Dicle Üniversitesi Tıp Fakültesi hastanesi dermatoloji polikliniğine başvuran hastalarda saptanan otoimmün büllöz hastalıklar
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Tarih
2021
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Dicle Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Giriş ve Amaç: Otoimmün büllöz hastalıklar (OBH) deride ve/veya mukozalarda bulunan çeşitli otoantijenlere karşı otoantikorların oluşması sonucunda klinik olarak başta oral mukoza olmak üzere mukozalarda ve/veya deride vezikülobüllöz lezyonlarla karakterize nadir görülen bir grup hastalıktır. Bizim amacımız bir yıllık süre zarfında dermatoloji polikliniğine başvuran ve/veya dermatoloji kliniğine kabul edilen hastalarda saptanan yeni tanı almış tüm otoimmün büllöz hastalıkları sayı ve oran olarak tespit etmekti. Dicle Üniversitesi Tıp Fakültesi Hastanesi Güneydoğu Anadolu Bölgesinde yer alan referans merkezlerden biri olarak kabul edildiğinden yürüttüğümüz bu çalışma ile Diyarbakır ve çevre illerde otoimmün büllöz hastalıkların yıllık insidansı ve epidemiyolojisi hakkında bilgi edinilmesi amaçlandı. Gereç ve Yöntem: Bu çalışmada 4 Mayıs 2020-4 Mayıs 2021 tarihleri arasındaki bir yıllık periyotta Dicle Üniversitesi Tıp Fakültesi Deri ve Zührevi Hastalıkları polikliniğine başvuran ve/veya dermatoloji kliniğinde yatarak tedavi gören, yaş sınırlaması ve herhangi bir dışlama kriteri olmaksızın ilk kez otoimmün büllöz hastalık tanısı almış, bilgilendirilmiş gönüllü olur formu imzalayan toplam 52 hasta değerlendirildi. Bulgular: Bu çalışma ile belirtilen süre zarfında ilk kez otoimmün büllöz hastalık tanısı alan toplam 52 hasta değerlendirildi. Hastaların 27'sinin kadın (%51,9), 25'inin erkek (%48,1) ve yaş ortalamalarının 52,67±25,262 (yıl) olduğu saptandı. Olguların 28'i (%53,8) pemfigus, 24'ü ise (%46,2) pemfigoid grubundaydı. Otoimmün büllöz hastalıklar alt gruplarına göre incelendiğinde; pemfigus grubunda 22 hastanın (%42,3) pemfigus vulgaris, 5 hastanın (%9,6) pemfigus foliaseus, 1 hastanın (%1,9) paraneoplastik pemfigus olduğu, pemfigoid grubunda ise 19 hastanın (%36,5) büllöz pemfigoid, 3 hastanın (%5,8) lineer IgA dermatozu, 1 hastanın (%1,9) edinsel epidermolizis bülloza, 1 hastanın (%1,9) ise çocukluk çağının kronik büllöz dermatozu olduğu saptandı. Çalışmamızda polikliniğimize başvuran hastalarda en sık saptanan otoimmün büllöz hastalık (OBH) %42,3'lük oran ile pemfigus vulgaris, ikinci en sık %36,5'lik oran ile büllöz pemfigoid olmuştur. Tüm pemfigus olgularının büllöz pemfigoid olgularına oranı 1,47 olarak saptandı. Pemfigus vulgaris hastalarının 9'u, büllöz pemfigoid hastalarının 12'si Diyarbakır ilinde ikamet ediyordu. Diyarbakır ilinde pemfigus vulgaris insidansının en az milyonda 5,00, büllöz pemfigoid insidansının ise en az milyonda 6,66 olduğu saptandı. Büllöz pemfigoid hastalarının yaş ortalamasının 72,84±10,495 (yıl), pemfigus vulgaris hastalarının yaş ortalamasının 42,55±19,680 (yıl) olduğu saptanmıştır. Olgular otoimmün büllöz hastalığa eşlik eden diğer hastalıklar açısından incelendiğinde; %23,1'inde hipertansiyon, %21,2'sinde endokrinopati, %9,6'sında kardiyolojik hastalık, %5,8'inde nörolojik hastalık, %5,8'inde astım/KOAH, %3,8'inde diğer deri hastalıkları, %1,9'unda malignite ve %1,9'unda hematolojik hastalıklar mevcut idi. Büllöz pemfigoid ile hipertansiyon, endokrinopatiler, kardiyolojik hastalıklar arasında istatistiksel olarak anlamlı bir ilişki olduğu saptandı (p<0,05). Pemfigus vulgaris ile hipertansiyon, endokrinopatiler, kardiyolojik hastalıklar arasında istatistiksel olarak anlamlı bir ilişki olduğu saptandı (p<0,05). Sonuçlar: Literatürde pemfigus ve pemfigoid grubu hastalıkları komorbid durumlarıyla birlikte birbirleriyle karşılaştıran, prospektif olarak yürütülmüş nadir epidemiyolojik çalışma mevcuttur. Yürüttüğümüz bu çalışma, Diyarbakır ve çevre illerde görülen otoimmün büllöz hastalıkların yıllık insidansı ve epidemiyolojisi hakkında katkı sağlamıştır. Büllöz pemfigoidin yaşlı popülasyonda gelişiyor olması ve aynı zamanda bu popülasyondaki tıbbi komorbiditelerin daha yüksek bir prevalansa sahip olması, bu hastalıkların neden bir arada görüldüğünü açıklayabilir; yine de patogenezlerinde bazı ortak yolakların yer alıp almadığı araştırmaya açık bir konudur. Pemfigoid ve pemfiguslu hastalarda bazı hastalıkların ortak patofizyolojik yolaklar nedeniyle daha yaygın olup olmadığını veya tamamen tesadüfi olup olmadığını anlamak için daha fazla çalışma yapılmasına ihtiyaç vardır.
Introduction and Aim: Autoimmune bullous diseases (AIBD) are a rare group of diseases characterized clinically by vesiculobullous lesions on the mucous membranes, primarily the oral mucosa, and/or skin, as a result of the formation of autoantibodies against various autoantigens in the skin and/or mucous membranes. Our aim was to determine the number and rate of all the patients diagnosed an autoimmune bullous disease for the first time who applied to the dermatology outpatient department and/or were hospitalized in the dermatology clinic during a one-year period. Since Dicle University Medical Faculty Hospital is regarded as one of the reference centers in the Southeastern Anatolia Region, it was aimed to obtain information about the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. Materials and Methods: In this study, a total of 52 patients who applied to the Dicle University Faculty of Medicine Dermatology and Venereal Diseases polyclinic and/or were hospitalized in the dermatology clinic between May 4, 2020 and May 4, 2021 were evaluated. The patients included in the study were the ones diagnosed with an autoimmune bullous disease for the first time without age restriction and any exclusion criteria, and signed an informed consent form before inclusion in the study. Results: A total of 52 patients diagnosed with autoimmune bullous disease for the first time during the specified period were evaluated in this study. It was determined that 27 (51.9%) of the patients were female, 25 (48.1%) were male, and their mean age was 52.67±25.262 (years). Twenty-eight (53.8%) of the cases were in the pemphigus group and 24 (46.2%) were in the pemphigoid group. When examined according to autoimmune bullous diseases subgroups, it was seen that in the pemphigus group; 22 patients (42.3%) had pemphigus vulgaris, 5 patients (9.6%) had pemphigus foliaceus, 1 patient (1.9%) had paraneoplastic pemphigus, and in the pemphigoid group; 19 patients (36.5%) had bullous pemphigoid, 3 patients (5.8%) had linear IgA dermatosis, 1 patient (1.9%) had acquired epidermolysis bullosa and 1 patient (1.9%) had chronic bullous dermatosis of childhood. In our study, the most frequently seen autoimmune bullous disease (AIBD) in the patients admitted to our clinic was pemphigus vulgaris with a rate of 42.3%, and bullous pemphigoid with a rate of 36.5% was the second most common. The ratio of all pemphigus cases to bullous pemphigoid cases was 1.47. Nine of the pemphigus vulgaris patients and 12 of the bullous pemphigoid patients were residing in Diyarbakır. It was determined that the incidence of pemphigus vulgaris in Diyarbakır was at least 5.00 per million, and the incidence of bullous pemphigoid was at least 6.66 per million. The mean age of bullous pemphigoid patients was 72.84±10,495 (years), and the mean age of pemphigus vulgaris patients was 42.55±19,680 (years). When the cases were examined in terms of other diseases accompanying autoimmune bullous disease; hypertension in 23.1%, endocrinopathy in 21.2%, cardiological disease in 9.6%, neurological disease in 5.8%, asthma/COPD in 5.8%, other skin diseases in 3.8%, malignancy in 1.9%, and hematological diseases in 1.9% were determined. A statistically significant association was found between bullous pemphigoid and hypertension, endocrinopathies and cardiological diseases (p<0.05). A statistically significant association was also found between pemphigus vulgaris and hypertension, endocrinopathies and cardiological diseases (p<0.05). Conclusion: There are rare prospective epidemiological studies in the literature comparing pemphigus and pemphigoid group diseases with their comorbid conditions. This study we conducted contributed to the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. The fact that bullous pemphigoid develops in the elderly population and medical comorbidities have a higher prevalence in this population may explain why these diseases occur together; however, whether some common pathways are involved in their pathogenesis is open to research. Further studies are needed to understand whether certain diseases are more common in patients with pemphigoid and pemphigus due to common pathophysiological pathways or whether they are purely coincidental.
Introduction and Aim: Autoimmune bullous diseases (AIBD) are a rare group of diseases characterized clinically by vesiculobullous lesions on the mucous membranes, primarily the oral mucosa, and/or skin, as a result of the formation of autoantibodies against various autoantigens in the skin and/or mucous membranes. Our aim was to determine the number and rate of all the patients diagnosed an autoimmune bullous disease for the first time who applied to the dermatology outpatient department and/or were hospitalized in the dermatology clinic during a one-year period. Since Dicle University Medical Faculty Hospital is regarded as one of the reference centers in the Southeastern Anatolia Region, it was aimed to obtain information about the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. Materials and Methods: In this study, a total of 52 patients who applied to the Dicle University Faculty of Medicine Dermatology and Venereal Diseases polyclinic and/or were hospitalized in the dermatology clinic between May 4, 2020 and May 4, 2021 were evaluated. The patients included in the study were the ones diagnosed with an autoimmune bullous disease for the first time without age restriction and any exclusion criteria, and signed an informed consent form before inclusion in the study. Results: A total of 52 patients diagnosed with autoimmune bullous disease for the first time during the specified period were evaluated in this study. It was determined that 27 (51.9%) of the patients were female, 25 (48.1%) were male, and their mean age was 52.67±25.262 (years). Twenty-eight (53.8%) of the cases were in the pemphigus group and 24 (46.2%) were in the pemphigoid group. When examined according to autoimmune bullous diseases subgroups, it was seen that in the pemphigus group; 22 patients (42.3%) had pemphigus vulgaris, 5 patients (9.6%) had pemphigus foliaceus, 1 patient (1.9%) had paraneoplastic pemphigus, and in the pemphigoid group; 19 patients (36.5%) had bullous pemphigoid, 3 patients (5.8%) had linear IgA dermatosis, 1 patient (1.9%) had acquired epidermolysis bullosa and 1 patient (1.9%) had chronic bullous dermatosis of childhood. In our study, the most frequently seen autoimmune bullous disease (AIBD) in the patients admitted to our clinic was pemphigus vulgaris with a rate of 42.3%, and bullous pemphigoid with a rate of 36.5% was the second most common. The ratio of all pemphigus cases to bullous pemphigoid cases was 1.47. Nine of the pemphigus vulgaris patients and 12 of the bullous pemphigoid patients were residing in Diyarbakır. It was determined that the incidence of pemphigus vulgaris in Diyarbakır was at least 5.00 per million, and the incidence of bullous pemphigoid was at least 6.66 per million. The mean age of bullous pemphigoid patients was 72.84±10,495 (years), and the mean age of pemphigus vulgaris patients was 42.55±19,680 (years). When the cases were examined in terms of other diseases accompanying autoimmune bullous disease; hypertension in 23.1%, endocrinopathy in 21.2%, cardiological disease in 9.6%, neurological disease in 5.8%, asthma/COPD in 5.8%, other skin diseases in 3.8%, malignancy in 1.9%, and hematological diseases in 1.9% were determined. A statistically significant association was found between bullous pemphigoid and hypertension, endocrinopathies and cardiological diseases (p<0.05). A statistically significant association was also found between pemphigus vulgaris and hypertension, endocrinopathies and cardiological diseases (p<0.05). Conclusion: There are rare prospective epidemiological studies in the literature comparing pemphigus and pemphigoid group diseases with their comorbid conditions. This study we conducted contributed to the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. The fact that bullous pemphigoid develops in the elderly population and medical comorbidities have a higher prevalence in this population may explain why these diseases occur together; however, whether some common pathways are involved in their pathogenesis is open to research. Further studies are needed to understand whether certain diseases are more common in patients with pemphigoid and pemphigus due to common pathophysiological pathways or whether they are purely coincidental.
Açıklama
Anahtar Kelimeler
Otoimmün büllöz hastalık, Pemfigus, Pemfigoid, Epidemiyoloji, Autoimmune bullous disease, Pemphigus, Pemphigoid, Epidemiology
Kaynak
WoS Q Değeri
Scopus Q Değeri
Cilt
Sayı
Künye
Uçan, E. (2021). Dicle Üniversitesi Tıp Fakültesi hastanesi dermatoloji polikliniğine başvuran hastalarda saptanan otoimmün büllöz hastalıklar. Yayımlanmamış uzmanlık tezi, Dicle Üniversitesi, Diyarbakır.
