Dermatology: Practical and Conceptual Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 1 Evaluation of the Quality of Life and the Demographic and Clinical Characteristics of Patients With Pemphigus With Oral Mucosal İnvolvement: A Multicenter Observational Study Asude Kara Polat1, Mehmet Kamil Mülayim2, Tuğba Falay Gür3, Ayda Acar4, Burçin Cansu Bozca5, Can Ceylan4, Fadime Kılınç6, Rukiye Yasak Güner7, Hülya Albayrak8, Murat Durdu9, Ayşe Esra Koku Aksu10, Fatma Nalbant11, Ekin Şavk12, Dilek Bayramgürler13, Munise Daye14, Ralfi Singer15, Emine Tuğba Alataş16, Vefa Aslı Erdemir17, Mehmet Salih Gürel17, Soner Uzun5, Savaş Yaylı18 1 Memorial Bahçelievler Hospital, Department of Dermatology, Istanbul, Turkey 2 Kahramanmaraş Sütçü İmam University Faculty of Medicine, Department of Dermatology, Kahramanmaraş, Turkey 3 University of Health Sciences, Sultan Abdulhamid Han Training and Research Hospital, Department of Dermatology, Istanbul, Turkey 4 Ege University Faculty of Medicine, Department of Dermatology, İzmir, Turkey 5 Akdeniz University, Faculty of Medicine, Department of Dermatology, Antalya, Turkey 6 Ankara City Hospital, Department of Dermatology, Ankara, Turkey 7 Sivas Cumhuriyet University, Faculty of Medicine, Department of Dermatology, Sivas, Turkey 8 Namık Kemal University Faculty of Medicine, Department of Dermatology, Tekirdağ, Turkey 9 Başkent University Adana Dr. Turgut Noyan Application and Research Center, Department of Dermatology, Adana, Turkey 10 University of Health Sciences, İstanbul Training and Research Hospital, Department of Dermatology, Istanbul, Turkey 11 Edirne Keşan State Hospital, Department of Dermatology, Edirne, Turkey 12 Aydın Adnan Menderes University Faculty of Medicine, Department of Dermatology, Aydın, Turkey 13 Kocaeli University Faculty of Medicine, Department of Dermatology, Kocaeli, Turkey 14 Necmettin Erbakan University Meram Faculty of Medicine, Department of Dermatology, Konya, Turkey 15 Prof. Dr. Cemil Taşcıoğlu City Hospital, Department of Dermatology, Istanbul, Turkey 16 Muğla Sıtkı Koçman University Faculty of Medicine, Faculty of Medicine, Department of Dermatology, Mugla, Turkey 17 Medeniyet University Faculty of Medicine, Department of Dermatology, Istanbul, Turkey 18 Koç University Faculty of Medicine, Department of Dermatology, Istanbul, Turkey Key words: quality of life, oral health, oral mucosa, pemphigus Citation: Kara Polat A, Mülayim MK, Falay Gür T. Dermoscopy Use in Africa: Determinants and Challenges. Dermatol Pract Concept. 2024;14(2):e2024099. DOI: https://doi.org/10.5826/dpc.1402a99 Accepted: November 11, 2023; Published: April 2024 Copyright: ©2024 Kara Polat et al. This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Corresponding Author: Assoc. Prof. Asude Kara Polat, MD., Memorial Bahçelievler Hospital, Bahçelievler Merkez, Adnan Kahveci Blv. No: 227, 34180 Bahçelievler, Istanbul, Turkey. Phone number: +905052512142, Fax: +9002126320060, E-mail: asudekara@yahoo.com.tr 2 Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 Introduction Pemphigus vulgaris (PV) is an autoimmune disease char- acterized by intraepithelial bullae and erosions in the skin and mucosa [1]. The disease is characterized by painful ero- sions, irregularly circumscribed ulcers, and small vesicles and flaccid bullae, especially in the buccal mucosa and gingiva [2]. Yaylı et al in a 1-year prospective study that evaluated patients with pemphigus in Turkey found that the annual incidence of pemphigus was 4.7/1 million, and PV was deter- mined as the most common clinical subtype (87.3%) [3]. The presence of painful lesions disrupt oral intake depending on their location and markedly impairs the quality of life for pa- tients. In the existing literature, research on the quality of life in individuals with pemphigus is scarce, and it often evaluate pemphigus with other diseases within the pemphigus spec- trum [4-7]. Only Ghodsi et al. have specifically evaluated the quality of life of patients with PV [7]. However, in this study a specialized scale designed specifically for patients with oral manifestations of PV was not used [7]. In Turkey, two studies have evaluated the quality of life of patients with autoim- mune bullous diseases in general but no study has specifi- cally evaluated the quality of life of patients with PV [8,9]. Objectives This multicenter study aimed to determine the demographic and clinical and treatment characteristics as well as the qual- ity of life of patients with PV with oral mucosal involvement in Turkey and contribute to the epidemiological data with a large patient series. Methods In our prospective observational study, patients with PV with newly diagnosed oral mucosal involvement in Skin and Venereal Diseases clinics of 16 tertiary care institutions from different regions of Turkey were consecutively included be- tween February 2020 and August 2021. The diagnosis of the patients was confirmed using clinical features, histopathol- ogy, direct immunofluorescence, and ELISA methods. Di- agnostic criteria of the European Academy of Dermatology and Venereology guideline were applied in the diagnosis of PV [10]. The study was approved by the Ethics Committee of the University of Health Sciences, Istanbul Training and Research Hospital on 21/02/2020 (decision number: 2202). Sociodemographic and clinical and treatment characteristics of the patients were recorded and quality of life scales were applied. The following data were collected: (1) Demographic characteristics of the patients (age, gender, education level, income level, marital status, occupation, and body mass in- dex), personal and family history, and personal history of illness, smoking, and alcohol use and (2) variables related to sociodemographic and clinical characteristics of the patients regarding pemphigus disease (age of onset of PV, duration of disease, delay in diagnosis, history of pemphigus in fam- ily and/or relatives, initial localization, clinical type, surface area involved, area of involvement in the oral mucosa, and Introduction: Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa. Objectives: This study aimed to determine the demographic, clinical and treatment characteristics of PV patients with oral mucosal involvement and to assess the impact on their quality of life. Methods: We conducted a prospective observational study among 106 patients diagnosed with PV and presenting oral mucosal involvement. Demographic data, clinical and treatment characteristics, and quality of life questionnaires were recorded. Results: The study included 106 patients, 55 (51.89%) were male and there was a predominance of the mucocutaneous subtype in 83 individuals (78.38%). Oral mucosa was the initial site of manifesta- tion in 44 patients (41.51%). Bilateral buccal mucosa was the most frequently affected site. The pre- dominant symptom reported was a burning sensation, noted in 91 patients (85.85%). Oral mucosal examination revealed erosions in 85.85% of the patients. Systemic steroids were the most commonly administered treatment, and rituximab was used in 18 patients (16.98%). A positive and significant correlation was found between pemphigus severity and Oral Health Impact Profile-14, Dermatology Life Quality Index and Dermatological Quality of Life Scale scores (P < 0.05). The presence of super- ficial ulcers, flaccid bullae, lesion diameter ≥1 cm, and >10 lesions were factors that markedly dimin- ished quality of life. Complete response to treatment was noted in all patients administered rituximab. Conclusions: The most common area of involvement was bilateral buccal mucosa, and the severity of PV closely correlated with a decline in quality of life measures. These results highlight the need for careful clinical oversight of PV, taking into account its effects on patients quality of life. ABSTRACT Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 3 symptoms and findings in the oral mucosa), oral mucosal lesion type, number, diameter, disease severity, and disease activity and variables related to treatment and prognosis. When assessing the severity of the disease, the Pemphi- gus Disease Severity Index (PDAI), a Pemphigus-Specific Severity Assessment scale developed in 2009 by Rosenbach et al, scored separately according to the number and diame- ter of lesions on the localized skin (12 anatomical regions), mucosal surface (12 anatomical regions), and scalp was used—250 points (120 points for the skin, 120 points for the mucosa, 10 points for the scalp) indicate disease activity and 13 points (12 points for the skin and 1 point for the scalp) indicate disease damage [11]. While evaluating the quality of life, the Oral Health Impact Profile-14 (OHIP14-TR) scale, Dermatology Life Quality Index (DLQI), and Dermatologi- cal Quality of Life Scale (DQLS) were used. Oral Health Impact Profile Scale The Oral Health Impact Profile (OHIP), which was first developed in Australia and consisted of 49 items, has been shortened due to its length, which made it time-consuming [12-14]. Basol et al, in 2014, developed and evaluated the Turkish OHIP14-TR, and its validity and reliability were demonstrated. The total scale score range is 0–56 [15]. Dermatology Life Quality Index The DLQI scale, first created by Finlay and Khan in 1994, is an easy-to-use scale in practice and has been shown to be valid and reliable in Turkey [16,17]. Dermatological Quality of Life Scale There are 11 questions in the DQLS developed by Gurel et al, and the total score range is 0–44 [18]. Statistical analyses were performed with the SPSS ver- sion 23.0 program. The conformity of the variables to the normal distribution was examined by histogram graphs and Kolmogorov-Smirnov/Shapiro-Wilk test. Mean, standard deviation, and median values were used when presenting de- scriptive analyses. The Mann–Whitney U test was used when evaluating non-normally distributed (nonparametric) vari- ables between two groups, while the Kruskal–Wallis test was used when evaluating between more than two groups. The Bonferroni multiple comparison tests were used while inves- tigating the reason for the significant difference between the groups. While presenting the categorical variables, the fre- quency and percentage values of the variables were used, and the analysis of the categorical variables was carried out with the chi-square (exact) test. The Spearman correlation test was used to evaluate the relationships between quantitative variables. Cases with a P value below 0.05 were considered statistically significant results. Results A total of 106 patients with PV with newly diagnosed oral mucosal involvement in 16 different dermatology clinics from different regions of Turkey were included in this study. Demographic and Clinical Characteristics of the Patients The mean age of all the patients was 50.06 ± 14.88 and 51.89% (N = 55) were males. While 34.91% were primary school graduates, 16.04% were university graduates. Body mass index was found to be 26.88 ± 4.04 (26.77). The most common personal disease history was diabetes (17.92%), followed by hypertension and coronary artery disease ( Table 1). Of the patients, 19.81% were active smokers and 9.43% were regular alcohol users. Among the autoimmune diseases, Hashimoto thyroiditis was found in four patients, rheumatoid arthritis in two, Sjögren syndrome in one, and Graves in one. Clinical Features of Pemphigus Disease The mean age of pemphigus onset was 49.45 ± 14.98 years, the mean duration of illness was 7.59 ± 8.57 months, and the mean delay in diagnosis was 4.39 ± 5.26 months. There was no family history of pemphigus in 99.06% of the patients. Mucocutaneous subtype was found in 78.38% of the patients. While the initial localization of 41.51% of the pa- tients was only the oral mucosa, 7.55% had only the skin, 40.57% had the oral mucosa and skin simultaneously, and 7.55% had the first lesions on the skin. The body surface area involved was localized in 50.94% of the patients. The involvement of the oral mucosa with PV in 63.21% of the patients was in the bilateral buccal mucosa, fol- lowed by the lower lip (46.23%) and the tongue (45.28%). In 98.11% of the patients, symptoms were present in the oral mucosa and most commonly (85.85%) accompanied by burning; 77.36% had pain and 0.94% had difficulty swallowing. Oral mucosal examination revealed erosions in 85.85% of the patients, superficial ulcers in the oral mucosa in 66.04%, small vesicles in 12.26%, and flaccid bullae in 10.38%. Of the patients, 57.55% had lesion(s) 1 cm or more in diameter. While the mean PDAI was 27.58 ± 21.28, the mean damage score was 0.89 ± 3.01 (Table 1). The mean PDAI mucosal score, which indicates the severity of the dis- ease, was 15.19 ± 13.68 and the PDAI oral mucosa mean score was 13.85 ± 12.17. Treatment Features Systemic steroids were the most commonly administered treatment (N = 88, 83.02%). This was followed by topical steroid (N = 42, 39.62%) and rituximab (RTX) (N = 18, 16.98%) treatments. Other agents used in the treatment 4 Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 Table 1. Sociodemographic characteristics and disease-related features of the patients. Characteristics Mean ± SD or N (%) Age, x ̅ ± s.s. (median) (year) 50,06 ± 14.88 (52.00) Gender Female Male 51 (48.11) 55 (51.89) Body mass index (x ̅ ± s.s. (median) 26.88 ± 4.04 (26.77) Comorbidities Hypertension Diabetes Dyslipidemia Thyroid dysfunction Coronary artery disease Liver diseases Others (neurological, rheumatological, chronic obstructive pulmonary diseases) 17 (16.04) 19 (17.92) 4 (3.77) 6 (5.66) 12 (11.32) 2 (1.89) 6 (5.66) Duration of disease (months) 7.59 ± 8.57 (4.00) Delay in diagnosis (months) 4.39 ± 5.26 (3.00) Age-onset of pemphigus x ̅ ± s.s. (median) 49.45 ± 14.98 (51.00) Family history of pemphigus 1 (0.94) Retained body surface area Localized Generalized 57 (53.77) 49 (46.23) Clinical subtype Mucocutaneous Mucosal 83 (78.30) 23 (21.70) Onset location Oral mucosa Skin Oral mucosa and skin (both) Scalp Nasal mucosa Oral mucosa & skin & scalp Oral nasal anogenital Oral nasal skin 44 (41.51) 8 (7.55) 43 (40.57) 1 (0.94) 2 (1.89) 2 (1.89) 5 (4.72) 1 (0.94) Involvement site in the oral mucosa Bilateral buccal mucosa Hard palate Lower lip Tongue Gingiva Soft palate Upper lip Floor of mouth Oropharynx Unilateral buccal mucosa 67 (63.21) 50 (47.17) 49 (46.23) 48 (45.28) 43 (40.57) 40 (37.74) 33 (31.13) 29 (27.36) 22 (20.75) 18 (16.98) Symptom in the oral mucosa Burning Pain Dysphagia 104 (98.11) 91 (85.85) 82 (77.36) 1 (0.94) Clinical findings in the oral mucosa Erosions Superficial ulcer Small vesicles Flaccid bullae 91 (85.85) 70 (66.04) 13 (12.26) 11 (10.38) Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 5 21.37  ±  11.30, respectively) were statistically significantly higher than those with only mucosal involvement (P < 0.001 and P = 0.010, respectively). No statistically significant differ- ence was observed between the mean scores of OHIP14-TR (27.70 ± 13.53 and 24.57 ± 11.96, respectively) in the dis- ease progressing with involvement (P = 0.351) (Table 4). Conclusions PV is an autoimmune disease characterized by the devel- opment of autoantibodies against intracellular adhesion proteins in the epidermis and progresses with intraepithe- lial bullae and erosions in the skin and mucous membranes [1]. Oral mucosal involvement is quite high in the disease [19-21], and has been reported as the most common ini- tial localization in previous studies globally [22,23-25]. In our study, the most prevalent initial presentation was oral mucosal involvement, occurring in 41.51% of the patients, which is consistent with the literature. It is also noted that pemphigus vulgaris may manifest with concurrent cutane- ous and oral mucosal involvement which was observed in 40.57% of our study participants [23-25]. Additionally, the onset of the disease can occur in other mucosal areas includ- ing nasal, anogenital, conjunctival, as well as the laryngeal and pharyngeal regions. Notably, in our study, nasal muco- sal presentation was the initial sign in two individuals. Oral mucosal involvement typically manifests with symptoms such as pain, a burning sensation, and challenges in eating, were azathioprine (N = 16, 15.09%), intravenous immuno- globulin (IVIG) (N = 5, 4.72%), and mycophenolate mofetil (N = 5, 4.72%). Of the patients, 78.3% received inpatient treatment. Evaluation of Quality of Life Scales The mean DLQI of the patients was 11.6 ± 9.01, the DQLS mean 19.98 ± 11.34, and the OHIP14-TR mean was 27.02 ± 13.21. A positive and significant correlation was found between pemphigus severity and OHIP14-TR, DLQI, and DQLS scores (P <  0.05). There was a positive and signif- icant relationship between PDAI mucosa and OHIP14-TR and DLQI, as well as between PDAI oral mucosa and OHIP14-TR. While there was a moderately strong relation- ship between PDAI and DLQI, other significant relationships were of low strength (Table 2). OHIP14-TR and DLQI scores were significantly higher in patients with superficial ulcers in the oral mucosa than those without, and this was statistically significant (P < 0.05). OHIP14-TR and DQLS scores were significantly higher in patients with flaccid bullae in the oral mucosa than those without and were statistically significant (P < 0.05). Those with more than 10 lesions in the oral mucosa had a signifi- cantly higher DQLS score. Again, the OHIP14-TR score was found to be significantly higher in patients with a lesion di- ameter of 1 cm and above in the oral mucosa (Table 3). In the disease progressing with mucocutaneous involve- ment, the mean scores of DLQI and DQLS (13.24 ± 9.06 and Table 2. The relationship between pemphigus severity and quality of life scale scores. OHIP-14-TR DLQI DQLS PDAI r 0.193 0.494 0.232 P 0.047 < 0.000 0.017 PDAI mucosa r 0.207 0.208 0.016 P 0.033 0.032 0.870 PDAI oral mucosa r 0.194 0.184 0.016 P 0.046 0.059 0.869 DLQI = Dermatology Life Quality Index; DQLS = Dermatological Quality of Life Scale; OHIP-14-TR: Oral Health Impact Profile-14-TR scale; PDAI = Pemphigus disease area index. Characteristics Mean ± SD or N (%) Diameter of the lesion in the oral mucosa < 1 cm ≥ 1 cm 45 (42.45) 61 (57.55) PDAI, x ̅ ± s.s. (median) 27.58 ± 21.28 (20.50) Damage score 0.89 ± 3.01 PDAI = Pemphigus disease area index. Table 1. Sociodemographic characteristics and disease-related features of the patients. (continued) 6 Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 clinical subtype was mucocutaneous (72%), followed by mucosal (20%) subtype. Similarly, the most frequently ob- served subtype in our study was mucocutaneous [7]. Oral mucosal lesions can be observed as painful erosions, irregularly circumscribed ulcers, small vesicles, and loose bullae and bulla residues anywhere in the oral mucosa, often in the buccal mucosa and gingiva [29]. Uzun et al reported that the disease started with persistent oral ulcers and ero- sion in 101 patients with PV [22]. In our study, 85.85% of the patients had erosions, 66.04% had superficial ulcers in the oral mucosa, 12.26% had small vesicles, and 10.38% had loose bullae. Suliman et al study, the most common site of involvement was found to be the buccal mucosa [2]. In our study, the most common site of oral involvement was the bilateral buccal mucosa with a rate of 63.21%. The severity of oral involvement is variable. Lesions may be localized or widespread. Symptoms may differ depending on the area of involvement and the number of lesions and their diameter, and the impact on quality of life may differ. The lesion diam- eter was 1 cm and above in 57.55% of the patients. significantly impairing the patients quality of life. We found that OHIP14-TR was significantly affected in patients with superficial ulcers, loose bullae, and lesion diameter of 1 cm and above in the oral mucosa compared to those without. Again, the DYQS score was found to be significantly higher in patients with more than 10 lesions in the oral mucosa. Godshi et al found burning sensations in 83.1% of patients and pain in 68.4% [7]. This is consistent with our findings in which the most common symptom was burning (n = 91, 85.85%), followed by pain (N = 82, 77.36%), while one patient had swallowing difficulties. In the literature, several studies from various countries have evaluated the quality of life of patients with autoim- mune bullous diseases. The number of patients with PV in these studies ranged from 32 to 43 [4-6]. Only Ghodsi et al have evaluated the quality of life in a study involv- ing 61 patients with a diagnosis of PV [7]. In this context, various scales were used [9,11,26], as there are different quality-of-life scales for diseases with oral mucosal involve- ment [13,27,28]. In Godshi et al study, the most common Table 4. Comparison of quality of life scale score levels according to clinical type. Clinical type P Mucocutaneous Mucosal Mean Std. Median Mean Std. Median OHIP-14-TR 27.70 ±13.53 28.00 24.57 ±11.96 26.00 0.351 DLQI 13.24 ±9.06 12.00 5.70 ±5,89 4.00 < 0.001 DQLS 21.37 ±11.30 21.00 14.96 ±10.19 13.00 0.010 DLQI = Dermatology Life Quality Index; DQLS = Dermatological Quality of Life Scale; OHIP-14-TR: Oral Health Impact Profile-14-TR scale. Table 3. Comparison of quality of life scale score levels according to oral mucosa. OHIP14-TR P DLQI P DQLS PMean Std. Median Mean Std. Median Mean Std. Median Superficial ulcer Absent 22.53 ±13.11 20.00 0.007 9.53 ±9.32 7.50 0.035 17.61 ±11.44 15.00 0.081 Present 29.33 ±12.75 30.50 12.67 ±8.72 12.00 21.20 ±11.17 21.00 Erosions Absent 27.00 ±11.35 29.00 0.989 10.60 ±8.10 8.00 0.768 19.87 ±11.80 19.00 0.939 Present 27.02 ±13.55 27.00 11.77 ±9.18 11.00 20.00 ±11.33 19.00 Small vesicles Absent 26.42 ±13.43 26.00 0.143 11.17 ±9.07 10.00 0.113 19.77 ±11.37 19.00 0.441 Present 31.31 ±11.05 33.00 14.69 ±8.23 16.00 21.46 ±11.43 26.00 Flaccid bullae Absent 26.07 ±13.31 26.00 0.034 11.13 ±8.98 10.00 0.077 19.25 ±11.44 18.00 0.034 Present 35.18 ±9.33 35.00 15.73 ±8.59 15.00 26.27 ±8.49 28.00 Number of lesions < 3 19.94 ±13.50 15.50 0.314 11.38 ±10.64 9.00 0.515 18.81 ±11.36 19.00 < 0.001 3–5 28.29 ±13.44 29.50 9.65 ±7.52 9.00 18.12 ±10.76 19.00 6–10 22.11 ±12.16 20.00 11.37 ±8.22 10.00 20.11 ±10.33 18.00 > 10 34.00 ±10.12 34.00 14.24 ±10.11 14.00 22.69 ±12.85 23.00 Lesion diameter < 1 cm 23.18 ±12.56 23.00 0.013 11.24 ±8.54 10.00 0.883 18.62 ±9.82 19.00 0.360 ≥ 1 cm 29.85 ±13.06 32.00 11.87 ±9.40 11.00 20.98 ±12.32 19.00 DLQI = Dermatology Life Quality Index; DQLS = Dermatological Quality of Life Scale; OHIP-14-TR: Oral Health Impact Profile-14-TR scale. Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 7 superficial ulcers, flaccid bullae, lesions measuring 1 cm or more in diameter, and those with more than ten lesions within the oral cavity. A successful response was observed in all the patients that used RTX as a treatment agent. References 1. Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11(3): 462-481. DOI: 10.1111/j.1582-4934.2007.00033.x. PMID: 17521 373; PMCID: PMC3922353. 2. Suliman NM, Astrom AN, Ali RW, Salman H, Johannessen AC. Clinical and histological characterization of oral pemphigus le- sions in patients with skin diseases: a cross sectional study from Sudan. BMC Oral Health. 2013;13:66. DOI: 10.1186/1472- 6831-13-66. PMID: 24261459; PMCID: PMC3871015. 3. Yayli S, Harman M, Baskan EB, et al. Epidemiology of Pemphi- gus in Turkey: One-year Prospective Study of 220 Cases. Acta Dermatovenerol Croat. 2017;25(3):181-188. PMID: 29252169. 4. Penha MA, Farat JG, Miot HA, Barraviera SR. Quality of life index in autoimmune bullous dermatosis patients. An Bras Dermatol. 2015;90(2):190-194. DOI: 10.1590/abd1806-4841.20153372. PMID: 25830988. PMCID: PMC4371667. 5. Sung JY, Roh MR, Kim SC. Quality of Life Assessment in Korean Patients with Pemphigus. Ann Dermatol. 2015;27(5):492-498. DOI: 10.5021/ad.2015.27.5.492. PMID: 26512162. PMCID: PMC4622882. 6. Krain RL, Kushner CJ, Tarazi M, et al. Assessing the Correla- tion Between Disease Severity Indices and Quality of Life Mea- surement Tools in Pemphigus. Front Immunol. 2019;10:2571. DOI: 10.3389/fimmu.2019.02571. PMID: 31781098. PMCID: PMC6851056. 7. Ghodsi SZ, Chams-Davatchi C, Daneshpazhooh M, Valikhani M, Esmaili N. Quality of life and psychological status of patients with pemphigus vulgaris using Dermatology Life Quality Index and General Health Questionnaires. J Dermatol. 2012;39(2):141-144. DOI: 10.1111/j.1346-8138.2011.01382.x. PMID: 21967321. 8. Atıcı A, Ünsal Avdal E. Investigation of the Quality of Life of Individuals with Autoimmune Bullous Disease Diagnosis. İzmir Kâtip Çelebi Üniversitesi Sağlık Bilimleri Fakültesi Dergisi. 2016;1(2):21-25. 9. Bilgic Temel A, Irican C, Uzun S, Feng GYH, Murrell DF, Akman Karakas A. Quality of Life in Turkish Patients with Autoimmune Blistering Diseases: Reliability and Validity of the Autoimmune Bullous Disease Quality of Life and the Treatment of Autoim- mune Bullous Disease Quality of Life Questionnaires. Turkish J Dermatol. 2019;13(2). 10. Hertl M, Jedlickova H, Karpati S, et al. Pemphigus. S2 Guideline for diagnosis and treatment--guided by the European Dermatol- ogy Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2015;29(3):405-414. DOI: 10.1111/jdv.12772. PMID: 25338479. 11. Rosenbach M, Murrell DF, Bystryn JC, et al. Reliability and convergent validity of two outcome instruments for pemphigus. J Invest Dermatol. 2009;129(10):2404-2410. DOI: 10.1038/ jid.2009.72. Epub 2009 Apr 9. PMID: 19357707. PMCID: PMC3010359. 12. John MT, Patrick DL, Slade GD. The German version of the Oral Health Impact Profile--translation and psychometric properties. PV is observed more frequently in individuals aged 40– 60 years. In the study conducted by Thansov et al0 over a 16-year period, the onset of the disease was observed in the 5th or 6th decade of life. Bozdag et alreported the mean age of onset of the disease as 48.3 ± 12.6 years [30,31]. In our study, similar to the literature, the median age at onset was 49.45 ± 14.98 (51.00) years. Pemphigus is observed more frequently in women according to most of the litera- ture [7,23,31]. In a retrospective study by Abdolsamadi et al in which they examined 20 years of data on patients with pemphigus in Tehran, 380 (56.9%) patients with PV with only oral mucosal involvement were females, while 288 (43.1%) were males [32]. In the same study, 146 (62.9%) of 232 patients with oral mucosa and skin involvement were females and 86 (37.1%) were males [32]. In the study con- ducted by Arduino et al with patients with OPV in Italy, 62.25% of the patients were females [20]. In contrast to the literature, most of the patients in our study were males (n = 55, 51.89%). The association of pemphigus with various autoimmune diseases has been reported in the literature. In Taiwan, Chiu et al examined the co-existing autoimmune diseases in pa- tients with pemphigus and observed that pemphigus was most commonly accompanied by Sjögren syndrome, pso- riasis, systemic lupus erythematosus, and alopecia areata. [33] In our study, Sjögren syndrome accompanied PV in one patient, while four patients had Hashimoto thyroiditis, one had Graves, rheumatoid arthritis, and two had rheu- matoid arthritis. Chiu et al found no statistically significant relationship between pemphigus and other diseases such as Graves disease, Hashimoto thyroiditis, pernicious ane- mia, rheumatoid arthritis, vitiligo, or ankylosing spondy- litis [33]. Regarding treatment, systemic steroids, azathioprine, mycophenolate mofetil, methotrexate, chlorambucil, cyclo- phosphamide, cyclosporine, rituximab, and IVIG can be used for patients with PV. Again, intralesional corticoste- roid injections, Orobase or inhaled steroids, and antiseptic mouthwashes are used to treat oral mucosal lesions [34]. In our study, the most frequently used treatment agent was a systemic steroid (N = 88, 83.02%). Fortuna et al used RTX as a combination therapy or alone in the treatment of 10 pa- tients with oral PV and reported a positive response in all the patients [35]. We treated 18 patients with OPV in our study with RTX and obtained a successful response in all of them. In our study, we determined the sociodemographic, clin- ical and treatment, and quality of life characteristics of PV disease with oral mucosal involvement were determined. The most common area of involvement was bilateral buccal mucosa, and the quality of life was affected in correlation with the severity of the disease. Notably, a marked decline in quality of life was noted among patients presenting with 8 Original Article | Dermatol Pract Concept. 2024;14(2):e2024099 24. Altun E, Yayli S, Selcuk LB, Arica DA, Bahadir S. Clinical and De- mographic Characteristics of Pemphigus Vulgaris Patients. Acta Dermatovenerol Croat. 2018;26(2):119-125. PMID: 29989867. 25. Michailidou EZ, Belazi MA, Markopoulos AK, Tsatsos MI, Mourellou ON, Antoniades DZ. Epidemiologic survey of pemphi- gus vulgaris with oral manifestations in northern Greece: retrospec- tive study of 129 patients. Int J Dermatol. 2007;46(4):356-361. p DOI: 10.1111/j.1365-4632.2006.03044.x. PMID: 17442072. 26. Sebaratnam DF, Hanna AM, Chee SN, et al. Development of a quality-of-life instrument for autoimmune bullous disease: the Autoimmune Bullous Disease Quality of Life questionnaire. JAMA Dermatol. 2013;149(10):1186-1191. DOI: 10.1001 /jamadermatol.2013.4972. PMID: 23925444. 27. Llewellyn CD, Warnakulasuriya S. The impact of stomatologi- cal disease on oral health-related quality of life. Eur J Oral Sci. 2003;111(4):297-304. L DOI: 10.1034/j.1600-0722.2003.00057 .x. PMID: 12887394. 28. Rajan B, Ahmed J, Shenoy N, Denny C, Ongole R, Binnal A. Assess- ment of quality of life in patients with chronic oral mucosal diseases: a questionnaire-based study. Perm J. 2014;18(1):e123-127. DOI: 10.7812/TPP/13-095. PMID: 24626087; PMCID: PMC3951046. 29. Baykal C. Dermatoloji Atlası. İstanbul: Nobel Tıp Kitabevi; 2012.PLEASE PROVIDE PAGES 30. Tsankov N, Vassileva S, Kamarashev J, Kazandjieva J, Kuzeva V. Epidemiology of pemphigus in Sofia, Bulgaria. A 16-year retro- spective study (1980-1995). Int J Dermatol. 2000;39(2):104-108. DOI: 10.1046/j.1365-4362.2000.00864.x. PMID: 10692058. 31. Bozdag K, Bilgin I. Epidemiology of pemphigus in the western region of Turkey: retrospective analysis of 87 patients. Cutan Ocul Toxicol. 2012;31(4):280-285. DOI: 10.3109/15569527 .2011.653598. PMID: 22309241. 32. Abdolsamadi HR, Abdollahzadeh S, Bakianian Vaziri P, Beheshti A, Shafigh E, Vahedi M. Epidemiology of pemphigus in tehran, iran: a 20-year retrospective study. J Dent Res Dent Clin Dent Prospects. 2007;1(3):108-113. DOI: 10.5681/joddd.2007.019. PMID: 23277844. PMCID: PMC3529885. 33. Chiu YW, Chen YD, Hua TC, Wu CH, Liu HN, Chang YT. Comor- bid autoimmune diseases in patients with pemphigus: a nation- wide case-control study in Taiwan. Eur J Dermatol. 2017;27(4): 375-381. DOI: 10.1684/ejd.2017.3060. PMID: 28747284. 34. Bolognia JL, Schaffer JV, Duncan KO, Ko C. Dermatology Essen- tials. Elsevier Saunders; 2014.PLEASE PROVIDE PAGES 35. Fortuna G, Calabria E, Ruoppo E, et al. The use of rituximab as an adjuvant in the treatment of oral pemphigus vulgaris. J Oral Pathol Med. 2020;49(1):91-95. DOI: 10.1111/jop.12951. PMID: 31420993. Eur J Oral Sci. 2002;110(6):425-433. DOI: 10.1034/j.1600-0722. 2002.21363.x. PMID: 12507215. 13. Slade GD, Spencer AJ. Development and evaluation of the Oral Health Impact Profile. Community Dent Health. 1994;11(1): 3-11. PMID: 8193981. 14. Slade GD. Derivation and validation of a short-form oral health im- pact profile. Community Dent Oral Epidemiol. 1997;25(4):284-290. SDOI: 10.1111/j.1600-0528.1997.tb00941.x. PMID: 9332805. 15. Başol ME, Karaağaçlıoğlu L, Yılmaz B. Developing a Turkish Oral Health Impact Profile-OHIP-14-TR. Turkiye Klinikleri J Dental Sci. 2014;20(2):85-92. 16. Finlay AY, Khan GK. Dermatology Life Quality Index (DLQI)—a simple practical measure for routine clinical use. Clin Exp Der- matol. 1994;19(3):210-216. Fi DOI: 10.1111/j.1365-2230.1994. tb01167.x. PMID: 8033378. 17. Ozturkcan S, Ermertcan AT, Eser E, Sahin MT. Cross valida- tion of the Turkish version of dermatology life quality index. Int J Dermatol. 2006;45(11):1300-1307. DOI: 10.1111/j.1365- 4632.2006.02881.x. PMID: 17076710. F 18. Gurel MS, Yanik M, Simsek Z, Kati M, Karaman A. Qual- ity of life instrument for Turkish people with skin diseases. Int J Dermatol. 2005;44(11):933-938. DOI: 10.1111/j.1365- 4632.2004.02225.x. PMID: 16336527. 19. Daltaban O, Ozcentik A, Akman Karakas A, Ustun K, Hatipoglu M, Uzun S. Clinical presentation and diagnostic delay in pem- phigus vulgaris: A prospective study from Turkey. J Oral Pathol Med. 2020;49(7):681-686. DOI: 10.1111/jop.13052. PMID: 32516514. 20. Arduino PG, Broccoletti R, Carbone M, et al. Long-term eval- uation of pemphigus vulgaris: A retrospective consideration of 98 patients treated in an oral medicine unit in north-west Italy. J Oral Pathol Med. 2019;48(5):406-412. DOI: 10.1111 /jop.12847. PMID: 30860627. 21. Kavala M, Zindanci I, Turkoglu Z, Kuru BC, Ozlu E, Simsek M. Nineteen-year retrospective evaluation of pemphigus in a single dermatology centre in Istanbul, Turkey. Postepy Dermatol Aler- gol. 2020;37(1):23-28. DOI: 10.5114/ada.2020.93380. PMID: 32467679. PMCID: PMC7247063. 22. Uzun S, Durdu M, Akman A, et al. Pemphigus in the Mediter- ranean region of Turkey: a study of 148 cases. Int J Dermatol. 2006;45(5):523-528. DOI: 10.1111/j.1365-4632.2004.02533.x. PMID: 16700784. 23. Karaosmanoğlu N, Karaaslan E, İmren Baskovski IG, Kıratlı E, Ekşioğlu HM. The evaluation of autoimmune bullous diseases: a retrospective analysis of 63 cases. Med J Ankara Tr Res Hosp. 2018;51(3):223-228.