Materiały źródłowe

• #1 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV is the most common subtype of pemphigus in Europe, the United States and Japan, accounting for around 70% of pemphigus cases. The incidence of PV varies globally, ranging from 0.550 new cases per million people. Epidemiology studies in Europe indicate that PV incidence tends to be lower at higher latitudes, with incidence ranging from 0.5 cases per million Germany to 8 cases per million in Greece. PV occurs slightly more frequently in women than men. PV affects all races but is more prevalent in people of Mediterranean or Jewish ancestry. PV is typically diagnosed between the ages of 5060 years, but age of onset varies globally. […] PV is a serious condition that is associated with significant morbidity and a high risk of mortality. PV has been associated with a 3-fold increased risk of death compared with the general population and had a 1-year mortality rate of 12% (819%) in a UK population.

• #2 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  Pemphigus is an epidemiologically heterogeneous group of autoimmune bullous diseases comprising pemphigus vulgaris (PV), pemphigus foliaceus, paraneoplastic pemphigus, IgA pemphigus, and pemphigus herpetiformis. […] Recently, our knowledge about the frequency of pemphigus, which is highly variable between different populations, has considerably expanded, and the first non-HLA genes associated with PV have been identified. […] The relative distribution of PV and PF varies in different countries. Out of all patients with pemphigus, the frequency of PV ranges between 13% (in Mali) and 95% (in Saudi Arabia). […] In Europe and Northern America, 65-90% of pemphigus cases are PV, which was reviewed in Kridin (2018). […] The epidemiology of paraneoplastic pemphigus (PNP), a rare pemphigus variant accounting for about 5% of pemphigus cases, is summarized later.

• #3 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Pemphigus vulgaris accounts for 70% of all pemphigus cases worldwide although it is extremely rare in New Zealand (about one case per million of the population). […] Pemphigus vulgaris affects people of all races, age, and sex. It most commonly appears between the ages of 30 and 60 years and is more common in Jews and Indians than in other races, presumably for genetic reasons. […] About 50% of patients with pemphigus vulgaris also have anti-DSG1 antibodies. Large epidemiological studies have suggested a rare link between herpes virus infection and pemphigus. […] Pemphigus vulgaris can cause very extensive, life-threatening erosions, especially if the diagnosis is delayed. Other potentially severe complications may include: […] Diagnosis of pemphigus vulgaris generally requires a biopsy of a blister. Diagnosis of pemphigus vulgaris generally requires a biopsy from the skin adjacent to a lesion. […] The severity of pemphigus can be scored using various scoring systems. […] There is future hope that future treatment for pemphigus will be more specific with fewer side effects.

• #4 Orphanet: Pemphigus vulgaris

  https://www.orpha.net/en/disease/detail/704

  A rare autoimmune bullous skin diseases characterized by painful, flaccid blisters and erosions of the oral mucosa, predominantly involving the buccal area, and with or without extension to the epidermis. Mucosa of the larynx, oesophagus, conjunctiva, nose, genitalia and anus, are less frequently affected. […] Prevalence: 1-5 / 10 000. […] Annual incidence worldwide ranges from 1/143,000 to 1,430,000. […] The prognosis of pemphigus has markedly improved over the last decades with steroid therapy. Nevertheless, mortality remains an issue (5% of cases). In these cases, death occurs during the first years of disease progression, mostly as a consequence of treatment-related systemic infections and in a smaller proportion, as a consequence of superinfected lesions.

• #5 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. […] Despite being observed worldwide, the distribution of PV is ethnically and geographically unequal. Its incidence ranges from 0.76 new cases per million/year in Finland and 3.5 new cases per million/year in Japan to 16.1 cases per million/year in Jerusalem – worldwide, the incidence of this disease is highest in Ashkenazi Jews of Mediterranean origin. […] In Brazil, endemic foci of PV are suspected in the central-west (Brasilia, DF) and southeast regions (Ribeiro Preto, SP). Studies present conflicting data regarding the evolution of PV incidence: while in Brazil and in the United Kingdom the incidence has increased in the last decade, in Israel a reduction was observed over the last 16 years.

• #6 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  Multiple lines of evidence suggest that PV displays a heterogeneous geographic and ethnic distribution. The annual incidence rates of PV range between 0.76 cases per million in Finland and 32.0 cases per million among Jewish individuals in the United States. […] A prominent predisposition to PV was observed in certain ethnic groups, namely in individuals of Ashkenazi Jewish and Mediterranean ancestries. […] The annual incidence rate of PF in South Korea was 1.1 per million and in Northern Israel was 0.8 per million, with no evident ethnic predilection. […] Data about prevalence in pemphigus are scarce. In the Danish National Patient Registry, the prevalence of pemphigus was calculated to be 60 per million in 2006. […] Based on data from the largest German health insurance, the prevalence of pemphigus was estimated to be 148 per million individuals in 2014 and 53 per million children and/or adolescents in 2015.

• #7 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris is uncommon in the United States, and the exact incidence and prevalence depend on the population studied. […] Pemphigus vulgaris has been reported to occur worldwide. The incidence of this condition has been reported to be in the range of 0.5-3.2 cases per 100,000 population. It is higher in patients of Ashkenazi Jewish descent and those of Mediterranean origin. Few familial cases have been reported. As with endemic pemphigus, there is some evidence to suggest clustering near industrial sites. […] Although most cases of pemphigus vulgaris occur between the ages of 50 and 60 years, the range is broad, and disease onset in older individuals and in children has been described. Patients are younger at presentation in India than they are in Western countries. […] The male-to-female ratio is approximately equal. In adolescence, girls are more likely to be affected than boys.

• #8 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #9 A brief review of pemphigus vulgaris | Biomedical Dermatology | Full Text

  https://biomeddermatol.biomedcentral.com/articles/10.1186/s41702-017-0008-1

  PV has an average age of onset of 40-60 years (Joly and Litrowski 2011). It has a prevalence of around 30,000 cases in the USA and an incidence of 110 new cases per 1 million people (Pemphigus. Pemphigus Pemphigoid Foundation (IPPF) 2014). […] It is a rare diseaseespecially in the pediatric population, but it needs no less study because it does affect patients and also does affect certain groups of people more than others. Ashkenazi Jews and people from India and the Middle East have higher rates of the disease (Pisanti et al. 1974). […] It is equally distributed among genders.

• #10 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV is the most common subtype of pemphigus in Europe, the United States and Japan, accounting for around 70% of pemphigus cases. The incidence of PV varies globally, ranging from 0.550 new cases per million people. Epidemiology studies in Europe indicate that PV incidence tends to be lower at higher latitudes, with incidence ranging from 0.5 cases per million Germany to 8 cases per million in Greece. PV occurs slightly more frequently in women than men. PV affects all races but is more prevalent in people of Mediterranean or Jewish ancestry. PV is typically diagnosed between the ages of 5060 years, but age of onset varies globally. […] PV is a serious condition that is associated with significant morbidity and a high risk of mortality. PV has been associated with a 3-fold increased risk of death compared with the general population and had a 1-year mortality rate of 12% (819%) in a UK population.

• #11 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #12 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Pemphigus vulgaris (PV) is by far the most common variant of pemphigus. Even this variant is rare. UK incidence has been claimed to be rising and is estimated to be 0.68 per 100,000 person years. Incidence is higher in women and in older age groups. […] The exact prevalence and incidence depends on the population studied reflecting the immunogenetics of pemphigus: Pemphigus occurs worldwide but has a disproportionate geographic and ethnic distribution, with a significantly higher prevalence in patients of Ashkenazi Jewish or Mediterranean descent. […] PV may occur at any age, but is most commonly seen between the ages of 30 and 70 years. In adolescents, girls are more often affected than boys. […] Pemphigus, like pemphigoid, appears to be triggered by environmental factors. Such factors include: Drugs (for example, penicillamine, captopril, rifampicin). Burns. Stress. Hormones and pregnancy. Vaccinations. UV light. X-rays. Tumours. Nutritional factors. […] Removal of the trigger factor does not bring about resolution of the condition.

• #13 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #14 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #15

  https://www.ispor.org/conferences-education/conferences/past-conferences/ispor-europe-2023/program/program/session/euro2023-3784/132832

  Pemphigus diseases are life-threatening, chronic, autoimmune blistering diseases (AIBDs). This research aims to explore pemphigus vulgaris (PV) epidemiology in Italy, using real world database. […] Prevalence and Incidence were calculated on an integrated database including both in- and out-patients, geographically distributed and with demographic characteristics representative of the Italian population, covering 6 million health-assisted subjects. […] From the claims database, we identified 537 patients with an exemption code for PV. Among 28% (n=149) had at least one hospitalization discharge diagnosis for PV and 72% (n=388) had no hospitalization. […] In 2020 prevalence and incidence were respectively 22 and 2 cases per million. The incidence is quite stable over time and prevalence increases of +38%. […] In 2020, the overall incidence of Pemphigus vulgaris is estimated at 6 cases per million with a predominance in women.

• #16 Pemphigus in India – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/pemphigus-in-india/

  Epidemiology of pemphigus has shown different trend in India compared with Western literature in various counts. The incidence of pemphigus among the dermatology outpatient attendees has varied widely, 0.09 to 1.8%. […] The incidence assessed by clinic-based questionnaire survey conducted in Thrissur district, Kerala, was 4.4 per million population per year. The incidence was found to be higher than available data from Germany, France, and lower than Tunisia. […] A majority of pemphigus patients have been diagnosed to have PV, the proportion varying between 75 and 92% of total pemphigus patients. […] The types of pemphigus that followed PV in incidence are PF, PE, and PVeg, in decreasing order of frequency. […] A significant proportion of patients have been younger than 40 years of age. This is in contrast to other parts of the World where pemphigus occurs much later, 40 to 60 years being the age when pemphigus first appears.

• #17 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #18 Epidemiological study of pemphigus vulgaris in the southwest of Iran: a 15-year retrospective study and a review of literature

  https://www.iranjd.ir/article_144014.html

  Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of PV in the southwest of Iran. […] In total, 249 PV patients with a mean age of 42.72 13.3 years were included. The female to male ratio was 1.74:1. The annual incidence of PV in Fars province was 2.7 per million people. […] The findings indicate that PV is a rare disease in the southwest of Iran compared with other regions. According to previous studies in this region, the incidence has decreased since 2005.

• #19 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #20 Insights on the Global Pemphigus Vulgaris Market to 2030 – ResearchAndMarkets.com

  https://www.businesswire.com/news/home/20200917005378/en/Insights-on-the-Global-Pemphigus-Vulgaris-Market-to-2030—ResearchAndMarkets.com

  The disease epidemiology covered in The report provides historical as well as forecasted epidemiology segmented by Total Diagnosed Prevalent Population of PV, Gender-specific Diagnosed Prevalent Population of PV, Severity-specific Diagnosed Prevalent Population of PV, and Age-specific Diagnosed Prevalent Population of PV scenario of PV in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom) and Japan from 2017 to 2030. […] The increasing geriatric population and rising prevalence of PV generates a large patient population in the 7MM. In 2017, the diagnosed prevalent population of PV in the 7MM was 41,217. […] The United States contributes the largest share with 13,526 cases in 2017 acquiring near about 32.81% of the totals 7MM share. On the other hand, EU5 and Japan account for 60.10% and 7.09% diagnosed prevalent population share respectively in 2017.

• #21 Insights on the Global Pemphigus Vulgaris Market to 2030 – ResearchAndMarkets.com

  https://www.businesswire.com/news/home/20200917005378/en/Insights-on-the-Global-Pemphigus-Vulgaris-Market-to-2030—ResearchAndMarkets.com

  The disease epidemiology covered in The report provides historical as well as forecasted epidemiology segmented by Total Diagnosed Prevalent Population of PV, Gender-specific Diagnosed Prevalent Population of PV, Severity-specific Diagnosed Prevalent Population of PV, and Age-specific Diagnosed Prevalent Population of PV scenario of PV in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom) and Japan from 2017 to 2030. […] The increasing geriatric population and rising prevalence of PV generates a large patient population in the 7MM. In 2017, the diagnosed prevalent population of PV in the 7MM was 41,217. […] The United States contributes the largest share with 13,526 cases in 2017 acquiring near about 32.81% of the totals 7MM share. On the other hand, EU5 and Japan account for 60.10% and 7.09% diagnosed prevalent population share respectively in 2017.

• #22 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Pemphigus vulgaris is a relatively rare disease that only affects about 1 to 5 people in 1 million in the United Kingdom, with an incidence of 1-10 cases per 1 million people globally. […] There is an estimated prevalence of 14,000+ cases in the United States and 42,400+ in the seven major markets (US, UK, Japan, Germany, France, Italy, Spain) which is five times as prevalent as Pemphigus foliaceous. […] Cases of P. vulgaris usually don’t develop until after the age of 50 or so. […] The disease is not contagious which means it cannot be spread from person to person. […] In 2012, Israeli researchers indicated they had identified a genetic cause for the disease, which they indicated as 40 times more likely to afflict Jews compared to other demographic groups. […] The data pool is small for rare diseases.

• #23 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  Multiple lines of evidence suggest that PV displays a heterogeneous geographic and ethnic distribution. The annual incidence rates of PV range between 0.76 cases per million in Finland and 32.0 cases per million among Jewish individuals in the United States. […] A prominent predisposition to PV was observed in certain ethnic groups, namely in individuals of Ashkenazi Jewish and Mediterranean ancestries. […] The annual incidence rate of PF in South Korea was 1.1 per million and in Northern Israel was 0.8 per million, with no evident ethnic predilection. […] Data about prevalence in pemphigus are scarce. In the Danish National Patient Registry, the prevalence of pemphigus was calculated to be 60 per million in 2006. […] Based on data from the largest German health insurance, the prevalence of pemphigus was estimated to be 148 per million individuals in 2014 and 53 per million children and/or adolescents in 2015.

• #24 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  Multiple lines of evidence suggest that PV displays a heterogeneous geographic and ethnic distribution. The annual incidence rates of PV range between 0.76 cases per million in Finland and 32.0 cases per million among Jewish individuals in the United States. […] A prominent predisposition to PV was observed in certain ethnic groups, namely in individuals of Ashkenazi Jewish and Mediterranean ancestries. […] The annual incidence rate of PF in South Korea was 1.1 per million and in Northern Israel was 0.8 per million, with no evident ethnic predilection. […] Data about prevalence in pemphigus are scarce. In the Danish National Patient Registry, the prevalence of pemphigus was calculated to be 60 per million in 2006. […] Based on data from the largest German health insurance, the prevalence of pemphigus was estimated to be 148 per million individuals in 2014 and 53 per million children and/or adolescents in 2015.

• #25 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #26 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Pemphigus vulgaris (the most common form of pemphigus) occurs worldwide and the frequency is influenced by geographic location and ethnicity. Incidence rates are between 0.1 and 2.7 per 100,000 people per year. The higher rates have been documented in certain populations. People of Jewish ancestry, particularly Ashkenazi Jews, and inhabitants of India, Southeast Europe, and the Middle East have the greatest risk for pemphigus vulgaris. […] In certain locations, such as North Africa, Turkey, and South America, the prevalence of pemphigus foliaceus exceeds pemphigus vulgaris. […] Pemphigus usually occurs in adults, with an average age of onset between 40 to 60 years of age for pemphigus vulgaris and nonendemic pemphigus foliaceus. Pemphigus is rare in children, with the exception of endemic pemphigus foliaceus, which affects children and young adults in endemic areas. Neonatal pemphigus is a rare transient form of pemphigus that occurs as a consequence of placental transmission of autoantibodies to the fetus from a mother with the disease.

• #27 Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2706

  Pemphigus has an uneven geographical and ethnic distribution. […] The incidence rate of PV among Jews was 3.6-fold higher than among Arabs (p0.001), whereas no ethnic predisposition to PF was noted (p=0.379). […] The risk of death for patients with PV was almost 3-fold higher than in the general population (standardized mortality ratio (SMR) 2.6). […] For patients with PF, the risk of mortality was not significantly increased relative to the general population (SMR 1.4). […] The objectives of this study were to investigate and compare the 2 major subtypes of pemphigus regarding: (i) the incidence rate in 2 distinct ethnic populations; and (ii) mortality rates compared with the general population, by tracking an immunopathologically validated large cohort of consecutive PV and PF patients over an extended period of time.

• #28 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Pemphigus vulgaris is a relatively rare disease that only affects about 1 to 5 people in 1 million in the United Kingdom, with an incidence of 1-10 cases per 1 million people globally. […] There is an estimated prevalence of 14,000+ cases in the United States and 42,400+ in the seven major markets (US, UK, Japan, Germany, France, Italy, Spain) which is five times as prevalent as Pemphigus foliaceous. […] Cases of P. vulgaris usually don’t develop until after the age of 50 or so. […] The disease is not contagious which means it cannot be spread from person to person. […] In 2012, Israeli researchers indicated they had identified a genetic cause for the disease, which they indicated as 40 times more likely to afflict Jews compared to other demographic groups. […] The data pool is small for rare diseases.

• #29 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #30 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #31 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #32 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #33 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris affects persons of all races and ethnic groups. As noted, it is more prevalent in regions where the Jewish population is predominant and in Mediterranean regions. For example, in Jerusalem, the prevalence of pemphigus vulgaris has been estimated at 1.6 cases per 100,000 population, whereas in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population. […] The incidence in the United Kingdom is 0.68 case per 100,000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases (women, 3.9; men, 1.2) per million population per year, whereas in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women). […] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.

• #34 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #35 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris is uncommon in the United States, and the exact incidence and prevalence depend on the population studied. […] Pemphigus vulgaris has been reported to occur worldwide. The incidence of this condition has been reported to be in the range of 0.5-3.2 cases per 100,000 population. It is higher in patients of Ashkenazi Jewish descent and those of Mediterranean origin. Few familial cases have been reported. As with endemic pemphigus, there is some evidence to suggest clustering near industrial sites. […] Although most cases of pemphigus vulgaris occur between the ages of 50 and 60 years, the range is broad, and disease onset in older individuals and in children has been described. Patients are younger at presentation in India than they are in Western countries. […] The male-to-female ratio is approximately equal. In adolescence, girls are more likely to be affected than boys.

• #36 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Similarly to other autoimmune diseases, PV is more prevalent among women. The male/female ratio ranges from 1:1.5 in Israel and Iran to 1:4 in Tunisia. PV may occur at any age, and disease onset is usually between 40 and 60 years of age. An increased frequency in the elderly and children has been observed. Interestingly, in some countries of the Middle East and Brazil, disease onset is earlier: a Brazilian study estimated that 17.7% of cases occur before the age of 30 years.

• #37 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  Pemphigus vulgaris is uncommon in the United States, and the exact incidence and prevalence depend on the population studied. […] Pemphigus vulgaris has been reported to occur worldwide. The incidence of this condition has been reported to be in the range of 0.5-3.2 cases per 100,000 population. It is higher in patients of Ashkenazi Jewish descent and those of Mediterranean origin. Few familial cases have been reported. As with endemic pemphigus, there is some evidence to suggest clustering near industrial sites. […] Although most cases of pemphigus vulgaris occur between the ages of 50 and 60 years, the range is broad, and disease onset in older individuals and in children has been described. Patients are younger at presentation in India than they are in Western countries. […] The male-to-female ratio is approximately equal. In adolescence, girls are more likely to be affected than boys.

• #38 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Pemphigus vulgaris (the most common form of pemphigus) occurs worldwide and the frequency is influenced by geographic location and ethnicity. Incidence rates are between 0.1 and 2.7 per 100,000 people per year. The higher rates have been documented in certain populations. People of Jewish ancestry, particularly Ashkenazi Jews, and inhabitants of India, Southeast Europe, and the Middle East have the greatest risk for pemphigus vulgaris. […] In certain locations, such as North Africa, Turkey, and South America, the prevalence of pemphigus foliaceus exceeds pemphigus vulgaris. […] Pemphigus usually occurs in adults, with an average age of onset between 40 to 60 years of age for pemphigus vulgaris and nonendemic pemphigus foliaceus. Pemphigus is rare in children, with the exception of endemic pemphigus foliaceus, which affects children and young adults in endemic areas. Neonatal pemphigus is a rare transient form of pemphigus that occurs as a consequence of placental transmission of autoantibodies to the fetus from a mother with the disease.

• #39 Pemphigus Vulgaris – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris

  Pemphigus vulgaris usually occurs in middle-aged patients, affecting men and women in equal numbers. Rarely, cases have been reported in children. […] Pemphigus vulgaris should be suspected in patients with unexplained chronic mucosal ulceration, particularly if they have bullous skin lesions. This disorder must be differentiated from other disorders that cause chronic oral ulcers and from other bullous dermatoses (eg, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, drug eruptions, toxic epidermal necrolysis, erythema multiforme, dermatitis herpetiformis, bullous contact dermatitis). […] The diagnosis of pemphigus vulgaris is confirmed by biopsy of lesional and surrounding (perilesional) normal skin. Immunofluorescence testing shows IgG autoantibodies against the keratinocyte’s cell surface. […] About half of patients with pemphigus vulgaris have only oral lesions. […] Use Nikolsky and Asboe-Hansen signs to help clinically differentiate pemphigus vulgaris from other bullous disorders. […] Confirm the diagnosis by immunofluorescence testing of skin samples.

• #40 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  There have only been a handful of large scale (≥100 patients) epidemiologic studies of PV. Most have shown a greater female:male ratio ranging from 1.17:1 to 2.25:1. To our knowledge, this study is the largest dataset in PV published to date, and shows an increased female prevalence of 2.54:1. […] Recent studies have shown an estimated prevalence of 7.6-9.4% of the population have an autoimmune disease, and those individuals are at an increased risk of developing a second autoimmune disease. […] Overall, 15.5% of PV patients reported a co-existing autoimmune disease (mainly thyroid disease).

• #41 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #42 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Similarly to other autoimmune diseases, PV is more prevalent among women. The male/female ratio ranges from 1:1.5 in Israel and Iran to 1:4 in Tunisia. PV may occur at any age, and disease onset is usually between 40 and 60 years of age. An increased frequency in the elderly and children has been observed. Interestingly, in some countries of the Middle East and Brazil, disease onset is earlier: a Brazilian study estimated that 17.7% of cases occur before the age of 30 years.

• #43 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  There have only been a handful of large scale (≥100 patients) epidemiologic studies of PV. Most have shown a greater female:male ratio ranging from 1.17:1 to 2.25:1. To our knowledge, this study is the largest dataset in PV published to date, and shows an increased female prevalence of 2.54:1. […] Recent studies have shown an estimated prevalence of 7.6-9.4% of the population have an autoimmune disease, and those individuals are at an increased risk of developing a second autoimmune disease. […] Overall, 15.5% of PV patients reported a co-existing autoimmune disease (mainly thyroid disease).

• #44 Pemphigus in India – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/pemphigus-in-india/

  The gender predisposition has projected contrasting results. Overall, it appears that both sexes are equally affected, though. […] The studies reported from North India have reported a higher incidence of pemphigus in patients from poor socioeconomic strata. […] ABO blood group B has been found to be most commonly associated with the development of pemphigus.

• #45 Pemphigus Vulgaris

  https://fpnotebook.com/Derm/Blister/PmphgsVlgrs.htm

  Incidence: 0.5 to 3.2 cases per 100,000 (U.S.) […] Higher Incidence in Ashkenazi jews […] Mean age of onset: 40 to 60 years old […] Men and women are affected equally.

• #46 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV is the most common subtype of pemphigus in Europe, the United States and Japan, accounting for around 70% of pemphigus cases. The incidence of PV varies globally, ranging from 0.550 new cases per million people. Epidemiology studies in Europe indicate that PV incidence tends to be lower at higher latitudes, with incidence ranging from 0.5 cases per million Germany to 8 cases per million in Greece. PV occurs slightly more frequently in women than men. PV affects all races but is more prevalent in people of Mediterranean or Jewish ancestry. PV is typically diagnosed between the ages of 5060 years, but age of onset varies globally. […] PV is a serious condition that is associated with significant morbidity and a high risk of mortality. PV has been associated with a 3-fold increased risk of death compared with the general population and had a 1-year mortality rate of 12% (819%) in a UK population.

• #47 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #48 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #49 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #50 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #51 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #52 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus vulgaris and pemphigus foliaceus are the two primary subtypes of pemphigus. Of these, pemphigus vulgaris is the more common. […] A predisposing factor for pemphigus diseases is the presence of HLA gene variants such as HLA-DRB1*04:02 and HLA-DQB1*05:03, alleles that are reportedly detected in most patients with pemphigus vulgaris. […] An increased prevalence of other autoimmune diseases (eg, rheumatoid arthritis, autoimmune thyroid disease, and myasthenia gravis), psoriasis, and neurologic disease (eg, dementia, epilepsy, Parkinson disease) is found in patients with pemphigus. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases.

• #53

  https://journals.lww.com/ijdv/fulltext/2022/03000/epidemiology_of_pemphigus__a_single_center.4.aspx

  Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium. The global distribution of Pemphigus varies according to genetic, ethnic, socioeconomic, and cultural backgrounds. The average annual incidence was 0.32/100,000 inhabitants. The incidence doubled to 0.72 in 2020. The most common variant was pemphigus vulgaris (125 cases) followed by pemphigus erythematosus (99 cases), pemphigus foliaceous (40 cases), and vegetans (27 cases). In 2020, an epidemiological peak occurred during the coronavirus disease 2019 pandemic; probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2. The incidence of the disease varies from 0.5 to 34 cases/million inhabitants/year, with the highest incidence rates in Brazil. In Tunisia, the incidence is 0.67/100,000 population, in Saudi Arabia (0.16/100,000) and 0.18/100,000 in France. In North America, Europe, and Asia most cases of pemphigus are sporadic, but higher incidence were noticed in Jerusalem with an estimate of 1.6 per 100,000 populations per year. In USA, the crude prevalence of PV is 6.1/100,000 inhabitants for a total population of 29,396,600. In Rabat, which is the capital of Morocco, 10 new cases of pemphigus are diagnosed every year for a population of 577,827 inhabitants. While in Marrakech, the prevalence of 10.4 new cases per year has been noticed, and approximately 20 new cases are recorded in Egypt per year (Cairo University hospital records), 15 new cases in Jordan per year, and 10 new cases in Mauritania. The average annual incidence was estimated to be 0.32/million populations. In 2020, the incidence increased from 0.04 in 1995 to 0.72 per 1 million populations, a peak that occurs simultaneously with the coronavirus disease 2019 pandemic. The prevalence of the disease for the general population at the end of the study period was 1.56/100,000. The mean duration of the disease before diagnosis was 13.36 months. The female-to-male ratio was 0.75. The average age at diagnosis was (53 14.9) years old. In Morocco, pemphigus is associated with two HLA haplotypes (DR4-DQ3 and DR14-DQ5). The average age of onset is 48 3 years, with some variability between Universities (Rabat: 53 years, Casablanca: 47years and 53.8 in another study, Fs: 47.8 years, Marrakech: 44.4 years). In the Maghreb, the disease affects the fifth decade (eg, Tunisia, Egypt). The delayed time consultation in our country may reflect the difficulties to access medical services and health care centers, a low socio-economic level and can be related to some cultural and mystical-religious beliefs such as consultation of herborists, diviners, but also the non/mis-diagnosis by some general practitioners or other medical specialists; thus, resulting in diagnosis of moderate and severe forms. The main limitations of our study are: the one center study, the retrospective design, and the absence of dosage of the anti-desmoglein antibodies in our country. A multicenter study based on the demographic profile and genetic analysis is, therefore, necessary to understand the epidemiology of pemphigus in our country.

• #54 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Pemphigus vulgaris is a relatively rare disease that only affects about 1 to 5 people in 1 million in the United Kingdom, with an incidence of 1-10 cases per 1 million people globally. […] There is an estimated prevalence of 14,000+ cases in the United States and 42,400+ in the seven major markets (US, UK, Japan, Germany, France, Italy, Spain) which is five times as prevalent as Pemphigus foliaceous. […] Cases of P. vulgaris usually don’t develop until after the age of 50 or so. […] The disease is not contagious which means it cannot be spread from person to person. […] In 2012, Israeli researchers indicated they had identified a genetic cause for the disease, which they indicated as 40 times more likely to afflict Jews compared to other demographic groups. […] The data pool is small for rare diseases.

• #55 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  There have only been a handful of large scale (≥100 patients) epidemiologic studies of PV. Most have shown a greater female:male ratio ranging from 1.17:1 to 2.25:1. To our knowledge, this study is the largest dataset in PV published to date, and shows an increased female prevalence of 2.54:1. […] Recent studies have shown an estimated prevalence of 7.6-9.4% of the population have an autoimmune disease, and those individuals are at an increased risk of developing a second autoimmune disease. […] Overall, 15.5% of PV patients reported a co-existing autoimmune disease (mainly thyroid disease).

• #56 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #57 Pemphigus Vulgaris | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26884

  Though PV is prevalent worldwide, the occurrence of PV may be related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year. However, a higher rate is recorded in certain ethnicities. Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 and 60 years. Also, people in India, Southeast Europe, and the Middle East are at greatest risk for PV. The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women than men by a ratio of 4 to 1. […] PV has been shown to have a genetic component, although familial cases are uncommon. Patients with PV have been found to have a higher frequency of nonsymptomatic first-degree relatives with circulating PV immunoglobulin G (IgG) antibodies compared with healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles, with the latter protective against rheumatoid arthritis. PV has been associated with myasthenia gravis and ulcerative colitis.

• #58 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus vulgaris and pemphigus foliaceus are the two primary subtypes of pemphigus. Of these, pemphigus vulgaris is the more common. […] A predisposing factor for pemphigus diseases is the presence of HLA gene variants such as HLA-DRB1*04:02 and HLA-DQB1*05:03, alleles that are reportedly detected in most patients with pemphigus vulgaris. […] An increased prevalence of other autoimmune diseases (eg, rheumatoid arthritis, autoimmune thyroid disease, and myasthenia gravis), psoriasis, and neurologic disease (eg, dementia, epilepsy, Parkinson disease) is found in patients with pemphigus. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases.

• #59 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus vulgaris and pemphigus foliaceus are the two primary subtypes of pemphigus. Of these, pemphigus vulgaris is the more common. […] A predisposing factor for pemphigus diseases is the presence of HLA gene variants such as HLA-DRB1*04:02 and HLA-DQB1*05:03, alleles that are reportedly detected in most patients with pemphigus vulgaris. […] An increased prevalence of other autoimmune diseases (eg, rheumatoid arthritis, autoimmune thyroid disease, and myasthenia gravis), psoriasis, and neurologic disease (eg, dementia, epilepsy, Parkinson disease) is found in patients with pemphigus. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases.

• #60 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus vulgaris and pemphigus foliaceus are the two primary subtypes of pemphigus. Of these, pemphigus vulgaris is the more common. […] A predisposing factor for pemphigus diseases is the presence of HLA gene variants such as HLA-DRB1*04:02 and HLA-DQB1*05:03, alleles that are reportedly detected in most patients with pemphigus vulgaris. […] An increased prevalence of other autoimmune diseases (eg, rheumatoid arthritis, autoimmune thyroid disease, and myasthenia gravis), psoriasis, and neurologic disease (eg, dementia, epilepsy, Parkinson disease) is found in patients with pemphigus. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases.

• #61 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus vulgaris and pemphigus foliaceus are the two primary subtypes of pemphigus. Of these, pemphigus vulgaris is the more common. […] A predisposing factor for pemphigus diseases is the presence of HLA gene variants such as HLA-DRB1*04:02 and HLA-DQB1*05:03, alleles that are reportedly detected in most patients with pemphigus vulgaris. […] An increased prevalence of other autoimmune diseases (eg, rheumatoid arthritis, autoimmune thyroid disease, and myasthenia gravis), psoriasis, and neurologic disease (eg, dementia, epilepsy, Parkinson disease) is found in patients with pemphigus. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases.

• #62

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #63

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #64

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #65

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #66

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #67

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #68

  https://link.springer.com/article/10.1007/s10238-024-01354-8

  Background: The incidence of malignant tumors has increased in patients with non-paraneoplastic pemphigus, although there has been no systematic analysis of global epidemiology. […] Objective: To explore the epidemiology of various types of non-paraneoplastic pemphigus associated with malignant tumors. […] Results: A total of 6679 participants were included in our meta-analysis from 16 studies. The aggregated prevalence of tumors in patients diagnosed with pemphigus was 8%. The prevalence was 7% in patients with pemphigus vulgaris, 10% in those with pemphigus foliaceus, and 12% in individuals diagnosed with other types of pemphigus. The prevalence was 8% in Asia, 11% in Europe, and 8% in North America. From a country-specific perspective, patients with pemphigus from Israel, Greece, and Germany exhibited a higher prevalence of tumors at 11%. Furthermore, when categorized by the duration of the study period, the highest prevalence was observed in studies spanning 10 to 20 years, at 11%.

• #69 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV is the most common subtype of pemphigus in Europe, the United States and Japan, accounting for around 70% of pemphigus cases. The incidence of PV varies globally, ranging from 0.550 new cases per million people. Epidemiology studies in Europe indicate that PV incidence tends to be lower at higher latitudes, with incidence ranging from 0.5 cases per million Germany to 8 cases per million in Greece. PV occurs slightly more frequently in women than men. PV affects all races but is more prevalent in people of Mediterranean or Jewish ancestry. PV is typically diagnosed between the ages of 5060 years, but age of onset varies globally. […] PV is a serious condition that is associated with significant morbidity and a high risk of mortality. PV has been associated with a 3-fold increased risk of death compared with the general population and had a 1-year mortality rate of 12% (819%) in a UK population.

• #70 Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2706

  Pemphigus has an uneven geographical and ethnic distribution. […] The incidence rate of PV among Jews was 3.6-fold higher than among Arabs (p0.001), whereas no ethnic predisposition to PF was noted (p=0.379). […] The risk of death for patients with PV was almost 3-fold higher than in the general population (standardized mortality ratio (SMR) 2.6). […] For patients with PF, the risk of mortality was not significantly increased relative to the general population (SMR 1.4). […] The objectives of this study were to investigate and compare the 2 major subtypes of pemphigus regarding: (i) the incidence rate in 2 distinct ethnic populations; and (ii) mortality rates compared with the general population, by tracking an immunopathologically validated large cohort of consecutive PV and PF patients over an extended period of time.

• #71 SciELO Brazil – Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus – Brazilian Society of Dermatology Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus folia

  https://www.scielo.br/j/abd/a/k7TDXkwSmCqWRYgGnZTKfwM/

  PV is the main clinical form of pemphigus, accounting for 70% of cases. It is a rare disease, with an annual incidence that varies between 0.76 (Finland) and 16.1 (Israel) cases/million inhabitants, depending on the study population. Although it is universally occurring, PV primarily affects adults, peaking in incidence between the fourth and sixth decades of life. In Brazil, it also develops in young adults. Most studies have noted a higher incidence in women, with the female:male ratio ranging from 1.1 (Finland) to 5.0 (US). PV is considered the most severe form of pemphigus. […] Before the advent of corticosteroids and immunosuppressants, PV had a 2-year mortality rate of 50%. Since then, the mortality rate has dropped to approximately 10%. The main cause of death in patients with PV is septicemia.

• #72 SciELO Brazil – Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus – Brazilian Society of Dermatology Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus folia

  https://www.scielo.br/j/abd/a/k7TDXkwSmCqWRYgGnZTKfwM/

  PV is the main clinical form of pemphigus, accounting for 70% of cases. It is a rare disease, with an annual incidence that varies between 0.76 (Finland) and 16.1 (Israel) cases/million inhabitants, depending on the study population. Although it is universally occurring, PV primarily affects adults, peaking in incidence between the fourth and sixth decades of life. In Brazil, it also develops in young adults. Most studies have noted a higher incidence in women, with the female:male ratio ranging from 1.1 (Finland) to 5.0 (US). PV is considered the most severe form of pemphigus. […] Before the advent of corticosteroids and immunosuppressants, PV had a 2-year mortality rate of 50%. Since then, the mortality rate has dropped to approximately 10%. The main cause of death in patients with PV is septicemia.

• #73 SciELO Brazil – Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus – Brazilian Society of Dermatology Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus folia

  https://www.scielo.br/j/abd/a/k7TDXkwSmCqWRYgGnZTKfwM/

  PV is the main clinical form of pemphigus, accounting for 70% of cases. It is a rare disease, with an annual incidence that varies between 0.76 (Finland) and 16.1 (Israel) cases/million inhabitants, depending on the study population. Although it is universally occurring, PV primarily affects adults, peaking in incidence between the fourth and sixth decades of life. In Brazil, it also develops in young adults. Most studies have noted a higher incidence in women, with the female:male ratio ranging from 1.1 (Finland) to 5.0 (US). PV is considered the most severe form of pemphigus. […] Before the advent of corticosteroids and immunosuppressants, PV had a 2-year mortality rate of 50%. Since then, the mortality rate has dropped to approximately 10%. The main cause of death in patients with PV is septicemia.

• #74 Orphanet: Pemphigus vulgaris

  https://www.orpha.net/en/disease/detail/704

  A rare autoimmune bullous skin diseases characterized by painful, flaccid blisters and erosions of the oral mucosa, predominantly involving the buccal area, and with or without extension to the epidermis. Mucosa of the larynx, oesophagus, conjunctiva, nose, genitalia and anus, are less frequently affected. […] Prevalence: 1-5 / 10 000. […] Annual incidence worldwide ranges from 1/143,000 to 1,430,000. […] The prognosis of pemphigus has markedly improved over the last decades with steroid therapy. Nevertheless, mortality remains an issue (5% of cases). In these cases, death occurs during the first years of disease progression, mostly as a consequence of treatment-related systemic infections and in a smaller proportion, as a consequence of superinfected lesions.

• #75 Orphanet: Pemphigus vulgaris

  https://www.orpha.net/en/disease/detail/704

  A rare autoimmune bullous skin diseases characterized by painful, flaccid blisters and erosions of the oral mucosa, predominantly involving the buccal area, and with or without extension to the epidermis. Mucosa of the larynx, oesophagus, conjunctiva, nose, genitalia and anus, are less frequently affected. […] Prevalence: 1-5 / 10 000. […] Annual incidence worldwide ranges from 1/143,000 to 1,430,000. […] The prognosis of pemphigus has markedly improved over the last decades with steroid therapy. Nevertheless, mortality remains an issue (5% of cases). In these cases, death occurs during the first years of disease progression, mostly as a consequence of treatment-related systemic infections and in a smaller proportion, as a consequence of superinfected lesions.

• #76

  https://www.ijmedicine.com/index.php/ijam/article/view/1337

  Out of the 68 patients, 66.17% patients developed bacterial infections while 33.83% developed fungal infections. […] The prevalence of bacterial and fungal pathogens in urinary tract is 72.42% and 27.58%, pneumonitis is 29.42% and 70.58%, septicaemia is 81.82% and 18.18% and skin and soft tissue is 87.50% and 12.50% respectively. […] Mortality was seen in 7.35% patients, which was due to bacterial meningitis, septicaemia and fungal pneumonitis. […] The present study clearly states that the occurrence of the disease is uncommon but secondary infections are associated with worse prognosis.

• #77

  https://www.ijmedicine.com/index.php/ijam/article/view/1337

  Out of the 68 patients, 66.17% patients developed bacterial infections while 33.83% developed fungal infections. […] The prevalence of bacterial and fungal pathogens in urinary tract is 72.42% and 27.58%, pneumonitis is 29.42% and 70.58%, septicaemia is 81.82% and 18.18% and skin and soft tissue is 87.50% and 12.50% respectively. […] Mortality was seen in 7.35% patients, which was due to bacterial meningitis, septicaemia and fungal pneumonitis. […] The present study clearly states that the occurrence of the disease is uncommon but secondary infections are associated with worse prognosis.

• #78 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Some sources claim females are more affected by the disease, for others men and women are equally affected. […] The patient advocacy organisation NORD sees people of many different cultures and racial backgrounds similarly affected, but with a prevalence for Ashkenazi Jews, people of Mediterranean, North Indian, and Persian descent. […] There has been no found difference in the rate of disease when looking at socioeconomic factors as well. […] If left untreated, 8 of 10 people with the disease die within a year with a cause of death being infection or loss of fluids, which is very common for raw, open sores that are characteristic of P. vulgaris. […] With treatment, only about 1 in 10 people with the disease die, either from the condition, or side effects of the medicine. […] An effect of the disease being so rare is that there is not enough evidence to prove that the treatments currently being used are actually as effective as they could be.

• #79 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Some sources claim females are more affected by the disease, for others men and women are equally affected. […] The patient advocacy organisation NORD sees people of many different cultures and racial backgrounds similarly affected, but with a prevalence for Ashkenazi Jews, people of Mediterranean, North Indian, and Persian descent. […] There has been no found difference in the rate of disease when looking at socioeconomic factors as well. […] If left untreated, 8 of 10 people with the disease die within a year with a cause of death being infection or loss of fluids, which is very common for raw, open sores that are characteristic of P. vulgaris. […] With treatment, only about 1 in 10 people with the disease die, either from the condition, or side effects of the medicine. […] An effect of the disease being so rare is that there is not enough evidence to prove that the treatments currently being used are actually as effective as they could be.

• #80 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. […] Despite being observed worldwide, the distribution of PV is ethnically and geographically unequal. Its incidence ranges from 0.76 new cases per million/year in Finland and 3.5 new cases per million/year in Japan to 16.1 cases per million/year in Jerusalem – worldwide, the incidence of this disease is highest in Ashkenazi Jews of Mediterranean origin. […] In Brazil, endemic foci of PV are suspected in the central-west (Brasilia, DF) and southeast regions (Ribeiro Preto, SP). Studies present conflicting data regarding the evolution of PV incidence: while in Brazil and in the United Kingdom the incidence has increased in the last decade, in Israel a reduction was observed over the last 16 years.

• #81 Epidemiological study of pemphigus vulgaris in the southwest of Iran: a 15-year retrospective study and a review of literature

  https://www.iranjd.ir/article_144014.html

  Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of PV in the southwest of Iran. […] In total, 249 PV patients with a mean age of 42.72 13.3 years were included. The female to male ratio was 1.74:1. The annual incidence of PV in Fars province was 2.7 per million people. […] The findings indicate that PV is a rare disease in the southwest of Iran compared with other regions. According to previous studies in this region, the incidence has decreased since 2005.

• #82 SciELO Brazil – Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pe

  https://www.scielo.br/j/abd/a/NGVw9CwnBG3bf3xdrgtPxVP/?lang=en

  Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. […] Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population. […] There are no studies in Minas Gerais (southeastern region) that prove this reversal trend, nor any studies that address PV in this state; there are only epidemiological data on EPF. […] The present study suggests an increase in the number of cases of PV in the Service in recent years and is a pioneer in the state of Minas Gerais. […] The epidemiological similarities between PV and EPF cases, with some authors suggesting the use of the term endemic PV, were not identified in this study, in disagreement with recent Brazilian studies the groups maintain statistically significant differences regarding the main variables, such as age and area of residence. […] Studies that evaluate patients from reference services and specialists representing all regions of Minas Gerais are necessary to obtain knowledge about pemphigus that is closer to the state reality.

• #83

  https://www.ispor.org/conferences-education/conferences/past-conferences/ispor-europe-2023/program/program/session/euro2023-3784/132832

  Pemphigus diseases are life-threatening, chronic, autoimmune blistering diseases (AIBDs). This research aims to explore pemphigus vulgaris (PV) epidemiology in Italy, using real world database. […] Prevalence and Incidence were calculated on an integrated database including both in- and out-patients, geographically distributed and with demographic characteristics representative of the Italian population, covering 6 million health-assisted subjects. […] From the claims database, we identified 537 patients with an exemption code for PV. Among 28% (n=149) had at least one hospitalization discharge diagnosis for PV and 72% (n=388) had no hospitalization. […] In 2020 prevalence and incidence were respectively 22 and 2 cases per million. The incidence is quite stable over time and prevalence increases of +38%. […] In 2020, the overall incidence of Pemphigus vulgaris is estimated at 6 cases per million with a predominance in women.

• #84 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. […] While PV is rare (world-wide incidence is reported to be from 0.5-3.2 per 100,000 people), it can have devastating effects on those afflicted. […] Presently, the lack of large-scale clinical data is a major impediment to a better understanding of disease processes and management strategies in PV. The rare nature of PV makes the collection of epidemiologic data of a substantial number of patients difficult and time consuming. […] This report represents the first analysis of the IPPF registry data after enrollment of 599 patients, including 393 PV patients, over a 19-month period. Our data confirm results from previous epidemiologic studies in PV and reveals previously unknown differences between males and females in terms of lesion location, autoimmune comorbidity, and delay in diagnosis.

• #85 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. […] While PV is rare (world-wide incidence is reported to be from 0.5-3.2 per 100,000 people), it can have devastating effects on those afflicted. […] Presently, the lack of large-scale clinical data is a major impediment to a better understanding of disease processes and management strategies in PV. The rare nature of PV makes the collection of epidemiologic data of a substantial number of patients difficult and time consuming. […] This report represents the first analysis of the IPPF registry data after enrollment of 599 patients, including 393 PV patients, over a 19-month period. Our data confirm results from previous epidemiologic studies in PV and reveals previously unknown differences between males and females in terms of lesion location, autoimmune comorbidity, and delay in diagnosis.

• #86 Evidence gap analysis of the burden of illness and treatment of pemphigus vulgaris and foliaceus | RTI Health Solutions

  https://www.rtihs.org/publications/evidence-gap-analysis-burden-illness-and-treatment-pemphigus-vulgaris-and-foliaceus

  Epidemiology data are limited, and the true incidence and prevalence of pemphigus variants are not completely known worldwide. […] Unlike ICD-9 codes, ICD-10 codes are specific to PV and PF subtypes, but only a few epidemiology studies have used ICD-10 codes to date. […] There is a lack of studies that show the effect of treatment to prevent serious complications (e.g., opportunistic infections, cardiovascular disease), hospitalization, and death. […] Few treatment-pattern studies were found, but one study was found that included rituximab after it was approved. […] There are many gaps in the literature to support the launch of a new product in pemphigus, including epidemiology for budget-impact models, humanistic and economic burden studies, CE models, and treatment-pattern studies.

• #87 Evidence gap analysis of the burden of illness and treatment of pemphigus vulgaris and foliaceus | RTI Health Solutions

  https://www.rtihs.org/publications/evidence-gap-analysis-burden-illness-and-treatment-pemphigus-vulgaris-and-foliaceus

  Epidemiology data are limited, and the true incidence and prevalence of pemphigus variants are not completely known worldwide. […] Unlike ICD-9 codes, ICD-10 codes are specific to PV and PF subtypes, but only a few epidemiology studies have used ICD-10 codes to date. […] There is a lack of studies that show the effect of treatment to prevent serious complications (e.g., opportunistic infections, cardiovascular disease), hospitalization, and death. […] Few treatment-pattern studies were found, but one study was found that included rituximab after it was approved. […] There are many gaps in the literature to support the launch of a new product in pemphigus, including epidemiology for budget-impact models, humanistic and economic burden studies, CE models, and treatment-pattern studies.

• #88 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Some sources claim females are more affected by the disease, for others men and women are equally affected. […] The patient advocacy organisation NORD sees people of many different cultures and racial backgrounds similarly affected, but with a prevalence for Ashkenazi Jews, people of Mediterranean, North Indian, and Persian descent. […] There has been no found difference in the rate of disease when looking at socioeconomic factors as well. […] If left untreated, 8 of 10 people with the disease die within a year with a cause of death being infection or loss of fluids, which is very common for raw, open sores that are characteristic of P. vulgaris. […] With treatment, only about 1 in 10 people with the disease die, either from the condition, or side effects of the medicine. […] An effect of the disease being so rare is that there is not enough evidence to prove that the treatments currently being used are actually as effective as they could be.

• #89 Pemphigus Vulgaris in a Black Patient: Early Recognition of Disease Saves Lives

  https://www.dermatoljournal.com/articles/pemphigus-vulgaris-in-a-black-patient-early-recognition-of-disease-saves-lives.html

  Every year, PV incidence varies from 0.1-0.5 cases per 100,000 population with a higher prevalence in Europe and United States of America. […] Little is known about the prevalence or incidence of PV in dark skin tone or racial minorities. […] According to Risser et al., mortality for black persons was significantly higher for every pemphigoid bullous disease when compared to white people. […] Interestingly, PV rarely presents in black ethnicity. […] There is a well-known shortage of racial diversity in medical textbooks and literature contributing to race-based health care inequalities. […] The lack of images and evidence within literature concerning Pemphigus Vulgaris (PV) in patients with dark skin tone leads to a delay in diagnosis, thus having a negative impact on the patients management and overall prognosis.

• #90 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  In some rural areas in South America (Brazil, Northern Colombia, and Peru) and Northern Africa, PF is not only the predominant pemphigus variant but also occurs more frequently in the population. […] The burden of COVID-19 among patients with pemphigus is yet to be thoroughly investigated. […] The same recommendations advocate including patients with pemphigus with COVID-19 in the online registry initiated by the EADV task force and hosted in Groningen, The Netherlands. […] All epidemiological data about pemphigus have been retrieved from retrospective studies using the medical records of one or more specialized tertiary centers or databases constructed to store health data either by national or private institutions. […] To circumvent some of the shortcomings of the latter databases, for example, selection bias in specialized centers and the relatively poor, or at least difficult to control, ICD coding quality, in 2016, we established a prospective registry for all newly diagnosed patients with AIBD, including pemphigus, in the state of Schleswig-Holstein, Germany.

• #91 Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-1854

  Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. […] While PV is rare (world-wide incidence is reported to be from 0.5-3.2 per 100,000 people), it can have devastating effects on those afflicted. […] Presently, the lack of large-scale clinical data is a major impediment to a better understanding of disease processes and management strategies in PV. The rare nature of PV makes the collection of epidemiologic data of a substantial number of patients difficult and time consuming. […] This report represents the first analysis of the IPPF registry data after enrollment of 599 patients, including 393 PV patients, over a 19-month period. Our data confirm results from previous epidemiologic studies in PV and reveals previously unknown differences between males and females in terms of lesion location, autoimmune comorbidity, and delay in diagnosis.

• #92 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  In some rural areas in South America (Brazil, Northern Colombia, and Peru) and Northern Africa, PF is not only the predominant pemphigus variant but also occurs more frequently in the population. […] The burden of COVID-19 among patients with pemphigus is yet to be thoroughly investigated. […] The same recommendations advocate including patients with pemphigus with COVID-19 in the online registry initiated by the EADV task force and hosted in Groningen, The Netherlands. […] All epidemiological data about pemphigus have been retrieved from retrospective studies using the medical records of one or more specialized tertiary centers or databases constructed to store health data either by national or private institutions. […] To circumvent some of the shortcomings of the latter databases, for example, selection bias in specialized centers and the relatively poor, or at least difficult to control, ICD coding quality, in 2016, we established a prospective registry for all newly diagnosed patients with AIBD, including pemphigus, in the state of Schleswig-Holstein, Germany.

• #93

  https://journals.lww.com/ijdv/fulltext/2022/03000/epidemiology_of_pemphigus__a_single_center.4.aspx

  Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium. The global distribution of Pemphigus varies according to genetic, ethnic, socioeconomic, and cultural backgrounds. The average annual incidence was 0.32/100,000 inhabitants. The incidence doubled to 0.72 in 2020. The most common variant was pemphigus vulgaris (125 cases) followed by pemphigus erythematosus (99 cases), pemphigus foliaceous (40 cases), and vegetans (27 cases). In 2020, an epidemiological peak occurred during the coronavirus disease 2019 pandemic; probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2. The incidence of the disease varies from 0.5 to 34 cases/million inhabitants/year, with the highest incidence rates in Brazil. In Tunisia, the incidence is 0.67/100,000 population, in Saudi Arabia (0.16/100,000) and 0.18/100,000 in France. In North America, Europe, and Asia most cases of pemphigus are sporadic, but higher incidence were noticed in Jerusalem with an estimate of 1.6 per 100,000 populations per year. In USA, the crude prevalence of PV is 6.1/100,000 inhabitants for a total population of 29,396,600. In Rabat, which is the capital of Morocco, 10 new cases of pemphigus are diagnosed every year for a population of 577,827 inhabitants. While in Marrakech, the prevalence of 10.4 new cases per year has been noticed, and approximately 20 new cases are recorded in Egypt per year (Cairo University hospital records), 15 new cases in Jordan per year, and 10 new cases in Mauritania. The average annual incidence was estimated to be 0.32/million populations. In 2020, the incidence increased from 0.04 in 1995 to 0.72 per 1 million populations, a peak that occurs simultaneously with the coronavirus disease 2019 pandemic. The prevalence of the disease for the general population at the end of the study period was 1.56/100,000. The mean duration of the disease before diagnosis was 13.36 months. The female-to-male ratio was 0.75. The average age at diagnosis was (53 14.9) years old. In Morocco, pemphigus is associated with two HLA haplotypes (DR4-DQ3 and DR14-DQ5). The average age of onset is 48 3 years, with some variability between Universities (Rabat: 53 years, Casablanca: 47years and 53.8 in another study, Fs: 47.8 years, Marrakech: 44.4 years). In the Maghreb, the disease affects the fifth decade (eg, Tunisia, Egypt). The delayed time consultation in our country may reflect the difficulties to access medical services and health care centers, a low socio-economic level and can be related to some cultural and mystical-religious beliefs such as consultation of herborists, diviners, but also the non/mis-diagnosis by some general practitioners or other medical specialists; thus, resulting in diagnosis of moderate and severe forms. The main limitations of our study are: the one center study, the retrospective design, and the absence of dosage of the anti-desmoglein antibodies in our country. A multicenter study based on the demographic profile and genetic analysis is, therefore, necessary to understand the epidemiology of pemphigus in our country.

• #94

  https://journals.lww.com/ijdv/fulltext/2022/03000/epidemiology_of_pemphigus__a_single_center.4.aspx

  Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium. The global distribution of Pemphigus varies according to genetic, ethnic, socioeconomic, and cultural backgrounds. The average annual incidence was 0.32/100,000 inhabitants. The incidence doubled to 0.72 in 2020. The most common variant was pemphigus vulgaris (125 cases) followed by pemphigus erythematosus (99 cases), pemphigus foliaceous (40 cases), and vegetans (27 cases). In 2020, an epidemiological peak occurred during the coronavirus disease 2019 pandemic; probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2. The incidence of the disease varies from 0.5 to 34 cases/million inhabitants/year, with the highest incidence rates in Brazil. In Tunisia, the incidence is 0.67/100,000 population, in Saudi Arabia (0.16/100,000) and 0.18/100,000 in France. In North America, Europe, and Asia most cases of pemphigus are sporadic, but higher incidence were noticed in Jerusalem with an estimate of 1.6 per 100,000 populations per year. In USA, the crude prevalence of PV is 6.1/100,000 inhabitants for a total population of 29,396,600. In Rabat, which is the capital of Morocco, 10 new cases of pemphigus are diagnosed every year for a population of 577,827 inhabitants. While in Marrakech, the prevalence of 10.4 new cases per year has been noticed, and approximately 20 new cases are recorded in Egypt per year (Cairo University hospital records), 15 new cases in Jordan per year, and 10 new cases in Mauritania. The average annual incidence was estimated to be 0.32/million populations. In 2020, the incidence increased from 0.04 in 1995 to 0.72 per 1 million populations, a peak that occurs simultaneously with the coronavirus disease 2019 pandemic. The prevalence of the disease for the general population at the end of the study period was 1.56/100,000. The mean duration of the disease before diagnosis was 13.36 months. The female-to-male ratio was 0.75. The average age at diagnosis was (53 14.9) years old. In Morocco, pemphigus is associated with two HLA haplotypes (DR4-DQ3 and DR14-DQ5). The average age of onset is 48 3 years, with some variability between Universities (Rabat: 53 years, Casablanca: 47years and 53.8 in another study, Fs: 47.8 years, Marrakech: 44.4 years). In the Maghreb, the disease affects the fifth decade (eg, Tunisia, Egypt). The delayed time consultation in our country may reflect the difficulties to access medical services and health care centers, a low socio-economic level and can be related to some cultural and mystical-religious beliefs such as consultation of herborists, diviners, but also the non/mis-diagnosis by some general practitioners or other medical specialists; thus, resulting in diagnosis of moderate and severe forms. The main limitations of our study are: the one center study, the retrospective design, and the absence of dosage of the anti-desmoglein antibodies in our country. A multicenter study based on the demographic profile and genetic analysis is, therefore, necessary to understand the epidemiology of pemphigus in our country.

• #95 Epidemiology of Pemphigus

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8659392/

  In some rural areas in South America (Brazil, Northern Colombia, and Peru) and Northern Africa, PF is not only the predominant pemphigus variant but also occurs more frequently in the population. […] The burden of COVID-19 among patients with pemphigus is yet to be thoroughly investigated. […] The same recommendations advocate including patients with pemphigus with COVID-19 in the online registry initiated by the EADV task force and hosted in Groningen, The Netherlands. […] All epidemiological data about pemphigus have been retrieved from retrospective studies using the medical records of one or more specialized tertiary centers or databases constructed to store health data either by national or private institutions. […] To circumvent some of the shortcomings of the latter databases, for example, selection bias in specialized centers and the relatively poor, or at least difficult to control, ICD coding quality, in 2016, we established a prospective registry for all newly diagnosed patients with AIBD, including pemphigus, in the state of Schleswig-Holstein, Germany.

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