Pęcherzyca pęcherzowa
Epidemiologia

Pęcherzyca pęcherzowa (BP) to najczęstsza autoimmunologiczna choroba pęcherzowa o podłożu podnaskórkowym, charakteryzująca się rosnącą zapadalnością, szczególnie w populacji osób starszych. Roczna zapadalność w populacji ogólnej wynosi od 2,4 do 23 przypadków na milion, z wyraźnym wzrostem u osób powyżej 80 roku życia (190-312 przypadków na milion) i najwyższą częstością w wieku 90-99 lat (81,9 na 100 000 w Szwecji). Wzrost zapadalności w ostatnich dwóch dekadach (1,9- do 4,3-krotny) przypisuje się starzeniu się populacji, ekspozycji na leki, poprawie diagnostyki klinicznej i laboratoryjnej. W 2020 roku w siedmiu głównych rynkach (USA, Niemcy, Francja, Włochy, Hiszpania, Wielka Brytania, Japonia) zdiagnozowano 124 517 przypadków BP, z prognozowanym wzrostem przy CAGR 0,96% do 2030 roku. Choroba dotyka głównie osoby w wieku 70-80 lat, bez istotnych różnic płciowych, choć niektóre badania wskazują na nieznaczną przewagę kobiet (np. 56% przypadków w USA w 2020 roku). Epidemiologia różni się regionalnie, z wyższą zapadalnością w Europie (2,5-42,8/milion) niż w Azji (2,6-7,5/milion).

Epidemiologia pęcherzycowego pemphigoidu

Pęcherzyca pęcherzowa (bullous pemphigoid, BP) to najczęstsza autoimmunologiczna choroba pęcherzowa skóry i błon śluzowych o podłożu podnaskórkowym. Wiedza na temat epidemiologii tej choroby znacznie się rozwinęła w ostatnich latach, wskazując na rosnącą częstość występowania oraz istotne obciążenie epidemiologiczne, szczególnie wśród osób starszych.12

Występowanie i zapadalność

Roczna zapadalność na pęcherzycę pęcherzową wynosi od 2,4 do 23 przypadków na milion osób w populacji ogólnej. Wartości te znacznie różnią się w zależności od regionu świata oraz metodologii badań.34 W Stanach Zjednoczonych odnotowuje się 6-13 nowych przypadków na milion osób rocznie, podczas gdy w Europie Środkowej liczba ta wynosi 12-13 na milion.3 Badania przeprowadzone w Wielkiej Brytanii wykazały zapadalność na poziomie 4,3 przypadków na 100 000 osobolat (co odpowiada 43 przypadkom na milion rocznie).56

Warto zauważyć, że zapadalność na pęcherzycę pęcherzową znacząco różni się w zależności od regionu:78

  • We Francji: 21,7 przypadków na milion mieszkańców w latach 2000-2005, co stanowi ponad 3-krotny wzrost w porównaniu do lat 1986-1992 (6,7 przypadków na milion)
  • W Wielkiej Brytanii: wzrost z 10,0 przypadków na milion w 1985 roku do 42,8 przypadków na milion w latach 2001-2004
  • W Izraelu: 1,9-krotny wzrost z 7,6 do 14,3 przypadków na milion między okresami 2000-2005 a 2011-2015
  • W Hiszpanii: 0,2-3 przypadki na 100 000 mieszkańców rocznie (2-30 na milion)
  • W Szwecji: 7,1 przypadków na 100 000 mieszkańców rocznie (71 na milion)

910

Szacuje się, że częstość występowania (prevalence) pęcherzycy pęcherzowej w Europie wynosi około 1/4000 (250 pacjentów na milion).11 W Niemczech w 2014 roku chorobowość oszacowano na 259 przypadków na milion populacji, co oznacza, że około 21 000 pacjentów żyło z tą chorobą.7 W Anglii w 2017 roku około 50 na 100 000 dorosłych żyło z pęcherzycą pęcherzową, a skupiając się wyłącznie na osobach starszych (powyżej 60 lat), ponad 1 na 1000 osób starszych cierpiało na tę chorobę.12

Trendy epidemiologiczne

Obserwuje się wyraźny trend wzrostowy w zapadalności na pęcherzycę pęcherzową, z 1,9- do 4,3-krotnym wzrostem w ciągu ostatnich dwóch dekad, co wykazano w badaniach z Wielkiej Brytanii, Francji, Niemiec i Izraela.7 Wzrost ten może być przypisany kilku czynnikom:12

  • Starzenie się populacji i zwiększona liczba osób z wieloma chorobami współistniejącymi
  • Ekspozycja na leki, które mogą potencjalnie wywoływać chorobę
  • Poprawa w diagnostyce klinicznej postaci bez pęcherzy
  • Zwiększenie dokładności technik laboratoryjnych do wykrywania autoprzeciwciał przeciwko białkom hemidesmosomów

Według analizy przeprowadzonej przez DelveInsight, całkowita liczba zdiagnozowanych przypadków pęcherzycy pęcherzowej w 2020 roku w siedmiu głównych rynkach (7MM: USA, Niemcy, Francja, Włochy, Hiszpania, Wielka Brytania i Japonia) wynosiła 124 517, z przewidywanym wzrostem do 2030 roku przy CAGR (złożonym rocznym wskaźniku wzrostu) na poziomie 0,96%.1314

Rozkład wiekowy i płciowy

Pęcherzyca pęcherzowa występuje przede wszystkim u osób starszych, głównie w ósmej dekadzie życia, choć zdarzają się również przypadki u młodszych pacjentów, a nawet u dzieci.111 Średni wiek w momencie diagnozy wynosi około 70-80 lat.15 Zapadalność na pęcherzycę pęcherzową rośnie wykładniczo z wiekiem:416

  • W populacji ogólnej: 2,4-23 przypadki na milion rocznie
  • U osób powyżej 80 roku życia: 190-312 przypadków na milion rocznie
  • U osób powyżej 90 lat: 300-krotnie wyższe ryzyko w porównaniu do osób w wieku 60 lat lub młodszych

1718

W Stanach Zjednoczonych w 2020 roku 47% wszystkich przypadków pęcherzycy pęcherzowej stanowiły osoby powyżej 80 roku życia.19 Badanie przeprowadzone w Szwecji wykazało, że największa zapadalność występuje w przedziale wiekowym 90-99 lat (81,9 na 100 000).9

Jeśli chodzi o rozkład płci, większość badań wskazuje na brak znaczącej różnicy między mężczyznami a kobietami, choć niektóre doniesienia sugerują nieznaczną przewagę wśród kobiet.35 W tureckim badaniu retrospektywnym stosunek kobiet do mężczyzn wynosił 1,42.15 Z kolei w Stanach Zjednoczonych w 2020 roku kobiety stanowiły 56%, a mężczyźni 44% wszystkich zdiagnozowanych przypadków.19

Geograficzne różnice w występowaniu

Badania epidemiologiczne wskazują na różnice w występowaniu pęcherzycy pęcherzowej w różnych regionach świata:12

  • Europa: 2,5-42,8 przypadków na milion rocznie
  • Azja: 2,6-7,5 przypadków na milion rocznie

Wśród krajów europejskich najwyższą liczbę przypadków pęcherzycy pęcherzowej w 2020 roku odnotowano w Wielkiej Brytanii (24 644 przypadki) i Francji (23 843 przypadki), natomiast najniższą w Hiszpanii (10 920 przypadków). Japonia miała najmniej przypadków spośród krajów 7MM, z 8 619 przypadkami.1920

W Stanach Zjednoczonych w 2020 roku zdiagnozowano 25 041 przypadków pęcherzycy pęcherzowej, z przewidywanym wzrostem przy CAGR 1,26% w latach 2018-2030.20 Różnice statystyczne między krajami mogą wynikać z rodzaju przeprowadzonych badań (retrospektywne vs. prospektywne), czasu trwania i zbieranych danych (krajowe bazy danych, przegląd dokumentacji medycznej).1

Ciężkość choroby i współwystępowanie

Pod względem ciężkości choroby, w Stanach Zjednoczonych w 2020 roku odnotowano 13 772 przypadki pęcherzycy pęcherzowej o nasileniu łagodnym do umiarkowanego oraz 11 268 przypadków o ciężkim przebiegu.1920

Pęcherzyca pęcherzowa jest często związana z chorobami współistniejącymi:2122

  • Choroby układu krążenia (w tym nadciśnienie tętnicze i choroba wieńcowa): 38-76,3% pacjentów
  • Cukrzyca: około 24% pacjentów
  • Choroby neurodegeneracyjne: 40,9% pacjentów
  • Przewlekła choroba nerek: 0,7% pacjentów
  • Osteoporoza: 0,7% pacjentów

Szczególnie istotne jest powiązanie między pęcherzycą pęcherzową a chorobami neurologicznymi. Przegląd systematyczny z metaanalizą obejmujący 14 badań z 23 369 pacjentami z BP i 128 697 osobami z grupy kontrolnej wykazał, że pacjenci z BP są 5 razy bardziej narażeni na rozwój zaburzeń neurologicznych, głównie stwardnienia rozsianego, demencji, choroby Parkinsona, padaczki i udaru mózgu, które zwykle poprzedzają wystąpienie BP o około 5,5 roku.23

Stwardnienie rozsiane ma najsilniejszy związek z BP, zwiększając ryzyko jej rozwoju 5-12 razy. Mechanizm leżący u podstaw tej korelacji może obejmować reakcję autoimmunologiczną na izoformy neuronalne BP180 i/lub BP230, które występują w mózgu ludzkim.2324

Umieralność związana z pęcherzycowym pemphigoidem

Pęcherzyca pęcherzowa wiąże się ze zwiększoną śmiertelnością w porównaniu z populacją ogólną. Standaryzowany wskaźnik umieralności (SMR) wynosi od 1,9 do 9,6, co oznacza, że pacjenci z BP mają nawet 9,6 razy wyższe ryzyko zgonu niż populacja ogólna w tym samym wieku.25

Badanie przeprowadzone w Finlandii wykazało, że SMR dla immunologicznie potwierdzonej BP wynosił 7,6, a śmiertelność jednoroczna i dwuletnia wynosiła odpowiednio 16,7% i 30,3%.22 W pierwszym roku po diagnozie śmiertelność wynosi od 10,8% do 41%.25

Przegląd systematyczny i metaanaliza obejmujące 25 badań wykazały, że połączony wskaźnik śmiertelności w ciągu pierwszego roku wynosi 23,5%. Najwyższe wskaźniki śmiertelności odnotowano w Europie (26,7%), następnie w Azji (20,5%) i USA (15,1%), co może być związane z większą liczbą chorób współistniejących i starszym wiekiem pacjentów europejskich w porównaniu z pacjentami amerykańskimi i azjatyckimi.23

W badaniu przeprowadzonym w Anglii wykazano, że osoby z pęcherzycą pęcherzową były trzy razy bardziej narażone na zgon w ciągu pierwszych dwóch lat po zdiagnozowaniu, a następnie o 50% bardziej narażone w porównaniu do podobnych osób bez tej choroby.12

Czynniki predysponujące i genetyczne

Badania wskazują na pewne czynniki predysponujące do rozwoju pęcherzycy pęcherzowej:26

  • Starszy wiek, szczególnie powyżej 80 lat
  • Choroby neurologiczne (udar, demencja, choroba Parkinsona)
  • Łuszczyca, zwłaszcza leczona fototerapią
  • Stosowanie określonych leków, szczególnie inhibitorów DPP-4

Istnieją również czynniki genetyczne predysponujące do rozwoju pęcherzycy pęcherzowej. U pacjentów rasy białej zidentyfikowano allel HLA-DQB1*0301, natomiast u pacjentów japońskich – allele DRB1*04, DRB1*1101 i DQB1*0302.3 Podobnie u pacjentów z mucous membrane pemphigoid (MMP) wśród białych pacjentów w Stanach Zjednoczonych stwierdzono związek z HLA-DQB1*0301.27

Nadzór epidemiologiczny nad pęcherzycowym pemphigoidem

Ze względu na rosnącą zapadalność i ciężar choroby, coraz większe znaczenie ma nadzór epidemiologiczny nad pęcherzycą pęcherzową.28 Większa świadomość choroby i jej związanej śmiertelności może przyczynić się do poprawy diagnostyki i leczenia pacjentów w przyszłości.

Metody nadzoru i zbierania danych

Nadzór epidemiologiczny nad pęcherzycą pęcherzową opiera się na różnych metodach zbierania danych:2930

  • Rejestry narodowe i populacyjne
  • Bazy danych szpitalne i ambulatoryjne
  • Systemy opieki zdrowotnej i ubezpieczeniowe
  • Badania kohortowe retrospektywne i prospektywne
  • Rejestry leków i działań niepożądanych

W Nowej Zelandii, w latach 1965-2018, Centrum Monitorowania Działań Niepożądanych (CARM) otrzymało 20 zgłoszeń reakcji pemphigoidowych podejrzewanych o związek z lekami.31 W Wielkiej Brytanii do zbierania danych wykorzystywano bazę danych praktyk lekarzy ogólnych, tzw. Clinical Practice Research Datalink (CPRD), natomiast w Niemczech opierano się na danych z ubezpieczeń zdrowotnych.732

Rejestry kliniczne i badania naukowe

Oprócz zbierania danych epidemiologicznych, prowadzone są także rejestry kliniczne i badania naukowe dotyczące pęcherzycy pęcherzowej:33

  • Badania kliniczne: co najmniej 60, w tym 2 aktywne, 22 zakończone i 14 w trakcie rekrutacji
  • Eksperckie ośrodki badawcze: 535 czołowych ekspertów medycznych w dziedzinie pęcherzycy pęcherzowej w 38 krajach i 30 stanach USA

Badania te wnoszą cenny wkład w lepsze zrozumienie choroby i opracowanie skuteczniejszych metod leczenia. Ważnym elementem tych badań jest również ocena aktywności choroby, np. przy użyciu wskaźnika aktywności choroby pęcherzycy pęcherzowej (Bullous Pemphigoid Disease Area Index, BPDAI), który posiada oddzielne punktacje dla aktywności skórnej i błon śluzowych.2634

Prognozy i przyszłe trendy

Analiza przyszłych trendów wskazuje na dalszy wzrost częstości występowania pęcherzycy pęcherzowej, co ma związek ze starzeniem się populacji i zwiększoną świadomością choroby wśród lekarzy.3536 Prognozy rynkowe przewidują, że rynek związany z leczeniem pęcherzycy pęcherzowej osiągnie wartość 1379,3 milionów dolarów do 2034 roku, przy CAGR na poziomie 21,15% w latach 2023-2034.37

Oczekuje się, że wzrost przypadków BP będzie wynikał z:36

  • Wzrostu populacji osób starszych
  • Zwiększenia czynników ryzyka, takich jak choroby neurologiczne
  • Stosowania określonych leków
  • Poprawy metod diagnostycznych

Ważnym elementem przyszłych badań epidemiologicznych będzie również analiza wpływu czynników środowiskowych i zawodowych na rozwój pęcherzycy pęcherzowej. Badanie przeprowadzone w Chinach wykazało wysoką częstość występowania autoimmunologicznych chorób pęcherzowych wśród pracowników przemysłu wymagającego ochrony zawodowej, co podkreśla pilną potrzebę wzmocnienia środków bezpieczeństwa i zwiększenia świadomości w tych sektorach.38

Globalny nadzór epidemiologiczny nad pęcherzycą pęcherzową jest niezbędny do lepszego zrozumienia ciężaru choroby i opracowania skutecznych strategii leczenia i zapobiegania. Dokładne dane epidemiologiczne stanowią podstawę do dalszych badań nad patogenezą, czynnikami ryzyka i nowymi metodami terapeutycznymi.

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 SciELO Brazil – Bullous pemphigoid Bullous pemphigoid
    https://www.scielo.br/j/abd/a/BsschZpGNWjSKJ3rFKBCXjm/
    Bullous pemphigoid is the most frequent autoimmune blistering disorder. It affects mainly elderly individuals, during the 8th decade of life, without gender predilection, with rare case reports of BP in children and adolescents. The incidence of BP has increased over the past decades as a result of population aging with multiple comorbidities and exposure to drugs that may potentially trigger the disease, as well as improvement in the clinical diagnosis of non-bullous presentations and in the accuracy of laboratory techniques to demonstrate the presence of autoantibodies against hemidesmosomal proteins. Epidemiological studies of BP in Europe demonstrate the incidence ranges from 2.5 to 42.8 cases/million/year, whereas in Asia the annual incidence is estimated in 2.6 to 7.5 cases/million. Statistical differences may be related to the type of study performed (retrospective vs. prospective), duration and collected data (national databases, chart review). The frequency of BP also increases in patients older than 80 years, with nearly 150-330 new cases/million/year at this age range.
  • #2 Bullous pemphigoid* | Anais Brasileiros de Dermatologia (Portuguese)
    https://www.anaisdedermatologia.org.br/pt-bullous-pemphigoid-articulo-S0365059620305778
    Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. […] The incidence of BP has increased over the past decades as a result of population aging with multiple comorbidities and exposure to drugs that may potentially trigger the disease, as well as improvement in the clinical diagnosis of non-bullous presentations and in the accuracy of laboratory techniques to demonstrate the presence of autoantibodies against hemidesmosomal proteins. […] Epidemiological studies of BP in Europe demonstrate the incidence ranges from 2.5 to 42.8 cases/million/year, whereas in Asia the annual incidence is estimated in 2.6 to 7.5 cases/million. […] The frequency of BP also increases in patients older than 80 years, with nearly 150-330 new cases/million/year at this age range.
  • #3 Bullous Pemphigoid – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK535374/
    Bullous pemphigoid affects individuals older than 60. Annual incidence is 6 to 13 new cases per million people in the United States, while it affects 12 to 13 per million in Central Europe. It has an equal incidence in males and females and no racial bias. […] Specific HLA class II alleles can be found in bullous pemphigoid patients, including the allele DQB1*0301 in White race patients and alleles DRB1*04, DRB1*1101, and DQB1*0302 in Japanese patients.
  • #4 The Growing Incidence of Bullous Pemphigoid: Overview and Potential Explanations
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6109638/
    Bullous pemphigoid (BP) is the most common type of subepidermal autoimmune bullous diseases. BP characteristically affects the elderly and is seen mainly in patients older than 70 years. While the annual incidence of BP has been estimated to be between 2.4 and 23 cases per million in the general population, it rises exponentially to 190312 cases per million in individuals older than 80 years. In addition, a growing body of evidence reports a remarkable trend of increased incidence of BP, showing a 1.9- to 4.3-fold rise over the past two decades. This demonstrable increase warrants a higher awareness of the increased risk to develop BP. This review summarizes the current understanding of the epidemiological features of BP and sheds light on the putative explanations for its growing incidence.
  • #5 Bullous Pemphigoid: Practice Essentials, Pathophysiology, Epidemiology
    https://emedicine.medscape.com/article/1062391-overview
    Bullous pemphigoid is uncommon, and its precise frequency has not been definitively established. […] In Europe, bullous pemphigoid has been identified as the most common subepidermal autoimmune blistering disease. In France and Germany, the reported incidence is 6.6 cases per million people per year. In a population-based cohort study from the United Kingdom, the incidence of bullous pemphigoid was found to be 4.3 cases per 100,000 person-years. […] Bullous pemphigoid primarily affects elderly individuals in the fifth through seventh decades of life (average age at onset, 65 y). Bullous pemphigoid of childhood onset has been reported in the literature. It has been suggested that the childhood-onset form may be more self-limited. […] The incidence of bullous pemphigoid appears to be equal in men and women. […] No racial predilection is apparent.
  • #6 Clinical review: Bullous pemphigoid | GPonline
    https://www.gponline.com/clinical-review-bullous-pemphigoid/dermatology/article/1072223
    Whenever tense bullae are present, bullous pemphigoid (BP), which is the most common autoimmune blistering condition in the developed world and one of the immunobullous disorders, must come to mind. […] The incidence has been underestimated in the past, but a recent study found it to be 4.3 (95% CI 4.0-4.6) per 100,000 person-years in the UK. […] It is likely that this figure still underestimates the true incidence, especially of those with milder forms of the condition. […] BP is a disease primarily affecting those over 70 years of age, so the incidence is expected to increase in future in countries with ageing populations. […] The mortality in patients with BP is twice that of the general population in the UK.
  • #7 The Growing Incidence of Bullous Pemphigoid: Overview and Potential Explanations
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6109638/
    The annual incidence of BP has been estimated to range between 2.4 and 21.7 new cases per million population in different populations worldwide. An even higher annual incidence of 42.8 cases per million population was reported in the United Kingdom (UK), although this report may be interpreted with caution as it is based on a computerized longitudinal general practice database. Also based on health insurance data, the prevalence of BP has recently been estimated at 259 per million population in Germany, i.e., ~21,000 patients with BP lived in Germany as for 2014. Despite this increase, BP is still considered as an orphan disease, i.e., 5 in 100,000 persons are affected by the disease. […] A growing incidence ranging from 1.9- to 4.3-fold in the past two decades has been reported in recent data from the UK, France, Germany, and Israel.
  • #8 The Growing Incidence of Bullous Pemphigoid: Overview and Potential Explanations
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6109638/
    In a retrospective cohort study, the annual incidence of BP was estimated at 21.7 cases per million population throughout the years 20002005 in three French regions: Haute-Normandie, Limousin, and Champagne-Ardennes. This figure represents more than 3-fold increase relative to the annual incidence previously estimated in three adjacent French regions between the years 19861992: Limousin, Touraine, and Picardie, with very similar demographic characteristics (6.7 cases per million population). […] A consistent rise of the annual incidence rates of BP has been reported in the UK during the last two decades; from a nadir of 10.0 cases per million population in 1985, to 14.0 cases per million population during 19912001, and up to 42.8 cases per million population during the 20012004. […] A recent population-based study investigating the epidemiology of BP in Northern Israel revealed a 1.9-fold increasing annual incidence: from 7.6 to 14.3 cases per million population in the calendar periods 20002005 and 20112015, respectively. This rise applied to both of the major ethnic populations residing in the region: Jews and Arabs.
  • #9
    https://link.springer.com/article/10.1007/s00403-017-1778-4
    The incidence of BP in Sweden remains sparse, as very few reliable studies have assessed the overall age- and gender-specific distribution of BPs incidence. […] From this large population-based cohort study, we estimated adjusted annual incidence rates of BP in Sweden to be 7.1/100,000 inhabitants. […] The overall incidence increased with age and reached its maximum between the ages of 90 and 99 years (81.9/100,000). […] The adjusted incidence rate of BP is 7.1/100,000 (95% CI 6.5-7.7). […] This is the first nationwide population-based registry study to present valid and robust data on the incidence of BP in an entire country. In total 3761 BP cases were included and the adjusted incidence rate was estimated to 7.1/100,000. This is the highest incidence level in Europe, thus confirming BP as a relatively common disease in the elderly population.
  • #10 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas
    https://actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778
    Bullous pemphigoid (BP) is the most common blistering disease in the adult population in developed countries, with an estimated incidence in Spain of 0.2 to 3 cases per 100,000 inhabitants per year. […] While recently published studies have pointed to an increase in recent years, a French study published in just 2012 reported an incidence of 21.7 cases per million inhabitants a year. […] The disease affects a similar proportion of men and women. Most patients are over 75 years old, but young adults and even children can also develop BP. […] Several cases reporting an association between BP and different malignant diseases have been published, but in one case-control study with matching for age and sex no increased risk for malignancy was observed in BP patients.
  • #11 Orphanet: Bullous pemphigoid
    https://www.orpha.net/en/disease/detail/703
    Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. It has an estimated prevalence of 1/4,000 in Europe. The incidence is reported to be increasing but currently ranges between 2-22/1,000,000 worldwide. […] BP predominantly affects the elderly with an average age of 80 years and is significantly associated with neurological disorders. Some cases have also been described in children and young adults.
  • #12
    https://www.nottingham.ac.uk/research/groups/cebd/projects/blistering-diseases/epidemiology-of-bullous-pemphigoid.aspx
    We aimed to improve our understanding of the people affected by bullous pemphigoid in England 1998-2017. Bullous pemphigoid most commonly affects older people, particularly older men. Over the last two decades, we found that slightly more people are being diagnosed with bullous pemphigoid each year. Those with bullous pemphigoid were more likely to die early than those without the disease. […] Understanding who is affected, how many are affected, and the consequences of bullous pemphigoid is important, but hasnt been looked at in the UK in over a decade. […] Every year, about 8 people in every 100,000 are diagnosed with bullous pemphigoid for the first time. This number has been rising slowly over the last two decades. Bullous pemphigoid more commonly affects older people, particularly older men. In 2017, about 50 in every 100,000 adults were living with bullous pemphigoid. Focusing only on older people (60 years), just over 1 in every 1,000 older person was living with bullous pemphigoid in 2017. People with bullous pemphigoid were more likely to die than similar people without the disease three times more likely in the first two years after being diagnosed, and then 50% more likely following this.
  • #13 Bullous Pemphigoid Epidemiology Forecast Report 2018-2020 & 2021-2030 – ResearchAndMarkets.com
    https://www.businesswire.com/news/home/20210415005567/en/Bullous-Pemphigoid-Epidemiology-Forecast-Report-2018-2020-2021-2030—ResearchAndMarkets.com
    The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Diagnosed Prevalent Cases of Bullous Pemphigoid, Gender-specific Diagnosed Prevalent Cases of Bullous Pemphigoid, Age-specific Diagnosed Prevalent Cases of Bullous Pemphigoid, and Severity-specific Diagnosed Prevalent Cases of Bullous Pemphigoid scenario in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan from 2018 to 2030. […] In 2020, the total diagnosed prevalent cases of bullous pemphigoid in the 7MM were 124,517 which is likely to increase by 2030 at a CAGR of 0.96% for the study period (2018-2030). […] In 2020, there were 25,041 diagnosed prevalent cases of bullous pemphigoid in the United States, these cases are expected to increase at a CAGR of 1.26% for the study period of 2018-2030.
  • #14 Bullous Pemphigoid Market is Expected to Grow With a CAGR of 16.27% for the Study Period of 2018-2030, says DelveInsight
    https://www.prnewswire.com/news-releases/bullous-pemphigoid-market-is-expected-to-grow-with-a-cagr-of-16-27-for-the-study-period-of-20182030–says-delveinsight-301247051.html
    Bullous Pemphigoid treatment market to derive enormous growth from rising geriatric population along with expected launch of emerging therapies in forecasted period (2021-2030) […] DelveInsight’s „Bullous Pemphigoid Market” report provides a thorough comprehension of the Bullous Pemphigoid, historical and forecasted epidemiology, and the Bullous Pemphigoid market trends in the 7MM [the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan]. […] According to DelveInsight’s analysts, the total diagnosed Bullous Pemphigoid prevalent cases was 124,517 in the 7MM in 2020. […] The Bullous Pemphigoid Market Report provides historical as well as forecasted epidemiological analysis segmented into: Diagnosed Prevalent cases, Gender-specific Diagnosed Prevalent Cases, Age-specific Diagnosed Prevalent Cases, Severity-specific Diagnosed Prevalent Cases.
  • #15 Epidemiology and Comorbidities of Bullous Pemphigoid: A Retrospective Study – Journal of the Turkish Academy of Dermatology
    https://jtad.org/articles/epidemiology-and-comorbidities-of-bullous-pemphigoid-a-retrospective-study/doi/jtad.galenos.2020.68552
    Bullous pemphigoid is the most common autoimmune subepidermal bullous dermatosis which is most commonly seen in the elderly population. […] The aim of this study is to determine the female-to-male ratio and the mean ages of diagnoses; disease durations and comorbidities separately in the female and male Turkish patient groups. […] For the 58 patients included in this study the female-to-male ratio was 1.42. The average age of diagnosis was 73.79 years (15-103 years). […] Bullous pemphigoid has a female predominance and is usually diagnosed during the seventh decade. […] This study showed an association between bullous pemphigoid and cardiac diseases or diabetes mellitus. […] Bullous pemphigoid is a disease of the elderly and it was previously reported that the prevalence of bullous pemphigoid in the 9th decade is 300-fold that of the 6th decade.
  • #16 Bullous pemphigoid: An immune disorder related to aging (Review)
    https://www.spandidos-publications.com/10.3892/etm.2021.10972
    Bullous pemphigoid (BP) is the most frequent subepidermal autoimmune blistering disease and is caused by autoantibodies directed against two principal antigens of the hemidesmosome, BP antigen 180 and BP antigen 230. […] The epidemiology of BP clearly shows that this is a disease of the elderly. Its triggers and comorbidities have been reviewed thus far, highlighting their association with advanced age. […] BP is the most frequent autoimmune blistering disorder, with a reported prevalence of 250 patients/million individuals and an incidence rate of 43 cases/million annually for the general population in the United Kingdom. […] Furthermore, the incidence rate of BP increases exponentially with age, reaching ~190-312 cases/million each year when considering only individuals over the age of 80.
  • #17 Bullous pemphigoid: An immune disorder related to aging (Review)
    https://www.spandidos-publications.com/10.3892/etm.2021.10972
    A 300-fold risk increase in developing BP has been estimated in patients aged over 90 compared to individuals of 60 years or younger. […] The pathology of BP results from the complex interaction of multiple mechanisms, including genetic predisposition, skin barrier changes, immunosenescence and triggering factors. The epidemiology of BP displays a predilection for elderly individuals. […] In conclusion, restoring skin barrier integrity should be a treatment goal for all patients with BP. This strategy could prove particularly beneficial in elderly individuals, in whom frequent comorbidities associated with age often limit the availability of treatment options.
  • #18 Bullous Pemphigoid: Causes, Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/15855-bullous-pemphigoid
    Bullous pemphigoid most commonly affects people over 60 years old, but it may also appear in younger people, too. Its more common in the Western world, and uncommon in the Far East. […] Bullous pemphigoid is relatively rare. There are between 2.4 and 23 cases per 1 million people in the general population. However, in people 80 years of age and older, there are between 190 and 312 cases per 1 million. […] Healthcare providers and medical researchers arent sure how to prevent bullous pemphigoid.
  • #19 Bullous Pemphigoid Epidemiology Forecast Report 2018-2020 & 2021-2030 – ResearchAndMarkets.com
    https://www.businesswire.com/news/home/20210415005567/en/Bullous-Pemphigoid-Epidemiology-Forecast-Report-2018-2020-2021-2030—ResearchAndMarkets.com
    Assessments as per this analysis showed that there were 13,772 cases of the mild-moderate form of bullous pemphigoid and 11,268 cases of the severe form of bullous pemphigoid in the United States in 2020. […] Assessments as per the analysts showed that 47% of total cases of bullous pemphigoid in the United States in 2020 were from the age group above 80 years. […] In 2020, males and females contributed to 44% and 56% of the total diagnosed prevalent cases of Bullous Pemphigoid in the United States, respectively. […] Among the European countries, the UK had the highest diagnosed prevalent population of bullous pemphigoid with 24,644 cases, followed by France which had 23,843 diagnosed prevalent cases in 2020. On the other hand, Spain had the lowest diagnosed prevalent population of 10,920 in 2020. […] In 2020, Japan accounted for 8,619 diagnosed prevalent cases of bullous pemphigoid which is the least cases among 7MM countries.
  • #20 Global Bullous Pemphigoid Market to 2025 – Insights, Epidemiology and Forecasts – ResearchAndMarkets.com
    https://www.businesswire.com/news/home/20210510005427/en/Global-Bullous-Pemphigoid-Market-to-2025—Insights-Epidemiology-and-Forecasts—ResearchAndMarkets.com
    In 2020, there were 25,041 diagnosed prevalent cases of bullous pemphigoid in the United States, these cases are expected to increase at a CAGR of 1.26% for the study period of 2018-2030. […] Assessments as per this analysis showed that there were 13,772 cases of the mild-moderate form of bullous pemphigoid and 11,268 cases of the severe form of bullous pemphigoid in the United States in 2020. […] Assessments as per the analysts showed that 47% of total cases of bullous pemphigoid in the United States in 2020 were from the age group above 80 years. […] In 2020, males and females contributed to 44% and 56% of the total diagnosed prevalent cases of Bullous Pemphigoid in the United States, respectively. […] Among the European countries, the UK had the highest diagnosed prevalent population of bullous pemphigoid with 24,644 cases, followed by France which had 23,843 diagnosed prevalent cases in 2020. On the other hand, Spain had the lowest diagnosed prevalent population of 10,920 in 2020. […] In 2020, Japan accounted for 8,619 diagnosed prevalent cases of bullous pemphigoid which is the least cases among 7MM countries.
  • #21 Epidemiology and Comorbidities of Bullous Pemphigoid: A Retrospective Study – Journal of the Turkish Academy of Dermatology
    https://jtad.org/articles/epidemiology-and-comorbidities-of-bullous-pemphigoid-a-retrospective-study/doi/jtad.galenos.2020.68552
    The average age of diagnosis was found to be 73.79 years for both genders. […] The most commonly observed comorbidities in bullous pemphigoid patients were hypertension in 26 patients (45%), diabetes mellitus in 14 patients (24%), coronary artery diseases in 6 patients (1%), chronic kidney disease in 4 patients (0.7%) and osteoporosis in 4 patients (0.7%). […] Cardiovascular diseases, including hypertension and coronary artery diseases, were reported to co-exist with bullous pemphigoid ranging from 38% up to 70% in several previous studies. […] Bullous pemphigoid is an autoimmune blistering disease with a female predominance which is usually diagnosed during the seventh decade; according to the previous literature and this study. […] Previous studies have shown that bullous pemphigoid was associated with cardiac diseases, diabetes mellitus, neurologic and psychiatric diseases. […] However, the results failed to show an association with neurologic or psychiatric diseases. […] Bullous pemphigoid was reported to be not associated with malignancies; similarly, this study did not find a significant prevalence of history of malignant neoplasms in bullous pemphigoid patients.
  • #22 Risk of Death in Bullous Pemphigoid: A Retrospective Database Study in Finland | HTML | Acta Dermato-Venereologica
    https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2347
    The most common comorbidities were cardiovascular diseases (76.3%) and neurodegenerative diseases (40.9%). […] Of these comorbidities, only malignancy predicted significantly increased 1-year mortality, the age- and sex-adjusted hazard ratio (HR) being 2.4. […] Concomitant medications used for other diseases were very common in patients with BP; the number of regularly used drugs ranging from 0 to 19. […] Interestingly, polypharmacy had a significant association with mortality: the higher the number of drugs, the greater the 1-year and 2-year mortality. […] This study found that the SMR mortality for immunologically confirmed BP was 7.6-fold that of an age-matched reference population and the 1- and 2-year mortalities were 16.7% and 30.3%, respectively. […] In general, polypharmacy in elderly patients has been shown to be an independent marker of increased mortality.
  • #23 SciELO Brazil – Bullous pemphigoid Bullous pemphigoid
    https://www.scielo.br/j/abd/a/BsschZpGNWjSKJ3rFKBCXjm/
    Both BP and neurological diseases affect elderly individuals with multiple comorbidities under the use of several medications, and epidemiological studies provided evidence that their coexistence is not coincidental. A systematic review with meta-analysis evaluated 14 studies with 23,369 BP patients and 128,697 controls. This review indicates that BP patients are 5 times more prone to develop any neurologic disorder, mainly multiple sclerosis, dementia, Parkinson’s disease, epilepsy and stroke, which usually precedes the onset of BP by 5.5 years. Multiple sclerosis has the highest association, with a 5-12 time risk of development of BP. […] A recent systematic review and meta-analysis included 25 studies and demonstrated a 1-year combined mortality rate of 23.5%. Higher mortality rates were obtained in Europe (26.7%) followed by Asia (20.5%) and US (15.1%), possibly related to an increased number of comorbidities and older age in European patients in comparison to Americans and Asians, respectively.
  • #24 Risk of Death in Bullous Pemphigoid: A Retrospective Database Study in Finland | HTML | Acta Dermato-Venereologica
    https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2347
    All studies, including ours, agree that patients with BP have increased mortality compared with the general population of the same age. […] The association between BP and neurological disorders has been established in many studies; the mechanism behind this could be an autoinflammatory reaction to BP180 and/or BP230 neuronal isoforms which are present in the human brain. […] In conclusion, patients with BP have increased mortality compared with the general population.
  • #25 Risk of Death in Bullous Pemphigoid: A Retrospective Database Study in Finland | HTML | Acta Dermato-Venereologica
    https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2347
    Bullous pemphigoid (BP) is an autoimmune skin disease of elderly people, which is associated with increased mortality. […] The aim of this study was to investigate the standardized mortality ratio (SMR) for BP in Finland, and concomitant comorbidities and medications. […] In conclusion, the mortality for BP in Finland is 7.6-fold that of a reference population, and malignancies and polypharmacy are associated with increased mortality. […] The incidence of BP is 2.5-42.8 per 1 million person-years, and it has been reported to have increased in the UK, France, USA and Finland. […] BP is associated with significant mortality; 10.8-41% within the first year after diagnosis. […] Patients with BP also have increased mortality compared with their age- and sex-adjusted counterparts in the general population; the standardized mortality ratio ranges from 1.9 to 9.6.
  • #26 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet
    https://dermnetnz.org/topics/bullous-pemphigoid
    Bullous pemphigoid often presents in people over 80 years of age, and mostly affects people over 50. […] It is more prevalent in older patients with neurological disease, particularly stroke, dementia and Parkinson disease. […] The risk of developing bullous pemphigoid is greater in people with psoriasis, and it can be precipitated by treatment of psoriasis with phototherapy. […] Bullous pemphigoid can be a serious disease, particularly when widespread or resistant to treatment. […] The Bullous Pemphigoid Disease Area Index (BPDAI) has separate scores for skin and mucous membrane activity. […] It is not known how to prevent bullous pemphigoid. […] Treatment for bullous pemphigoid is usually needed for several years.
  • #27 Cicatricial (Mucous Membrane) Pemphigoid: Background, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/1062534-overview
    The incidence of mucous membrane pemphigoid (MMP) has been estimated to be 2 cases per million in studies performed in France and Germany. […] Pure ocular mucous membrane pemphigoid occurs in less than 1 case per million in the United Kingdom. […] Among white patients in the United States, mucous membrane pemphigoid is associated with human leukocyte antigen DQB1*0301 (HLA-DQB1*0301). […] Most studies have demonstrated a female-to-male ratio of approximately 2:1. […] Most patients with mucous membrane pemphigoid are elderly, with a mean age of 62-66 years.
  • #28
    https://www.nottingham.ac.uk/research/groups/cebd/projects/blistering-diseases/epidemiology-of-bullous-pemphigoid.aspx
    This study has improved our understanding of bullous pemphigoid in England. Importantly, we have shown that bullous pemphigoid is more common in older people than what was previously thought and that it continues to be life-limiting. We hope that increased awareness of the disease and its associated mortality will improve the diagnosis and management of patients in the future.
  • #29 Bullous Pemphigoid – Market Insight, Epidemiology and Market Forecast -2032
    https://www.researchandmarkets.com/reports/5292790/bullous-pemphigoid-market-insight?srsltid=AfmBOorSLO__t7mdwC-rAdF__JfFnNPjFrzY7kUu6WEn1kndL_mrIBo2
    This „Bullous Pemphigoid- Market Insights, Epidemiology, and Market Forecast-2032” report delivers an in-depth understanding of the Bullous Pemphigoid, historical and forecasted epidemiology as well as the Bullous Pemphigoid market trends in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan. […] The Bullous Pemphigoid epidemiology division provide insights about historical and current Bullous Pemphigoid patient pool and forecasted trend for every seven major countries. It helps to recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of The report also provides the diagnosed patient pool and their trends along with assumptions undertaken. […] The disease epidemiology covered in the report provides historical as well as forecasted Bullous Pemphigoid epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2019 to 2032.
  • #30 Bullous Pemphigoid – Market Insight, Epidemiology and Market Forecast -2032
    https://www.researchandmarkets.com/reports/5292790/bullous-pemphigoid-market-insight?srsltid=AfmBOorSLO__t7mdwC-rAdF__JfFnNPjFrzY7kUu6WEn1kndL_mrIBo2
    The epidemiology segment also provides the Bullous Pemphigoid epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan. […] What is the historical Bullous Pemphigoid patient pool in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan? […] What would be the forecasted patient pool of Bullous Pemphigoid in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan? […] Out of all 7MM countries, which country would have the highest prevalent population of Bullous Pemphigoid during the forecast period (2019-2032)?
  • #31 Bullous pemphigoid – A blistering problem
    https://www.medsafe.govt.nz/profs/PUArticles/March2019/Bullous%20pemphigoid%20blistering%20problem.htm
    Bullous pemphigoid is an autoimmune blistering skin disease that mainly affects the elderly. […] Most cases of bullous pemphigoid occur in individuals aged over 60 years. […] The risk of bullous pemphigoid is increased with advanced age. […] In New Zealand, between 1965 and 2018, the Centre for Adverse Reactions Monitoring (CARM) received 20 reports of pemphigoid reactions suspected to be caused by a medicine.
  • #32 A retrospective cohort study of the epidemiology and clinical management of pemphigus in England | CPRD
    https://www.cprd.com/approved-studies/retrospective-cohort-study-epidemiology-and-clinical-management-pemphigus-england
    There are rare skin conditions called pemphigus and bullous pemphigoid that cause large and widespread blisters. These skin conditions can occur at any age but is most often seen in people when they are middle-aged or older. […] Previous UK studies, including those using data from the Clinical Practice Research Datalink (CPRD) and Hospital Episode Statistics (HES) data, show a rise in pemphigus and bullous pemphigoid incidence along with high mortality between the late 1990s and mid-2010s. […] Similarly, studies investigating bullous pemphigoid have reported incidences between 0.2 and 4.3 per 100000 person years, with higher incidence (7.6 and 8.4 per 100,000 person-years) being reported in recent years. […] While separate CPRD studies have looked at aspects of the burden and treatment of pemphigus and bullous pemphigoid, we aim to provide a complete summary of the disease epidemiology including incidence, prevalence, and mortality, along with detailed examination of treatment practices including a treatment pathway mapping and healthcare resource use for a consistent patient population using 12 years of CPRD-HES-ONS linked data.
  • #33 Top Published Expert Doctors for Bullous Pemphigoid
    https://findexpertmd.com/d/Bullous_Pemphigoid?physician=physician
    535 top medical experts on Bullous Pemphigoid across 38 countries and 30 U.S. states, including 400 MDs (Physicians). This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants. […] Clinical Trials: at least 60 including 2 Active, 22 Completed, 14 Recruiting.
  • #34 Autoimmune blistering diseases – Knowledge @ AMBOSS
    https://www.amboss.com/us/knowledge/autoimmune-blistering-diseases/
    Bullous pemphigoid is a chronic relapsing-remitting autoimmune blistering disease that primarily affects older adults. […] Peak incidence: 70 years of age. […] Most common bullous autoimmune disease. […] Oral involvement in 10-30% of patients. […] Disease extent can be assessed using the Bullous Pemphigoid Disease Activity Index or daily blister count. […] Long-term remission is possible. […] Relapse occurs in 30% of patients within 12 months of treatment and in 50% of patients after stopping treatment. […] All patients should be managed by a dermatologist. […] Consult a dermatologist for all patients. […] Patients with extensive pemphigus vulgaris typically require hospital admission.
  • #35 Bullous Pemphigoid Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034
    https://www.researchandmarkets.com/reports/5969776/bullous-pemphigoid-market-epidemiology?srsltid=AfmBOooNrG4_vuJjAF5GgypHDj9lBLuu37yzDTYcT_ivYOgQwPDioANs
    Epidemiology – Key Insights […] What is the number of prevalent cases (2018-2034) of bullous pemphigoid across the seven major markets? […] What is the size of the bullous pemphigoid patient pool (2018-2023) across the seven major markets? […] What would be the forecasted patient pool (2024-2034) across the seven major markets? […] What are the key factors driving the epidemiological trend of bullous pemphigoid? […] What will be the growth rate of patients across the seven major markets?
  • #36 Bullous Pemphigoid Market is Expected to Grow With a CAGR of 16.27% for the Study Period of 2018-2030, says DelveInsight
    https://www.prnewswire.com/news-releases/bullous-pemphigoid-market-is-expected-to-grow-with-a-cagr-of-16-27-for-the-study-period-of-20182030–says-delveinsight-301247051.html
    The rise in the geriatric population and an increase in the risk factors such as neurological disorders, use of certain drugs, and others globally contribute to increased Bullous Pemphigoid prevalence. […] DelveInsight’s Bullous Pemphigoid Epidemiology Forecast 2030 report delivers an understanding of the disease, historical forecasted epidemiology of Bullous Pemphigoid.
  • #37 Bullous Pemphigoid Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034
    https://www.researchandmarkets.com/reports/5969776/bullous-pemphigoid-market-epidemiology?srsltid=AfmBOooNrG4_vuJjAF5GgypHDj9lBLuu37yzDTYcT_ivYOgQwPDioANs
    The 7 major bullous pemphigoid markets reached a value of US$ 245.4 Million in 2023. Looking forward, the publisher expects the 7MM to reach US$ 1,379.3 Million by 2034, exhibiting a growth rate (CAGR) of 21.15% during 2023-2034. […] This report provides an exhaustive analysis of the bullous pemphigoid market in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan. This includes treatment practices, in-market, and pipeline drugs, share of individual therapies, market performance across the seven major markets, market performance of key companies and their drugs, etc. […] According to the report the United States has the largest patient pool for bullous pemphigoid and also represents the largest market for its treatment. Furthermore, the current treatment practice/algorithm, market drivers, challenges, opportunities, reimbursement scenario and unmet medical needs, etc. have also been provided in the report.
  • #38
    https://link.springer.com/article/10.1007/s44197-024-00277-7
    This study aims to conduct an extensive analysis of autoimmune bullous diseases, particularly pemphigus vulgaris and bullous pemphigoid, in Shanghai, China, from 2016 to 2023. […] The global epidemiology of autoimmune bullous disease indicates variability across populations, with some regions reporting higher incidences than others. Studies have demonstrated a greater cumulative incidence of bullous pemphigoid in Europe compared to Asia, while pemphigus, albeit less prevalent, also shows regional variation. Notably, the incidence of pemphigoid has been increasing, particularly in Western countries, over the past two decades. However, comprehensive data on the incidence and prevalence of autoimmune bullous disease in Asia, especially China, are still scarce. […] The mortality rate of 5.5% in autoimmune bullous disease, primarily due to interstitial lung disease, cardiovascular diseases, and pneumonia, is a stark reminder of the severity of these conditions. […] A notable finding is the high prevalence of autoimmune bullous disease among workers in industries requiring occupational protection. This underscores the urgent need for enhanced safety measures and increased awareness in these sectors to mitigate the risk of developing autoimmune bullous disease.