Materiały źródłowe

• #1 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. […] 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. […] Exposure to certain medications like penicillamine and captopril can trigger PV.

• #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. […] Scientists believe that the higher frequency of the disease in certain populations is partly due to genetics. […] In rare cases, pemphigus has resulted from taking certain medicines, such as certain antibiotics and blood pressure medications. […] Rarely, the development of a tumor in particular a growth in a lymph node, tonsil, or thymus gland can trigger the disease. […] 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. […] 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 | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pemphigus?content_id=CON-20154765

  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.

• #4 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  Pemphigus vulgaris is an autoimmune blistering disease. […] 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). […] Large epidemiological studies have suggested a rare link between herpes virus infection and pemphigus. […] 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.

• #5 Pemphigus: Types, Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/21130-pemphigus

  Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth. […] Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs. […] The exact cause of pemphigus is unknown. Research suggests that genetics and environmental factors play a role in your diagnosis. […] Pemphigus is an autoimmune condition. This means that your bodys defense system (immune system antibodies) attacks your bodys healthy cells, mistaking them for foreign invaders. […] In rare cases, certain medications, including penicillin, an antibiotic, piroxicam, a nonsteroidal anti-inflammatory drug used for rheumatoid arthritis, and blood pressure medications can cause the condition. […] Some studies found that specific HLA genes, which are genes that build your immune system, predispose you to certain types of the condition.

• #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: Causes, treatment, and images

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

  Pemphigus vulgaris is an autoimmune blistering skin condition. This means that the immune system mistakenly attacks healthy tissues in the skin. Specifically, the condition results from autoantibodies attacking proteins that help layers of skin stick together. This causes a buildup of fluid between the skin cells, which leads to the formation of blisters. […] In most cases, the autoantibodies bind to a protein known as desmoglein 3. However, roughly half of individuals who experience pemphigus vulgaris also have autoantibodies for another protein, desmoglein 1. While researchers know that this attack on desmoglein results in pemphigus vulgaris, it is still not clear why some people develop these autoantibodies. […] While the condition is not hereditary, genetic factors may play a role. Research highlights an association between pemphigus vulgaris and human leukocyte antigent (HLA) class II genes. As such, certain populations may have higher incidences of pemphigus vulgaris, including those who: […] Additionally, the following environmental factors may induce immune dysregulation, which can cause pemphigus vulgaris to flare up: […] Certain medications may also induce pemphigus vulgaris, including:

• #8 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  https://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. […] The formation of autoantibodies against components of desmosomes has long been considered the main process in pemphigus pathogenesis. […] The antigens involved in PV are desmogleins 1 (Dsg1) and 3 (Dsg3), which are 160 and 130 kDa transmembrane glycoproteins, respectively; they are an integral part of the desmosomes of the cadherin family, responsible for the intercellular adhesion of the squamous stratified epithelium. […] The basic pathophysiology of pemphigus is the inhibition of the adhesive function of desmogleins by autoantibodies, which leads to the formation of blisters. […] PV is caused by the presence of IgG autoantibodies directed against Dsg1 and/or Dsg3, part of the desmosomes present on the surface of keratinocytes, which play a primary pathogenic role in the induction of loss of intercellular adhesion, resulting in the formation of blisters.

• #9 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 (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. […] Pemphigus vulgaris should be suspected in patients with unexplained chronic mucosal ulceration, particularly if they have bullous skin lesions.

• #10 Pemphigus Vulgaris (PV) | Causes, Pathophysiology, Mechanism

  https://medically.roche.com/global/en/microsites/about-pv/pathophysiology-and-mechanisms-of-disease.html

  PV is an autoimmune disease and caused by the development of immunoglobulin G (IgG) antibodies against the extracellular domains of desmogleins, specifically desmoglein 1 (DSG1) and desmoglein 3 (DSG3). B cells play a significant pathophysiological role in PV, as antibodies are produced by B cells as part of the adaptive immune response, including the production of autoantibodies in PV (anti-DSG3 and anti-DSG1). The autoantibodies to DSG1 and DSG3 disrupt the DSG-mediated binding between epithelial cells driving the loss of cell-cell adhesions between keratinocytes, a process known as acantholysis. It is as a result of acantholysis that severe blisters develop on the skin and/or mucosal membranes. […] Anti-DSG antibodies are hypothesized to result in acantholysis through two mechanisms: (1) auto-antibody-mediated steric hindrance of desmosomal adhesion and/or interference with desmosome assembly, and (2) intracellular cell signaling pathways that augment the autoimmune response.

• #11 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. […] 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. […] Originally, the cause of this disease was unknown, and „pemphigus” was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. […] Pemphigus defines a group of autoimmune intraepithelial blistering diseases that are characterized by loss of normal cell-cell adhesion (acantholysis), and by the presence of pathogenic (predominantly IgG) autoantibodies reacting against epithelial adhesion molecules.

• #12 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.

• #13 Pemphigus vulgaris – Wikipedia

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

  Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. […] 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. […] Pemphigus is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes. […] The disease arises most often in middle-aged or older people, usually starting with a blister that ruptures easily. […] 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. […] 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.

• #14 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

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

  It has been demonstrated that the IgG4 subclass predominates in the acute phase of the disease, whereas IgG1 is associated with periods of remission. […] However, the mechanism by which the binding of autoantibodies to desmogleins leads to acantholysis remains uncertain. […] Furthermore, it is not clear what triggers pathogenic autoantibody production. It is known that there is a genetic predisposition determined by human leukocyte antigen (HLA); HLA–DRB1*04 and HLA-A*10 are more frequent in Ashkenazi Jews with pemphigus.

• #15 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.

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

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

  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. […] HLA-DRB1 0402 is associated with PV in Ashkenazi Jews; DRB1 1401/04 and DQB1 0503 HLA alleles are associated with PV in non-Jewish patients of European or Asian descent. […] 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. […] Exposure to certain medications like penicillamine and captopril can trigger PV.

• #17 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.

• #18 Pemphigus Vulgaris: Causes, Symptoms, and Treatment

  https://skinsight.com/skin-conditions/pemphigus-vulgaris/

  Pemphigus vulgaris (PV) is an autoimmune disease of the skin and mouth (mucous membrane). Autoantibodies attack molecules that essentially hold the skin cells together, causing them to separate and resulting in blisters. […] Pemphigus vulgaris can occur in persons of any ethnicity, but the disorder tends to appear more often in persons of Mediterranean ancestry, Ashkenazi Jews, and some subtypes in people from Brazil and Colombia. […] Pemphigus vulgaris is categorized as an ultra-orphan disease (meaning it is very rare), affecting approximately 10,00030,000 people in the United States. […] If pemphigus vulgaris fails to respond to corticosteroids, result in unacceptable side effects, or is extensive or progressing rapidly, your doctor may add an immunosuppressant, IVIg treatment, plasmapheresis, or biologic agents.

• #19 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. […] 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. […] 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.

• #20 Pemphigus vulgaris – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/pemphigus-vulgaris/

  Pemphigus vulgaris is a rare autoimmune skin disease and affects around 3 people per 100,000 population. […] What causes pemphigus vulgaris? […] 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. […] Although it is known that antibodies to desmoglein cause pemphigus vulgaris, it is still not clear why some people develop these autoantibodies. […] Pemphigus vulgaris does not pass from generation to generation. However, genetic factors are thought to be important in combination with other factors.

• #21 Pemphigus Vulgaris – Sussex Community Dermatology Service

  https://sussexcds.co.uk/patient-information/pemphigus-vulgaris/

  Pemphigus vulgaris is a rare autoimmune disease (up to 3.2 cases per 100,000 population) that causes severe blistering of the skin and of the mucous membranes lining the mouth, nose, throat and genitals. […] The mistake made by the immune system in pemphigus vulgaris is to view the cells in our skin and mucous membranes as foreign and to make antibodies, called auto antibodies that damage them. […] Although it is known that antibodies to desmoglein cause pemphigus vulgaris, it is still not clear why some people develop these auto antibodies in the first place. […] Strictly speaking it is not, as it does not pass from generation to generation. Genetic factors are important, but other factors are needed to trigger pemphigus vulgaris into activity.

• #22 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).

• #23 Bullous Pemphigoid & Pemphigus Vulgaris- Dermatology Advisor

  https://www.dermatologyadvisor.com/ddi/bullous-pemphigoid-pemphigus-vulgaris/

  Pemphigus vulgaris (PV) is a rare autoimmune disease with an incidence of approximately 0.42 per 100,000 people in the United States. The onset and course of pemphigus vulgaris depend on the interaction between predisposing and inducing factors. Medications, pesticides, malignancies, ultraviolet radiation, and stress have all been associated with the development of PV. […] Viral infections, especially of herpetic origin, may trigger a PV outbreak or complicate its clinical course. […] Physical agents (ultraviolet or ionizing radiation, thermal or electrical burns, surgery, and cosmetic procedures), contact allergens (organophosphate pesticides), dietary factors (foods that contain allyl compounds), and emotional stress are less commonly associated but still have been reported to cause PV.

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

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

  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.

• #25 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

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

  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.

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

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

  Pemphigus is an autoimmune bullous disease characterized by blisters and erosions of the skin and mucous membranes. Several variants of the disease exist, including pemphigus vulgaris, pemphigus foliaceous, and drug-induced pemphigus. Patients with drug-induced pemphigus have autoantibodies that are either circulating or tissue bound. […] Since the 1950s, evidence has grown that drugs may cause or exacerbate pemphigus. A drug origin should be considered in every new patient with pemphigus. The most common variant of pemphigus associated with drug exposure is pemphigus foliaceus, although pemphigus vulgaris has also been described. […] A variety of drugs have been implicated in the onset of drug-induced pemphigus. Some of these drugs induce antibody formation, which results in acantholysis via a mechanism identical to that found in idiopathic pemphigus. Other drugs are postulated to induce acantholysis directly in the absence of antibody formation.

• #27 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. […] 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.

• #28 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.

• #29 Pemphigus Vulgaris – UF Health

  https://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris

  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, pemphigus 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). […] Pemphigus vulgaris is an autoimmune disorder, where the body’s immune system attacks some of the proteins in the skin.

• #30 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).

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

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

  Such a trigger can happen through the effects on binding to molecules involved in cell adhesion, influence on enzymes that mediate keratinocyte aggregation, and by stimulating neoantigen formation. […] 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.

• #32 Pemphigus Vulgaris – MD Searchlight

  https://mdsearchlight.com/skin-problems-and-treatments/pemphigus-vulgaris/

  Pemphigus vulgaris (PV) is a type of disease where the immune system mistakenly attacks the body, resulting in blisters on the skin and the inner linings of the body. […] The exact cause of pemphigus vulgaris, a skin condition, isn’t known. However, research suggests it’s associated with our body’s own defenses (autoantibodies) mistakenly attacking proteins (cadherins) that hold skin cells together. […] Some people might have genes that make them more likely to develop pemphigus vulgaris. It’s suggested that certain types of human leukocyte antigens (HLA) proteins in our cells that help our immune system identify what’s part of our body and what’s not are linked to this condition. […] Environmental aspects such as diet, stress, viral infections, medications, sunlight exposure, radiation therapy, pesticides, or allergens might all cause immune system disruption, leading to an outbreak of pemphigus vulgaris. […] Medications like penicillamine and captopril may trigger pemphigus vulgaris as well. This could be due to their effect on molecules involved in cell binding, enzyme action that affects skin cell clustering, and the creation of new antigens (foreign bodies that trigger immune response).

• #33 Pemphigus: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/pemphigus-causes-symptoms-and-treatment?srsltid=AfmBOopuXOizDueb7kMdDcoBvnBT9tnsxefyvNhx2sI4MCBEq-V970LR

  Pemphigus is an autoimmune disorder, the exact cause of it is still unknown. […] In case of pemphigus, the body produces antibodies that attacks 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.

• #34 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.

• #35 Pemphigus Vulgaris – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/blistering-diseases/pemphigus-vulgaris

  Pemphigus vulgaris occurs when the immune system mistakenly attacks proteins in the upper layers of the skin. […] The antibodies produced by the immune system attack specific proteins that connect the epidermal cells (the cells in the top layer of skin) to each other. […] In an autoimmune disorder, the body’s immune system mistakenly attacks the body’s own tissues—in this case, the skin. […] Without treatment, pemphigus vulgaris is often fatal, usually within 5 years.

• #36 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.

• #37 About Pemphigus vulgaris (PV)

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

  PV is caused by the development of immunoglobulin G (IgG) auto-antibodies to desmogleins, desmosomal transmembrane proteins involved in maintaining the structure of the skin and mucosa. Anti-desmoglein 1 and/or anti-desmoglein 3 antibodies drive the loss of cell-cell adhesions between keratinocytes, resulting in severe blisters and erosions on the skin and/or mucous membranes. […] 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% (8-19%) in a UK population.

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

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

  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. […] Treatment of pemphigus vulgaris is aimed at decreasing production of pathogenic autoantibodies. […] One mainstay of treatment is systemic corticosteroids (1). […] 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.

• #39 Pemphigus Vulgaris (PV) Clinical Trials :: Cabaletta Bio, Inc. (CABA)

  https://www.cabalettabio.com/patients/phase-12-trial-in-pemphigus-vulgaris

  Pemphigus vulgaris (PV) is an autoimmune disease that occurs when the immune system produces antibodies that attack a protein called desmoglein (DSG). DSG normally enables skin cells and the cells lining the inside of your mouth, nose, throat, eyelids, etc. to bind tightly together. Disruption by the antibodies directed to DSG causes the painful blisters and erosions characteristic of PV. […] Although the trigger of PV is unknown, these antibodies are produced by B cells, a type of white blood cell in the immune system. […] CABA-201 is an investigational chimeric antigen receptor (CAR) T cell therapy designed to treat pemphigus vulgaris by targeting the B cells that may be causing the disease.

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