Materiały źródłowe

• #1 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 much higher incidence of PF than of PV in well-defined regions in South America and North Africa has inspired the term endemic PF.

• #2 Epidemiology of Pemphigus – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34909708/

  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. […] Here, initial data about the impact of COVID-19 on this fragile patient population are discussed and perspectives for future epidemiological studies are outlined.

• #3

  https://link.springer.com/article/10.1007/s12026-018-8986-7

  Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. […] The epidemiological features of pemphigus vary considerably in different regions of the world. […] Observational studies examining comorbidities and associations among patients with pemphigus are scarce and sometimes inconclusive. […] This review provides a brief overview about the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, pemphigus herpetiformis, and IgA pemphigus. […] In addition, it summarizes the most recent understanding of the epidemiology, mortality data, and comorbidities of this group of organ-specific autoimmune diseases. […] Remarkable differences in the epidemiology of pemphigus among two ethnic populations in the same geographic region. […] Incidence of pemphigus vulgaris exceeds that of pemphigus foliaceus in a region where pemphigus foliaceus is endemic: analysis of a 21-year historical series.

• #3 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 advent of corticosteroids in the early 1950s revolutionized the prognosis of pemphigus and led to a drastic decline in the mortality of patients, particularly those with PV, from 75% to 30%. […] Despite the aforementioned decline throughout the years, the mortality of patients with pemphigus nowadays remains excessive as compared with the general population. […] The burden of COVID-19 among patients with pemphigus is yet to be thoroughly investigated. […] 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.

• #4 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.

• #5 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

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

  In most countries, PV is more frequent than PF – in France, for example, PV accounts for 73% of cases of pemphigus, and in Japan the ratio between PV and PF is 2:1. […] 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. […] 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. […] 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.

• #6 Epidemiology of Pemphigus

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

  The epidemiology of paraneoplastic pemphigus (PNP), a rare pemphigus variant accounting for about 5% of pemphigus cases, is summarized later. […] 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. […] 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 | 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.

• #8 Pemphigus Vulgaris Market Size and Forecast 2024-2034

  https://www.imarcgroup.com/pemphigus-vulgaris-market

  The reported incidence of pemphigus vulgaris is between 0.1 and 0.5 per 100,000 people every year. […] Pemphigus vulgaris is more common among Ashkenazi Jews, with the average onset occurring between the ages of 40 and 60. […] Also, within the demographics, persons residing in India, Southeast Europeans, and the Middle Eastern are at the highest risk for pemphigus vulgaris. […] Pemphigus Vulgaris is more frequent in women than men by a ratio of 4:1. […] Pemphigus Vulgaris has a mortality rate of around 5-15%. […] IMARC Group’s new report provides an exhaustive analysis of the pemphigus vulgaris market in the United States, EU4 (Germany, Spain, Italy, and France), United Kingdom, and Japan. This includes treatment practices, in-market, and pipeline drugs, share of individual therapies, market performance across the seven major markets, market performance of key companies and their drugs, etc.

• #9 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.

• #10

  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. […] 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.

• #11 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.

• #12 P/P Clinical Information – IPPF

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

  A few studies have found large imbalances in the sex distribution, such as a study that found a 4:1 ratio of females to males with pemphigus foliaceus in Tunisia and a study that found a 19:1 ratio of males to females in an endemic location in Columbia. […] Epidemiological information on IgA pemphigus is sparse. The disorder may occur at any age and may be slightly more common in females. Paraneoplastic pemphigus is extremely rare and is more common in middle-aged adults, but may occur in children.

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

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

  Little is known about differences in epidemiological features and prognosis between pemphigus vulgaris (PV) and pemphigus foliaceus (PF). […] The incidence rate of PV among Jews was 3.6-fold higher than among Arabs (p?0.001), whereas no ethnic predisposition to PF was noted (p?=?0.379). […] There is a racial predisposition to PV, whereas PF is sporadic. Mortality among patients with PV is higher compared with PF and the general population.

• #14 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus refers to a group of uncommon and severe autoimmune blistering skin diseases that affect the epithelium, including skin and mucous membranes. […] Because pemphigus is rare and has features that mimic other more common disorders, diagnosis can be challenging and often delayed. […] Given the overlapping clinical presentations among the various epithelial antibody-associated immunobullous cutaneous diseases, broad serologic screening is recommended unless a specific disease type is suspected and/or supported by characteristic findings. […] 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.

• #15 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Pemphigus epidemiology1 6 […] 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.7 […] 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. […] HLA-DRB1*0402 is associated with the disease in Ashkenazi Jews and DRB1*1401/04 and DQB1*0503 in non-Jewish patients of European or Asian descent.4 […] 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. […] The majority of affected children and adolescents have mucocutaneous disease.8 […] Pemphigus, like pemphigoid, appears to be triggered by environmental factors. Such factors include: […] Removal of the trigger factor does not bring about resolution of the condition.

• #16 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.

• #17 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. […] 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. […] Pemphigus is confirmed by direct immunofluorescence staining of perilesional skin biopsy sections to reveal immunoglobulin (Ig)G antibodies or complement on the cell surfaces of keratinocytes.

• #18 A retrospective cohort study of the epidemiology and clinical management of pemphigus in England | CPRD

  https://www.cprd.com/approved-studies/retrospective-cohort-study-epidemiology-and-clinical-management-pemphigus-england

  There are rare skin conditions called pemphigus and bullous pemphigoid that cause large and widespread blisters. […] Previous UK studies, including those using data from the Clinical Practice Research Datalink (CPRD) and Hospital Episode Statistics (HES) data, show a rise in pemphigus and bullous pemphigoid incidence along with high mortality between the late 1990s and mid-2010s. Pemphigus vulgaris (PV) is the most common form of pemphigus, with annual incidence in the UK previously estimated to be 0.7 per 100,000 person-years. […] While separate CPRD studies have looked at aspects of the burden and treatment of pemphigus and bullous pemphigoid, we aim to provide a complete summary of the disease epidemiology including incidence, prevalence, and mortality, along with detailed examination of treatment practices including a treatment pathway mapping and healthcare resource use for a consistent patient population using 12 years of CPRD-HES-ONS linked data. […] Health Outcomes to be Measured: Incidence, prevalence and mortality; Treatment provision including immunosuppressive and immunomodulatory therapies; Treatment pathway including order of treatment given; Routine high-dose steroid; Hospital activity.

• #19 Large-scale global retrospective study on the interaction between ancestry and risk of comorbid autoimmune diseases in patients with pemphigus | Scientific Reports

  https://www.nature.com/articles/s41598-024-78031-z

  The pemphigus family of skin blistering diseases represents a rare yet potentially life-threatening condition characterized by multiple known genetic loci associated with other autoimmune disorders. […] While several studies have empirically indicated an increased risk of developing additional autoimmune diseases in individuals with pemphigus, the scarcity of data and the rarity of pemphigus have hindered efforts to establish and generalize these associations across diverse populations. […] Our findings reveal highly significant and generalizable associations between pemphigus and pemphigoid diseases, discoid lupus erythematosus, lichen planus, and undifferentiated connective tissue disease, among others. […] A nationwide case-control study conducted in Taiwan identified an elevated risk of developing Sjgrens syndrome, systemic lupus erythematosus, and psoriasis.

• #20 Large-scale global retrospective study on the interaction between ancestry and risk of comorbid autoimmune diseases in patients with pemphigus | Scientific Reports

  https://www.nature.com/articles/s41598-024-78031-z

  A review of pemphigus epidemiology corroborated these findings and highlighted a novel association with psoriasis and ulcerative colitis. […] In this study, we aim to investigate several autoimmune diseases suspected of being clinically associated with pemphigus and examine their interaction with patient ancestry. […] As a result, we quantify the overall likelihood of developing a comorbidity after pemphigus for 74 separate autoimmune diseases and find that the pemphigoid diseases, psoriasis, rheumatoid arthritis and Sjgren syndrome are the most likely to develop. […] The most consistently significantly comorbid diseases across ethnicities are the pemphigoid diseases, discoid lupus erythematosus, lichen planus, and undifferentiated connective tissue disease. […] Our findings hold significant relevance for clinical practice, underscoring the necessity of screening for comorbidities to ensure comprehensive care for pemphigus patients. […] The investigation thoroughly examined the risk of secondary autoimmune diseases among pemphigus patients across diverse racial and ethnic groups. […] The results consistently demonstrated robust associations between pemphigus and other autoimmune conditions, notably pemphigoid.

• #21

  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%.

• #22

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

  Conclusion: These findings demonstrate the incidence and prevalence of malignant tumors in patients with non-paraneoplastic pemphigus, which may achieve early detection and intervention, and then reduce mortality rates. […] This study scrutinized the global epidemiology of pemphigus and tumors. Overall, 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%. Therefore, it is necessary to strengthen the screening of tumors in patients with pemphigus, especially for those with a disease course of more than 10 years.

• #23 About Pemphigus vulgaris (PV)

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

  PV is a chronic disease that if left untreated can quickly progress to severe disease with multiple erosions that are susceptible to infections and dehydration. Secondary infections occurring in PV patients may lead to sepsis and cardiac failure and PV is, therefore, considered to be a life-threatening disease. PV patients are reported to have a 3-times higher risk of mortality than the general population.

• #24 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.

• #25 Editor’s Pick: Pemphigus Vulgaris and Pemphigus Foliaceus: A Single-Centre Comparative Study in Rabat, Morocco – European Medical Journal

  https://www.emjreviews.com/dermatology/article/pemphigus-vulgaris-and-pemphigus-foliaceus-a-single-centre-comparative-study-in-rabat-morocco/

  Pemphigus is a group of rare autoimmune blistering diseases affecting the skin and mucous membranes. […] The geographical distribution of pemphigus is unequal. The annual incidence of PV ranges from 16.1 /million in Jerusalem to 0.76 /million in Finland. […] In a recent Moroccan study, PV was the most reported form (50.3%), with fewer cases of PF (28.8%). […] While few studies have been interested in comparing the two major variants of pemphigus, the authors work focused on analysing their clinical course. […] The most interesting findings were the absence of significant differences concerning demographic patterns, such as age and gender. […] The mean duration of the disease before treatment was long: 13 months, as reported by the Tunisian study. […] The authors report showed that PV needed higher doses of corticosteroids to control the disease, and more cases of complications and mortality have been reported in this group as documented by Goon and Tan, and an Israeli study.

• #26

  https://www.scielo.br/j/abd/a/M6GwT4pRNH7xDySBrKFCPPC/?lang=en

  Patients’ distribution according to age and gender differs from country to country. […] The time elapsed from onset of symptoms and diagnosis was less than 1 month in 20% of PV and PF patients, and between 1 month and 1 year in 75% of patients with both forms of the disease. […] Although many regimens have been implemented to treat pemphigus, none of them has shown to provide absolute efficacy in controlling the disease. […] All cases of death from our sample were related, among other comorbidities, to sepsis, which evolved to some of its complicated forms (septic shock, severe sepsis, and organ failure). […] Currently, it is difficult to understand the real incidence of mortality for pemphigus because of the small number of studies involving a large sample of patients, the heterogeneous number and size of cohorts, and because it is not always possible to properly distinguish mortality for complications caused by PV from those caused by PF.

• #27 Paraneoplastic pemphigus – UpToDate

  https://www.uptodate.com/contents/paraneoplastic-pemphigus

  Paraneoplastic pemphigus (PNP) is an often fatal paraneoplastic mucocutaneous blistering disease that is most commonly induced by lymphoproliferative disorders. […] Although it is well accepted that PNP is a rare disorder, the incidence and prevalence of PNP are unknown. The rarity of the disorder is supported by a review of 100,000 adverse events reports to the US Food and Drug Administration from patients with non-Hodgkin lymphoma and chronic lymphocytic leukemia. The review identified only 12 patients who likely had the disease. […] Adults between the ages of 45 and 70 years constitute the most common demographic affected by PNP. However, the disorder may also occur in children. Both males and females may be affected. […] The frequency of PNP may be decreasing. In one academic medical center in the United States, the number of patients diagnosed with PNP fell from 22 between 2003 and 2010 to only 4 between 2011 and 2017. Increased use of anti-CD20 monoclonal antibody therapy (eg, rituximab) for non-Hodgkin lymphoma and chronic lymphocytic leukemia may be a contributor. Theories include reduced recognition of PNP due to treatment-related alterations in the clinical presentation of PNP (suppression of B cell-mediated blistering) and a true reduction in incidence of PNP related to the early institution of effective treatment for malignancies responsive to this treatment.

• #28 Orphanet: Paraneoplastic pemphigus

  https://www.orpha.net/en/disease/detail/63455

  The prevalence of this form of pemphigus is unknown. About 500 cases of paraneoplastic pemphigus have been reported worldwide in the literature. This form accounts for 3-5% of all pemphigus cases. […] Paraneoplastic pemphigus occurs in a background of suspected or proven neoplasia. […] The progression of paraneoplastic pemphigus rarely parallels neoplastic progression. […] Paraneoplastic pemphigus is often fatal (in 90% of cases), however, the prognosis depends on the nature of the underlying malignancy and is improved when the associated tumor is benign.

• #29 Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-paraneoplastic-pemphigus-a-life-threatening-autoimmune-articulo-S0001731017303708

  PNP may also develop after the tumor has been treated. […] The most commonly associated tumors are hematological, accounting for nearly 84% of all cases, these include non-Hodgkin’s lymphoma (38.6%), chronic lymphocytic leukemia (18.4%), Castleman’s disease (18.4%), thymoma (5.5%), Waldenstrm macroglobulinemia (1.2%), Hodgkin’s lymphoma (0.6%) and monoclonal gammopathy (0.6%). […] In children and adolescents, PNP is most commonly associated with Castleman’s disease and PNP is often the presenting sign of Castleman’s disease.

• #30

  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 purpose of our study is to evaluate the epidemiological features of pemphigus a single center in Morocco and compare our results with those reported elsewhere. […] The average annual incidence was 0.32/100,000 inhabitants. The incidence doubled to 0.72 in 2020. […] 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.

• #31

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

  In Rabat, which is the capital of Morocco, 10 new cases of pemphigus are diagnosed every year for a population of 577,827 inhabitants. […] The aim of our study was to describe the epidemiological and clinical characteristics of the disease, which provides epidemiological reference to the disease prevention and control. […] The mean duration of the disease before diagnosis was 13.36 months. […] 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 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.

• #32 Editor’s Pick: Pemphigus Vulgaris and Pemphigus Foliaceus: A Single-Centre Comparative Study in Rabat, Morocco – European Medical Journal

  https://www.emjreviews.com/dermatology/article/pemphigus-vulgaris-and-pemphigus-foliaceus-a-single-centre-comparative-study-in-rabat-morocco/

  This investigation, therefore, arises some uncertainty concerning the mildness of PF, as its prognosis may depend on geographic areas, genetic background, and environmental factors. […] Prospective, multicentre trials based on demographic profile, genetic analysis, and prognostic factors are, therefore, needed to enhance the knowledge of this pathology in Morocco, to facilitate patient stratification, and to design personalised therapeutic plans in the hospital setting.

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