patients. Surgery was indicated for those with or without previous keratoplasty who had visual acuity (VA) of counting fi ngers or worse and bilateral limbal stem cell deficiency or deep corneal vascularization in more than 3 quadrants. Information was collected retrospectively and prospectively from medical registries and was analyzed. Main variables were: visual acuity, device retention and complications. An univariate analysis was conducted and results are presented in absolute frequencies and percentages for categoric variables and for quantitative variables in average and standard deviation (SD) if they have normal distribution and in median and interquartile range if they do not have normal distribution. Results: Median follow up was 53.5 months (range 6 months to 10 years). Most patients (66.7%) had previous penetrating keratoplasty. Baseline diagnoses were in 53.8% non-infl ammatory conditions and in 46.2% inflammatory conditions. Preoperative VA ranged between light perception and counting fingers in all eyes; aft er surgery 97.4% had improvement in VA. By the end of follow up VA remained better than preoperative in 53.8%, was the same as preoperative in 17.94% and worse than preoperative in 28.2%. There was periprosthetic necrosis in 28.2%, and 38.5% had keratoprothesis exchange. At the end of follow up 89.7% of patients had a keratoprosthesis in-situ. Conclusion: Boston type 1 keratoprosthesis is a viable option to improve VA in patients with previously failed corneal transplants or in cases that anticipate bad prognosis for keratoplasty. In most patients there was a signifi cant initial improvement in vision, and more than half of them retained better than initial VA on their last follow up. Further refinements in surgical technique and postoperative care would decrease complications that lead to progressive visual loss.

Desquamative gingivitis as only presenting sign of mucous membrane pemphigoid.

Desquamative gingivitis (DG) is a clinical condition in which the gingiva appears reddish, glazed, and friable with loss of superficial epithelium. DG is considered a clinical manifestation of many gingival diseases and hence not identified as a diagnosis itself. Mucous membrane pemphigoid (MMP) is an autoimmune vesiculobullous disorder of mucous membrane characterized by subepithelial bullae formation. MMP can affect the mucous membranes of oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus and vary in its severity. The most commonly affected sites are oral cavity and conjunctiva. Since DG may be the early sign or only presenting sign of these conditions, most of the times, dental surgeon plays a key role in the diagnosis and prevention of the systemic complications of these diseases. We report a case of a 41-year-old male patient presented with DG. Histopathological examination revealed subepithelial clefting suggestive of MMP. The patient was treated with topical application of triamcinolone acetonide 0.1% 3-4 times a day for 1 month.