Materiały źródłowe

• #1 Pemphigus – Wikipedia

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

  Pemphigus, from 1886 medical book Microscopic image of direct immunofluorescence using an anti-IgG antibody. The tissue is skin from a patient with Pemphigus vulgaris. Note the intercellular IgG deposits in the epidermis and the early intraepidermal vesicle caused by acantholysis. Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root pemphix, meaning „blister”. In pemphigus, autoantibodies form against desmoglein, which forms the „glue” that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a large area of the skin.

• #2 What is Pemphigus? Symptoms & Causes | NIAMS

  https://www.niams.nih.gov/health-topics/pemphigus

  Pemphigus is an autoimmune disease in which the immune system mistakenly attacks cells in the top layer of the skin (epidermis) and the mucous membranes. […] Pemphigus is an autoimmune disorder that happens when the immune system attacks healthy skin. Immune molecules called antibodies target proteins called desmogleins, which help link neighboring skin cells to one another. When these connections are broken, skin becomes fragile and fluid can collect between layers of cells, forming blisters. […] Research suggests that both genetic and environmental factors can contribute to disease onset. Something in the environment may trigger pemphigus in people who are at risk because of their genetic makeup. In rare cases, pemphigus may be caused by a tumor or by certain medications.

• #3 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  Pemphigus vulgaris is a rare autoimmune disease that causes blistering on cutaneous and mucosal surfaces. […] This activity explores the historical context and etiology of PV, tracing back to its initial description in 1788, shedding light on its pathogenesis involving acantholysis, which leads to blister formation. […] The precise etiology of pemphigus vulgaris is unknown; however, multiple studies have linked pemphigus vulgaris with autoantibodies targeting cadherins, ultimately leading to acantholysis and dissociation of keratinocytes. […] Some patients at risk may have a genetic predisposition for pemphigus vulgaris. […] Several studies have linked PV with human leukocyte antigen (HLA) class II alleles. […] Environmental factors, such as diet, stress, viral infections, medications, ultraviolet radiation, ionizing radiation therapy, pesticides, and allergens, may all induce immune dysregulation leading to a flare of PV.

• #4 Pemphigus vulgaris – British Skin Foundation

  https://knowyourskin.britishskinfoundation.org.uk/condition/pemphigus-vulgaris/

  What causes pemphigus vulgaris? […] Our immune system makes antibodies to fight infection. Normally these antibodies do not attack our own body. However, in an autoimmune disease, such as pemphigus vulgaris, the immune system makes antibodies (autoantibodies) that work against tissues in the body. […] The autoantibodies in pemphigus vulgaris attack proteins called desmogleins. These proteins are present on the cells in the outer layer of skin (the epidermis) and form the glue that holds the cells of the skin together. […] When the autoantibodies formed in pemphigus vulgaris attack the desmoglein proteins, the cells in the skin and mucous membranes no longer hold together and separate. This causes blisters and erosions that are typical of pemphigus vulgaris. […] Although it is known that antibodies to desmoglein cause pemphigus vulgaris, it is still not clear why some people develop these autoantibodies.

• #5 Pemphigus vulgaris – Wikipedia

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

  Pemphigus was derived from the Greek word pemphix, meaning blister. […] It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. […] These blisters are due to acantholysis, or breaking apart of intercellular connections through an autoantibody-mediated response. […] Pemphigus is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes. […] The severity of the disease, as well as the mucosal lesions, is believed to be directly proportional to the levels of desmoglein 3. […] 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.

• #6 Orphanet: Pemphigus vulgaris

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

  The exact etiology of the disease is unknown. Autoantibodies directed against desmosome components cause acantholysis and intraepidermal cleft. In pemphigus vulgaris, autoantibodies bind to desmogleine 1 and 3 (keratinocyte membrane molecules).

• #7 Pemphigus Vulgaris Specimens – Bay Biosciences

  https://baybiosciences.com/pemphigus-vulgaris-specimens/

  Pemphigus vulgaris (PV) is an acquired autoimmune disease in which IgG antibodies target desmosomal proteins to produce intraepithelial, mucocutaneous blistering. Desmoglein (Dsg3) is the major antigen, but 50-60% of patients have additional antibodies to Dsg1, the antigen in pemphigus foliaceus (PF). The underlying antibody profile is a major determinant of the clinical phenotype of PV.

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

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

  Pemphigus vulgaris is characterized by IgG autoantibodies directed against the calcium-dependent cadherins desmoglein 3 and sometimes desmoglein 1. […] Paraneoplastic pemphigus has autoantibodies directed against these desmoglein antigens as well as others (eg, envoplakin, peiplakin, desmoplakin 1 and 2, BP-Ag 1). […] Acantholysis (loss of intercellular adhesion with consequent epidermal blister formation) results from either direct inhibition of desmoglein function by autoantibody binding or from autoantibody-induced cell signaling that results in down-regulation of cell-cell adhesion. […] The autoantibodies are present in both serum and skin during active disease. […] Treatment of pemphigus vulgaris is aimed at decreasing production of pathogenic autoantibodies. […] One mainstay of treatment is systemic corticosteroids. […] The immunosuppressants rituximab, azathioprine, and mycophenolate mofetil help reduce the need for corticosteroids and thus minimize the undesirable effects of long-term corticosteroid use. […] Without treatment, pemphigus vulgaris is often fatal, usually within 5 years of disease onset.

• #9 Pemphigus – IPPF

  https://www.pemphigus.org/pemphigus/

  Pemphigus is a group of potentially life-threatening disorders characterized by blisters in mucous membranes and skin. The blistering in pemphigus happens because of an immune response resulting in autoantibodies attacking the glue that holds mucous membranes or skin cells together. This process is called acantholysis and is not fully understood. […] Why autoantibodies attack cell adhesion is still intensively debated. Several reasons have been proposed, including events that cause cell separation and damage to adhesive molecule function. One theory suggests acantholysis results from autoantibody-caused disruption of cellular signals leading to a cells structural collapse and shrinkage. […] Autoantibodies that attack a variety of cell surface antigens have been identified in patients with pemphigus.

• #10 Pemphigus: Etiology, pathogenesis, and inducing or triggering factors: Facts and controversies | CoLab

  https://colab.ws/articles/10.1016%2Fj.clindermatol.2013.01.004

  Pemphigus includes a group of autoimmune bullous diseases with intraepithelial lesions involving the skin and Malpighian mucous membranes. […] The pathomechanism of PV hinges on autoantibodies damaging cell-cell cohesion and leading to cell-cell detachment (acantholysis) of the epidermis and Malpighian mucosae (mainly oral mucosa). […] The onset and course of PV depend on a variable interaction between predisposing and inducing factors. […] Genetic predisposition has a complex polygenic basis, involving multiple genetic loci; however, the genetic background alone („the soil”), although essential, is not by itself sufficient to initiate the autoimmune mechanism, as proven by the reports of PV in only one of two monozygotic twins and in only two of three siblings with an identical PV-prone haplotype.

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

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

  Although the cause of pemphigus vulgaris remains unknown, several potentially relevant factors have been identified, as follows. […] Predisposition to pemphigus is linked to genetic factors. Certain major histocompatibility complex (MHC) class II molecules in particular, alleles of human leukocyte antigen (HLA) DR4 (DRB1*0402) and HLA DRw6 (DQB1*0503) are common in patients with pemphigus vulgaris. […] One study found that the genes FGA (fibrinogen alpha chain), VWF (von Willebrand factor), and ACTG1 (actin gamma 1) were dysregulated in patients with pemphigus vulgaris and suggested that such dysregulation may play a role in the pathogenesis of the disease. […] Pemphigus occurs in patients with other autoimmune diseases, particularly myasthenia gravis (MG) and thymoma. […] A case-control study suggested that the following may be associated with an increased risk of developing pemphigus vulgaris: Consumption of foods containing thiol groups (eg, leeks and tomatoes), High mental stress levels, Consumption of certain supplements (eg, calcium and multivitamins), Use of chemical cleaning products that contain lime.

• #12 Pemphigus: Symptoms, Causes, Diagnosis, Treatment, and Coping

  https://www.verywellhealth.com/pemphigus-4691395

  With the exception of IgA pemphigus, the autoantibodies involved in pemphigus are immunoglobulin G (IgG). Certain types will target desmoglein 1 in superficial tissues (causing pemphigus foliaceus), while others will target desmoglein 3 in deeper tissues (causing pemphigus vulgaris. […] Genetics is believed to play a central role in the development of pemphigus. Certain genetic mutations are commonly seen in people with the disease, most of which involve the human leukocyte antigen (HLA) group of genes. HLA DR4 is the mutation most commonly seen in people with pemphigus. […] Pemphigus disproportionately affected certain ethnic groups, including Ashkenazi Jews and people of Mediterranean origin. There are even certain subtypes that occur almost exclusively in Colombian and Tunisian populations.

• #12 Pemphigus: Symptoms, Causes, Diagnosis, Treatment, and Coping

  https://www.verywellhealth.com/pemphigus-4691395

  While genetics may predispose a person to pemphigus, the actual symptoms are believed to be activated by environmental triggers, the most common of which include: Extreme emotional stress, Excessive exposure to UV radiation, including sunlight and phototherapy, Skin trauma, such as abrasions, cuts, sunburn, insect bites, and radiation therapy, Certain medications, especially penicillin, penicillamine, cephalosporin, captopril, and Vasotec (enalapril). […] Despite the long list of known triggers, the majority of cases will be idiopathic (meaning of no known origin).

• #13 Pemphigus: Who gets and causes

  https://www.aad.org/public/diseases/a-z/pemphigus-causes

  Most types of pemphigus are rare in children. Middle-aged or older people are most likely to get pemphigus, including the most common type, pemphigus vulgaris. This type usually begins between 50 and 60 years of age. […] Your risk of getting pemphigus vulgaris increases if you have: Jewish ancestry, especially Ashkenazi Jewish heritage, Mediterranean ancestry, An autoimmune disease, especially myasthenia gravis. […] The risk of getting other types of pemphigus increases if you: Take certain medicines: People who take penicillamine have a higher risk of getting pemphigus foliaceus or pemphigus vulgaris. […] People get pemphigus when their bodys immune system attacks healthy cells in their: Skin, Mouth, throat, or both, Moist tissues that lines the inside of the nose, eyelids, anus, genitals, or other areas.

• #14 Pemphigus: Etiology, pathogenesis, and inducing or triggering factors: Facts and controversies | CoLab

  https://colab.ws/articles/10.1016%2Fj.clindermatol.2013.01.004

  The intervention of inducing or triggering environmental factors („the seed”) seems to be crucial to set off the disease. […] The precipitating factors are many and various, most of them directly originating from the environment (eg, drug intake, viral infections, physical agents, contact allergens, diet), others being endogenous (eg, emotional stress, hormonal disorders) but somehow linked with the subject’s lifestyle. […] The assumption that genetic factors alone are not sufficient to cause the outbreak of the disease, inevitably instills the idea that PV may not occur spontaneously, but always results from an interaction between an individual predisposing genetic background and environmental precipitating factors, often concealed or apparently harmless.

• #15 What Triggers Pemphigus Vulgaris? Causes of Pemphigus Vulgaris

  https://www.medicinenet.com/what_triggers_pemphigus_vulgaris/article.htm

  causes of pemphigus vulgaris While what causes pemphigus vulgaris is unknown, the autoimmune disease can be triggered by environmental factors, medications, emotional stress, and infections. […] The cause of pemphigus vulgaris is not fully understood. It is an autoimmune disease, which is a disorder where the immune system attacks its own body cells and is mostly found in people aged between 50 to 60 years old. […] Experts believe that the condition is inherited from the parents, and it is triggered by some environmental factors, such as a chemical or drug. […] Other possible triggers of pemphigus vulgaris include: […] Medications […] Emotional stress […] Burns […] Exposure to sunlight (particularly the ultraviolet rays) […] Infections.

• #16 Pemphigus – IPPF

  https://www.pemphigus.org/pemphigus/

  Desmogleins are the antigens most studied in pemphigus vulgaris and pemphigus foliaceous. Desmogleins are components of desmosomes, key components for cell-to-cell adhesion. […] Like other autoimmune diseases, what causes the pemphigus diseases is not really understood. Researches believe genetic and environmental factors may influence the diseases. […] Some suggest that ultraviolet radiation could lead to pemphigus foliaceous and pemphigus vulgaris activity. Pemphigus has even developed following burns or electrical injury. Others have suggested viral infections, certain food compounds, ionizing radiation, and pesticides may trigger or worsen the disease.

• #17 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  Pemphigus vulgaris is an autoimmune blistering disease. […] Drug-induced pemphigus is also recognised and is most often caused by penicillamine, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and cephalosporins. […] Pemphigus is sometimes triggered by cancers particularly lymphomas and Castelman disease (paraneoplastic pemphigus), infection, or trauma. […] The keratinocytes are cemented together at unique sticky spots called desmosomes. In pemphigus vulgaris, immunoglobulin type G (IgG) autoantibodies bind to a protein called desmoglein 3 (DSG3), which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid (the blister). 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.

• #18 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  Exposure to certain medications like penicillamine and captopril can trigger PV. […] Furthermore, nonsteroidal antiinflammatory agents, penicillin, and cephalosporins have also been associated with drug-induced PV. […] Finally, controversial case reports associating PV with certain foods like red wine, garlic, leek, and peppers exist, though such an association is not supported by robust evidence.

• #19 Drug-induced pemphigus

  https://dermnetnz.org/topics/drug-induced-pemphigus

  Pemphigus refers to a group of autoimmune diseases that cause blistering. Pemphigus is characterised by the development of antibodies directed against a protein (either desmoglein 1, desmoglein 3 or both) involved in cell-to-cell adherence in the epidermis. […] Drug-induced pemphigus is diagnosed when a form of pemphigus has been precipitated by a medication. Pemphigus may occur days, weeks, or as long as 6 months after the drug has been commenced. […] Drugs that cause pemphigus include: Thiol drugs, including penicillamine, captopril; Antibiotics: penicillins, cephalosporins, vancomycin; Antihypertensive drugs: other angiotensin-converting enzyme inhibitors such as cilazapril, lisinopril, enalapril; Piroxicam. […] Drug-induced pemphigus is usually caused by the induction of an immune reaction to desmoglein proteins and it is identical to the idiopathic forms of pemphigus. In some cases, when no antibodies to desmoglein have been detected, a biochemical cause for the drug-induced pemphigus has been suspected.

• #20 Drug-Induced Pemphigus: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1063684-overview

  Drugs that induce pemphigus may be categorized into 2 groups: thiol drugs and nonthiol drugs. Thiol drugs are reported most frequently as the culprits of drug-induced pemphigus. […] Thiol drugs are postulated to induce acantholysis through biochemical mechanisms without antibody formation. […] Nonthiol drugs are more likely to induce acantholysis via immune mechanisms. Studies of cases of non-thiolinduced pemphigus reveal the presence of autoantibodies that recognize pemphigus antigens, in particular desmoglein 3, which is the pemphigus vulgaris antigen. […] One case report describes localized pemphigus foliaceus induced by topical imiquimod treatment. Imiquimod does not contain thiol, sulfur, or amide groups in its structure. The exact mechanism of acantholysis induction from this medication is unknown. Because imiquimod is known to cause a localized immune response at the site of application, the generation of antibodies to desmoglein 1 has been postulated as a mechanism of action.

• #21 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000882.htm

  Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes. […] The immune system produces antibodies against specific proteins in the skin and mucous membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown. […] In rare cases, PV is caused by some medicines, including: A medicine called penicillamine, which removes certain materials from the blood (chelating agent), Blood pressure medicines called ACE inhibitors, Nonsteroidal anti-inflammatory drugs (NSAIDs). […] PV is uncommon. It most often occurs in middle-aged or older people.

• #22 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Pemphigus vulgaris is an autoimmune condition that causes blisters to form on your skin and mucous membranes. […] The exact cause of pemphigus vulgaris is unknown. […] Since pemphigus vulgaris is an autoimmune condition, antibodies in your immune system, which are proteins that protect your body from foreign invaders like bacteria and toxins, target healthy cells and mistake them for foreign invaders. This attack on your healthy cells causes symptoms of blistering on your skin and mucous membranes. […] In rare cases, your body can negatively react to certain medicines that cause symptoms of pemphigus vulgaris, including: ACE inhibitors: Blood pressure medicines, Chelating agents: Medicines to remove specific materials from your blood, Antibiotics, Nonsteroidal anti-inflammatory drugs (NSAIDs). […] There isnt a cure for pemphigus vulgaris. Studies are ongoing to learn more about the cause and new treatment options to alleviate symptoms.

• #23 Pemphigus | Altru Health System

  https://www.altru.org/health-library/conditions/pemphigus

  Pemphigus is an autoimmune disorder, which means that your immune system mistakenly attacks healthy cells in your body. With pemphigus the immune system attacks cells in the skin and mucous membranes. […] Pemphigus isn’t passed from one person to another. In most cases, it’s unknown what causes the disease to arise. […] Rarely, the disease can develop as a side effect of medicines, like penicillamine and certain blood pressure drugs. This type of the condition usually clears up when the medicine is stopped.

• #24 Pemphigus: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/pemphigus-causes-symptoms-and-treatment?srsltid=AfmBOopg7fq5VV80f8BhJ_srp4qCuVe__RQeuKfibs4n1-QMjAbvTVGG

  Pemphigus is an autoimmune disorder, the exact cause of it is still unknown. […] In case of pemphigus, the body produces antibodies that attack its own cells and mucous membranes. […] In rare case scenarios, pemphigus is triggered by the use of angiotensin-converting enzyme inhibitors, penicillamine or other drugs.

• #25 Pemphigus: causes, symptoms, and treatment here – TeleMed2U

  https://www.telemed2u.com/dermatology/pemphigus

  Pemphigus is a rare autoimmune disease. […] Pemphigus produces antibodies that damage the bodys skin and mucous membranes in the mouth, throat, nose, eyelids, anus, genitals and other areas. […] It can also be caused by a reaction to taking certain medications, chiefly penicillamine. […] Your risk of getting pemphigus is higher if you: […] Have another autoimmune disease, especially myasthenia gravis. […] Pemphigus is a rare autoimmune disease. […] For an unknown reason, an autoimmune disease produces antibodies that cause the immune system to attack the body.

• #26 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. […] „Paraneoplastic autoimmune multiorgan syndrome” is an alternative term used to refer to PNP. This latter term reflects the inclusion of the nonbullous cutaneous eruptions and pulmonary involvement that may develop in the setting of PNP. […] 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.

• #27 Paraneoplastic pemphigus: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/paraneoplastic-pemphigus

  Paraneoplastic pemphigus is a rare autoimmune skin condition that causes blisters and oral lesions. It usually develops due to underlying cancer. […] The exact cause of paraneoplastic pemphigus is unclear. However, researchers believe that an underlying cancer triggers the autoimmune response in most cases. […] The condition most commonly occurs in people with B-cell lymphoproliferative disorders. It can also occur in those with: sarcoma, thymoma, carcinoma, melanoma. […] In around 84% of cases, an underlying lymphoproliferative neoplasm is present, which involves the uncontrolled production of white blood cells called lymphocytes. The most common include non-Hodgkins lymphoma and chronic lymphocytic leukemia (CLL). […] Paraneoplastic pemphigus usually develops due to an underlying malignancy. However, it can also develop due to a benign tumor.

• #28 SSA – POMS: DI 23022.635 – Paraneoplastic Pemphigus – 10/06/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022635

  Paraneoplastic Pemphigus (PNP) is a rare autoimmune disease that demonstrates many of the clinical and laboratory findings of pemphigus vulgaris. […] The binding of antibodies to the surface of the cells of the outer layer of skin (epidermis) causes pemphigus. […] While a tumor is essential in the diagnosis, people with tumors other than lymphoproliferative neoplasm can develop paraneoplastic pemphigus. These include thymoma, sarcoma, and lung carcinoma. […] Research suggests a genetic predisposition for the disease. […] The major causes of death for people with PNP are respiratory failure and infections.

• #29

  https://www.jci.org/articles/view/23475

  Autoantibodies against the epidermal desmosomal cadherins desmoglein 1 (Dsg1) and Dsg3 have been shown to cause severe to lethal skin blistering clinically defined as pemphigus foliaceus (PF) and pemphigus vulgaris (PV). […] It has been proposed that antibody-induced steric hindrance of Dsg transcellular binding (transinteraction) is the major pathogenic mechanism responsible for cellular dissociation and pemphigus development. However, other mechanisms, including antibody-induced activation of extracellular proteolysis, phosphorylation of Dsgs, and activation of protein kinase C followed by plakoglobin dislocation and subsequent depletion of Dsgs from desmosomes, appear to be important for pemphigus pathogenesis. […] Our study demonstrates for what we believe is the first time at the single molecule level that Dsg1 molecules undergo homophilic, Ca2+-dependent interaction and provides further insight into the mechanisms involved in skin blistering induced by pemphigus autoantibodies.

• #30

  https://www.jci.org/articles/view/23475

  The novel result of our study is that PF-IgGs did not inhibit Dsg1 binding by steric hindrance but rather reduced Dsg1-mediated adhesion by antibody-mediated cellular responses. […] However, since the goal of the study was not to determine the IgG epitope recognized by PF patient antibodies, we cannot exclude the possibility that IgG fractions that inhibit Dsg transinteraction may be present in other patients suffering from PF. […] These data indicate that PF-IgGs reduced Dsg1 binding by different, cell-dependent mechanisms whereas the monoclonal Dsg1 antibody seemed to directly inhibit Dsg1 adhesion. […] If cell dissociation in vitro reflects the skin blistering activity of PF-IgG, these results indicate that reduction of Dsg1-mediated adhesion by steric hindrance may not be sufficient for the pathogenesis of PF.

• #31 Mortality and Cause of Death in Patients with Pemphigus | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2611

  Mortality from pemphigus was dramatically reduced, from 75% to 30%, due to the use of corticosteroids in the early 1950s. […] The adjuvant use of immunosuppressants in the 1980s probably contributed to the further decrease in mortality from the disease itself to below 5% in study populations and 0.021 per 100,000 inhabitants in the USA. […] Infections, in particular pneumonia and septicaemia, were the most frequent causes of death in different study populations. […] Overall mortality among patients with pemphigus is 2.4-times greater than for the general population, mainly due to infections. […] Compared with the age- and gender-matched population of Israel, patients with pemphigus showed statistically significant increased likelihood of death as a consequence of infections, pneumonia, septicaemia and cardiovascular diseases.

• #32 Mortality and Cause of Death in Patients with Pemphigus | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2611

  The predominant mortality due to infections is ascribed mainly to steroidal and immunosuppressive therapies rendering patients more susceptible to infections. […] Although malignancy was a major cause of death, its SMR was not significantly higher than expected. […] Our study indicates that patients with pemphigus rarely die from the disease during the first year of diagnosis, as only 4.1% died in this time-period. […] The SMRs for infectious disease, mainly pneumonia and septicaemia, and for cardiovascular diseases were all significantly higher than expected in the age- and gender-matched population. […] This study contributes an epidemiological figure of all-cause and cause-specific mortalities compared with the general population, tracking cases with very high level of immunopathological validation.

• #33 Pemphigus: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/pemphigus-treatment

  Some medicines can cause or worsen pemphigus […] Blood test: This test can tell whether you have proteins in your blood that cause pemphigus. […] Plasma contains the proteins that cause your immune system to attack your skin and the moist tissues lining your mouth and other parts of your body. […] Researchers are looking for better treatment options […] One area of research shows promise. At the University of Pennsylvania School of Medicine, scientists have found a way to successfully remove the cells that cause pemphigus vulgaris. […] Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type. Thanks to medicines and other treatments, this has changed. Few people die of pemphigus.

• #34 The facts about Pemphigus.

  https://www.gbhealthwatch.com/pemphigus-details.php

  Pemphigus is a group of rare autoimmune diseases. Such diseases occur when the bodys immune system attacks healthy cells. […] The cause of this immune system attack is not known. Pemphigus does not spread from person to person. It does not appear to be passed from parent to child. But some peoples genes put them more at risk for pemphigus. […] Current research includes study of: Risk factors, Antibodies that attack skin cells, Better treatments with drugs that have fewer side effects.

• #35 Pemphigus and Pemphigoid > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/pemphigus-and-pemphigoid

  These conditions are caused by an immune system gone awry and manifest as blisters in the skin or membranes such as the mouth, nose and eyes, says Yale Medicine dermatologist Mary M. Tomayko, MD, PhD. […] People who get these diseases are genetically predisposed, says Dr. Tomayko. They could have a difference in their immune responses that make them a little more prone to getting these diseases. Exposures throughout their lives and age-related changes in the immune system could also put them at a higher risk, though we dont know the triggers. In short, its a combination of genes and environment that were still getting a handle on. […] Bullous pemphigoid is more common in people with pre-existing neurological diseases such as Parkinsons disease or dementia. The link between these two kinds of disorders is an active area of research here at Yale Medicine, Dr. Tomayko says. Our working hypothesis is that because people who have Parkinsons and dementia have elevated levels of antibodies against collagen proteins, their immune systems are primed to attack the skin.

• #36 Pemphigus: Who gets and causes

  https://www.aad.org/public/diseases/a-z/pemphigus-causes

  The body attacks these healthy cells because it mistakes them for something harmful like viruses or bacteria. […] Scientists are still trying to find out why the body does this. We know that pemphigus is NOT contagious, so its not something you catch that causes these attacks. […] Finding what causes pemphigus could lead to safer and more effective treatments.

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