Materiały źródłowe

• #1 Pemphigus – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409

  Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. If your symptoms were caused by use of certain medicines, stopping that medicine may be enough to clear up your symptoms. […] Your healthcare professional may suggest one or more of the following medicines. The choice of medicines depends on the type of pemphigus you have, how severe your symptoms are and whether you have other medical conditions. […] For people with mild disease, corticosteroid cream or injections may be enough to control it. For others, the main treatment is a corticosteroid medicine taken by mouth, such as prednisone pills. […] Using corticosteroids for a long time or in high doses may cause severe side effects. These include diabetes, bone loss, an increased risk of infection, stomach ulcers and a shift of body fat.

• #1 Pemphigus – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409

  To avoid these side effects, steroids may be used only for short periods of time to control flare-ups. And other medicines that target the immune system may be used long term to control the disease. […] Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide. […] If first line medicines aren’t helping you, your healthcare professional may suggest another drug, such as dapsone, intravenous immunoglobulin or rituximab-pvvr (Ruxience). […] Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time.

• #2 Pemphigus: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pemphigus/diagnosis-treatment-and-steps-to-take

  There is no cure for pemphigus, but treatment can control the disease in most people. The initial goal of treatment is to clear existing blisters and help prevent relapses. Treatment typically depends on the severity and stage of the disease. […] Symptoms of pemphigus may go away after many years of treatment, but most people need to continue taking medications to keep the disease under control. Treatment for pemphigus may involve the following medications: […] Corticosteroids. These anti-inflammatory medicines are a mainstay of treatment for pemphigus. They may be applied topically as a cream or ointment, or by mouth or injection (systemically). Most people will be prescribed systemic corticosteroids, at least initially, to bring the disease under control. Because they are potent drugs, your doctor will prescribe the lowest dose possible to achieve the desired benefit.

• #2 Pemphigus: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pemphigus/diagnosis-treatment-and-steps-to-take

  Biologic response modifiers (or biologics). These target specific immune messages and interrupt the signal, helping to stop the immune system from attacking the skin. Rituximab is an approved biologic administered directly in the vein. It targets and depletes the immune cells that ultimately make the disease-causing antibodies. […] Antibiotics, antivirals, and antifungal medications to control or prevent infections. […] If the above treatments do not work or are not tolerated, other treatments may be considered. These treatments include: […] Immunosuppressants. Although less effective than rituximab, these are oral medications that help suppress or curb the overactive immune system and may help to lower the dose of daily steroid. […] Plasmapheresis or immunoadsorption. These are procedures that remove or dilute out damaging antibodies from the blood. […] Intravenous immunoglobulin therapy. This is an intravenous infusion of pooled antibodies from 1,000 or more healthy blood donors, which dilute out the bad antibodies and calm inflammation. […] Be sure to report any problems or side effects from medications to your doctor.

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

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

  Treatment is unique to each person diagnosed with pemphigus and could include: […] Taking medicine to prevent infections and help your skin heal. […] Stopping the use of medicines that cause your symptoms. […] Wound care for blisters and sores. […] Your healthcare provider will treat your condition in stages. Most people go through all three stages of treatment, which include: […] Control: High doses of medications control the spread of blisters and begin healing existing ones. […] Consolidation: Steady doses of medications continue healing blisters until most clear up. […] Maintenance: Reduced levels of medications keep new blisters from forming. […] Medicines used to treat pemphigus include: […] Corticosteroids: Medication to reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (ointments or creams).

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

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

  Immunosuppressive drugs: Drugs that manage your bodys autoimmune response, which is what happens when your body attacks healthy cells. […] Rituximab: A monoclonal antibody that targets problematic B cells. […] Intravenous immunoglobulin: Healthy antibodies (proteins made by your immune system to attack foreign substances), given through a needle into your vein, to help reduce the antibodies that cause your diagnosis. […] Antibiotics: In some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe antibiotic medicine to treat the infection. […] Side effects vary based on the type of treatment and could include: […] Chest pain. […] Dizziness. […] Headache. […] Joint pain. […] Nausea. […] Upset stomach. […] Weight gain. […] Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus.

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

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

  Treatment for pemphigus takes time before you see results. With treatment, youll notice new blisters stop forming after several weeks and your skin will begin to heal. It could take months for your blisters and sores to heal completely. […] No, there isnt a cure for pemphigus. Treatment is effective to alleviate symptoms.

• #4 Initial management of pemphigus vulgaris and pemphigus foliaceus – UpToDate

  https://www.uptodate.com/contents/initial-management-of-pemphigus-vulgaris-and-pemphigus-foliaceus

  Initial management of pemphigus vulgaris and pemphigus foliaceus […] Systemic glucocorticoids and rituximab are the mainstays of therapy for pemphigus vulgaris and pemphigus foliaceus and are usually highly effective for obtaining control of disease. […] Other immunomodulatory agents, such as azathioprine and mycophenolate mofetil, are commonly prescribed in conjunction with systemic glucocorticoids in an attempt to minimize the risk for adverse effects of long-term, high-dose glucocorticoid therapy. […] Interventions such as intravenous immune globulin (IVIG), immunoadsorption, and cyclophosphamide are typically reserved for patients with refractory disease. […] The initial management of pemphigus vulgaris and pemphigus foliaceus will be reviewed here.

• #5 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Systemic corticosteroids are the mainstay of medical treatment for controlling the disease, usually in the form of moderate to high doses of oral prednisone or prednisolone, or as pulsed intravenous methylprednisolone. Since their use, many deaths from pemphigus vulgaris have been prevented (the mortality rate dropped from 99% to 515%). Corticosteroids are not a cure for the disease but improve the patient’s quality of life by reducing disease activity. The doses of corticosteroids needed to control pemphigus vulgaris and the length of time on treatment may result in serious side effects and risks. […] Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. These are most often: Azathioprine, Mycophenolate mofetil, Cyclophosphamide, Rituximab, which is now approved by the Food and Drug Administration (FDA) in the US.

• #5 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  Other medications that are sometimes used in pemphigus (often in combination) include: Dapsone, Methotrexate, Tetracyclines, Nicotinamide, Plasmapheresis, Intravenous immunoglobulin, Extracorporeal photopheresis, Immunoadsorption, The TNF inhibitor, infliximab. […] Topical therapy for cutaneous pemphigus vulgaris may include topical steroids and emollients. Treatment of mucosal pemphigus vulgaris may include various formulations of a topical steroid, intralesional steroid, topical tacrolimus, or topical ciclosporin. […] Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. […] Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. These include activities such as contact sports and eating or drinking food that may irritate or damage the inside of the mouth (spicy, acidic, hard and crunchy foods).

• #5 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  There is future hope that future treatment for pemphigus will be more specific with fewer side effects. The CD20 antagonist ofatumumab has been reported to be effective. Investigators have engineered specific chimeric auto-antibody receptor T-cells to eliminate desmoglein-3-specific B cells in mice. The use of anti-neonatal Fc receptor is under investigation in combination with rituximab.

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

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

  The treatment of autoimmune bullous dermatoses, including PV is usually based on systemic medications, because they comprise a severe group of mucosal and cutaneous diseases with significant morbidity and mortality. Treatment should be started as early as possible, and its goal is to achieve and maintain disease remission. […] Systemic corticosteroids are the basis of the treatment for PV. They have potent anti-inflammatory and immunosuppressive activities. The introduction of these drugs in the 1950s was followed by a reduction in mortality from 75% to 30%. […] Corticosteroids act rapidly in PV, effecting improvement in several days and impeding the emergence of new lesions after 2 to 3 weeks. […] To minimize the side effects, morbidity, and mortality of PV, contrary to what was advocated several decades ago, it is recommended that the daily dosage of prednisone does not exceed 1.5mg/kg/day-above this value, the likelihood of skin infection and evolution to septicemia (the main death cause in these patients) increases progressively. Thus, other drugs are recommended, in association with corticosteroids-termed adjuvant drugs (corticosteroid-sparing agents).

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

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

  When the condition is not controlled solely with corticosteroids or when the patient has clinical contraindications to high-dose corticosteroids (e.g., hypertension, diabetes mellitus, glaucoma, osteoporosis-all of which are frequent in the age group in which the prevalence of PV peaks), other drugs, called adjuvants or corticosteroid-sparing agents, should be incorporated. […] Azathioprine is a cytotoxic drug that is used in most autoimmune diseases. […] The efficacy of AZA as a corticosteroid-sparing agent in autoimmune bullous diseases, particularly in PV, is well documented and is the oldest and most prescribed immunosuppressive medication in this context. […] Mycophenolate mofetil (MMF) has been used as an adjuvant to corticosteroids in patients with PV as first-line treatment and in non-responders to AZA.

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

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

  Chimeric anti-CD20 monoclonal antibody (which depletes normal and pathogenic B lymphocytes) has been used for cases of severe and refractory PV since 2006. […] Rituximab is generally well tolerated, and serious adverse effects are rare. […] Cyclophosphamide is an alkylating agent that selectively affects B lymphocytes and antibody production. […] Methotrexate can be added as an adjuvant in PV at 10 to 20mg/ week in cases of therapeutic failure to other adjuvants. […] Drug with anti-inflammatory and anti-TNF activity that can be attempted as adjuvant medication in PV at 50 to 200mg/day orally, but there are conflicting reports in the literature. […] Plasmapheresis was first used in 1978 for PV to remove pathogenic autoantibodies from circulation. […] Topical treatment of PV lesions aims to reduce pain and prevent secondary infections.

• #7 Pemphigus: Current and Future Therapeutic Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6603181/

  In pemphigus, prednisolone is recommended as a first-line therapy in combination with an immunosuppressive agent, such as azathioprine (AZA) and mycophenolate mofetil (MMF), or RTX. In addition, prednisolone alone at a dose of 1-1.5 mg/kg/day is still recommended as first line therapy in patients who are not eligible for treatment with RTX or other immunosuppressive adjuvants. Higher CS doses (up to 1.5 mg/kg) may be administered, if disease control is not achieved within 3 weeks. As soon as disease control is reached, the doses should be tapered by a 25% reduction every other week. If lesions reappear, CS should be increased until two steps back at the previous dose to lead to disease control. However, the optimal dose has not been validated by randomized clinical trials. […] A recent prospective multicentre study by Joly et al. supports using RTX as a first line adjuvant therapy for pemphigus, showing superior efficacy compared to CS alone and reduced incidence of CS-related serious adverse events and overall mortality. The administration of intravenous immunoglobulin (IVIg) or immunoadsorption (IA) is a therapeutic option in patients with severe/refractory PV.

• #7 Pemphigus: Current and Future Therapeutic Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6603181/

  Since the advent of targeted therapies, the management of pemphigus has gradually changed. Until now, systemic corticosteroids (CS) and immunosuppressants have been the mainstay of pemphigus therapy. Among conventional adjuvant immunosuppressants, both EADV and BAD guidelines suggest azathioprine (AZA) and mycophenolate mofetil (MMF) as a first line steroid-sparing agent. However, different variables, including patients’ comorbidities, single institutional experience and costs have to be taken into account, and other drugs, such as methotrexate and cyclophosphamide, also demonstrate efficacy. Notably, these drugs have mainly a CS-sparing rather than a morbostatic effect. Accordingly, they do not lead to an improvement in achieving remission, but reduce the risk of relapse by 29% in comparison to CS alone. A recent prospective multicentre study by Joly et al. now supports using RTX as a first line adjuvant therapy for pemphigus, showing superior efficacy compared to CS alone and reduced incidence of CS-related serious adverse events and overall mortality. The administration of intravenous immunoglobulin (IVIg) or immunoadsorption (IA) is a therapeutic option in patients with severe/refractory PV. Proposed algorithms for the induction and maintenance therapy as well as therapy of relapse are summarized in Figures 5 and 7.

• #7 Pemphigus: Current and Future Therapeutic Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6603181/

  Rituximab is a highly effective therapy in pemphigus. By depleting B-cells, RTX leads to marked decrease of circulating anti-Dsg autoantibodies, and, since the pathogenic role of such autoantibodies, significant amelioration of the lesions. Multiple lines of evidence, however, suggest that RTX exerts a deep modulation of both humoral and acquired immune function in pemphigus, explaining the fact that, in many cases, disease amelioration lasts longer than B-cell re-appearance in the peripheral blood of the patients. […] Rituximab was initially shown to be effective for pemphigus patients resistant to standard immunosuppressive therapies. In one study in 2006, Ahmed et al. reported complete clinical remission, allowing successful tapering of immunosuppressive therapies, in 9 out of 11 patients with refractory pemphigus following a protocol combining IVIg and 10 RTX infusions over a 6 months period. After 10-year follow-up most patients were shown to maintain clinical remission. In 2007, in a larger clinical series, Joly et al. reported durable clinical response with significant corticosteroid sparing effects in 86% of 21 patients with refractory pemphigus following a single cycle of RTX.

• #7 Pemphigus: Current and Future Therapeutic Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6603181/

  In summary, while RTX was initially recommended as a third line therapy in patients without adequate disease control with standard immunosuppressants, several studies have definitively demonstrated that patients may benefit from early RTX treatment, in terms of both clinical efficacy and safety, leading current guidelines to recommend it as the gold standard for new onset pemphigus.

• #8 Emerging treatment options for the management of pemphigus vulgaris | TCRM

  https://www.dovepress.com/emerging-treatment-options-for-the-management-of-pemphigus-vulgaris-peer-reviewed-fulltext-article-TCRM

  The aim of treatment in PV is to induce and maintain remission, which clinically corresponds to the cessation of new vesicle formation, healing of old erosions, and the completion of treatment tapering to maintenance doses. […] The guidelines of the EDF and BAD define systemic corticosteroids as the first-line treatment of PV, recommending initial treatment with predniso(lo)ne at a dose of 0.51.5 mg/kg/day (EDF) and 1.0 mg/kg/day (BAD). […] Immunosuppressive adjuvants can be administered in combination with systemic corticosteroids. According to the EDF guidelines, the combination of predniso(lo)ne with azathioprine or mycophenolate mofetil (MMF) is considered as second-line treatment, whereas the combination of predniso(lo)ne with rituximab, intravenous immunoglobulin (IVIg), immunoadsorption, cyclophosphamide, dapsone, or methotrexate is considered as third-line treatment in PV.

• #8 Emerging treatment options for the management of pemphigus vulgaris | TCRM

  https://www.dovepress.com/emerging-treatment-options-for-the-management-of-pemphigus-vulgaris-peer-reviewed-fulltext-article-TCRM

  Dapsone is recommended in combination with systemic corticosteroids at a dose of 100 mg/day (or 1.5 mg/kg/day) as a second-line steroid-sparing agent. […] Methotrexate at a dosage of 1020 mg/week is currently under consideration as a second-line steroid-sparing option in PV according to the EDF guidelines. […] Cyclophosphamide is considered a second-line immunosuppressive adjuvant agent, whereas the BAD guidelines consider cyclophosphamide as a third-line therapy. […] IVIg is usually administered in recalcitrant disease or in case of contraindications to immunosuppressive adjuvants, and is recommended as a second-line adjuvant by the EDF guidelines and as a third-line therapy by the BAD guidelines.

• #9 Treatments – IPPF

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

  Potential side effects may include headaches, nausea, stomach aches, high blood pressure, stroke, emotional difficulties or mood swings, and weight gain. […] IVIG is considered to be safe, and the majority of people tolerate it without problems. The adverse reactions occur only in less than 1% of patients. […] Patients with pemphigus and pemphigoid who suffer from side effects of steroid therapy have a higher risk of adverse reactions. […] Infusion reactions often occur within the first 24 hours after first rituximab infusion. […] Azathioprine, Mycophenolate, and Cyclophosphamide are immunosuppressants used in the treatment of pemphigus.

• #9 Treatments – IPPF

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

  Mycophenolate (CellCept, Myfortic) is composed of several penicillium species that is used after initial treatment for pemphigus. […] Cyclophosphamide (Cytoxan) is an oral cyclophosphamide that is considered an alternative to azathioprine. Due to the potential toxicities, this drug should be reserved for patients who do not respond to other immunosuppressives. […] Rituximab is a B-cell antibody treatment option for patients with pemphigus that is being used as first line therapy by many clinicians. In June 2018, the FDA approved Rituxan for the treatment of adults with moderate to severe PV. […] Intravenous Immunoglobulin (IVIG) therapy is prepared from extracting the plasma in human blood. IVIG is given intravenously; under the skin via a syringe or catheter. The dosage required is patient-specific. To treat pemphigus, the doses are as high as 2000mg/kg.

• #9 Treatments – IPPF

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

  Anti-inflammatory agents such as dapsone and tetracyclines are used as they also may have a steroid sparing effect in mild to moderate disease, often in patients who are in maintenance phase but corticosteroid-dependent. […] Dapsone must be started after glucose-6-P-dehydrogenase screening and is administered as 7.5mg/kg/day, up to 200mg/day. […] Tetracycline, doxycycline and minocycline have been used by some in glucocorticoid-dependent patients in the maintenance phase of therapy, often with niacinamide (nicotinamide). […] For multiple oral erosions, corticosteroid mouthwashes are practical, for example, soluble betamethasone sodium phosphate 0.5 mg tablet dissolved in 10 mL water may be used up to four times daily, holding the solution in the mouth for about 5 min. […] It is important that all physicians, doctors, and specialists involved with a treatment are in contact with one another to avoid conflicting medications and to be sure that each doctors treatments are working in harmony.

• #9 Treatments – IPPF

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

  The IPPF does not endorse any drugs, treatments, or products. Information is provided for informational purposes only. Because the symptoms and severity of pemphigus and pemphigoid vary among individuals, we recommend all drugs and treatments be discussed with the readers physician(s) for proper evaluation, treatment, and care. […] Initial therapy is determined by the extent and rate of the progression of lesions. The priority is to control lesions. Usually in a slow progressive form of the disease, initial treatment includes intralesional injections of corticosteroids or topical application of corticosteroids. […] Once most lesions have healed, the dose and type of medication are gradually reduced to limit the risk of side effects. Understanding the rate of dose reduction is determined by clinical response and overall disease activity. It is important to monitor this balance and limit use of un-necessary medication as many fatalities are related to complications with therapy.

• #9 Treatments – IPPF

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

  Corticosteroids mimic the effect of the adrenal hormones your body naturally produces. Systemic corticosteroids are the most established therapy for the management of PV. In most cases, when used in high doses, they can rapidly control disease. The most common corticosteroids include Prednisone and Prednisolone. […] Once controlled, steroid use is slowly reduced to minimize side effects. Some patients then go into remission; however, many need a small maintenance dose to keep the disease under control. […] Topical steroids can be used for the treatment of pemphigus. To address oral erosion, steroid mouthwash, paste, ointment or aerosol can be used. Topical cyclosporine can also be used for the treatment of oral pemphigus lesions. […] Azathioprine (Imuran, Azasan) is used after initial treatment to manage pemphigus.

• #10 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet

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

  The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Treatment with corticosteroids has improved the overall mortality, but significant morbidity remains. Much of the mortality and morbidity in patients with pemphigus vulgaris is now related to the adverse effects of such therapy. […] Immunosuppressive drugs are steroid-sparing and should be considered early in the course of the disease. Mycophenolate mofetil and azathioprine have been the agents usually considered as initial choices.

• #10 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet

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

  Cyclophosphamide is used for refractory disease. […] Plasmapheresis has been used in refractory cases, usually in conjunction with cytotoxic therapy. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions.

• #10 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet

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

  A retrospective chart review suggested a therapeutic ladder for patients with pemphigus vulgaris, in which a combination of mycophenolate mofetil and prednisone was given initially, followed by rituximab if this initial regimen was unsuccessful. […] Rituximab is approved by the US Food and Drug Administration (FDA) for moderate-to-severe pemphigus vulgaris. It has been employed as first- or second-line therapy. Evidence suggests that outcomes are better when rituximab is given as a first-line agent, and it is increasingly being used in this way. […] The antitumor necrosis factor (TNF) drugs sulfasalazine and pentoxifylline have been reported to be effective adjunctive treatments, reducing the serum level of TNF and yielding rapid clinical improvement. […] Intravenous immunoglobulin (IVIG) therapy has been suggested as efficacious in pemphigus vulgaris treatment.

• #11 Pemphigus: Diagnosis and treatment

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

  If you are diagnosed with pemphigus, you’ll likely need treatment. Without treatment, pemphigus tends to worsen. In some people, pemphigus can become life-threatening without treatment. […] Pemphigus cannot be cured, but with treatment, most people can control their pemphigus. […] Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening. […] A treatment plan for pemphigus may include one or more of the following: […] A corticosteroid can clear the blisters and sores. […] Either azathioprine or mycophenalate mofetil is often used to treat pemphigus. These can stop the body from creating new blisters. […] One biologic, rituximab, seems promising. It appears to offer safe treatment. […] Taking both a corticosteroid like prednisone, and an immunosuppressant medication like azathioprine to quiet the immune system, may deliver better results.

• #11 Pemphigus: Diagnosis and treatment

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

  When pemphigus is severe or medicine fails to work, a dermatologist may talk with you about one of the following treatment options: […] This treatment involves removing plasma from your blood. […] This treatment usually takes two days, but you can go home each day after treatment. […] While in the hospital, treatment can also be given to help get pemphigus under control. […] To give patients safer and more effective treatment options, researchers continue to study pemphigus. […] This could lead to better treatment for pemphigus vulgaris. […] For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while. […] Thanks to medicines and other treatments, this has changed. Few people die of pemphigus. […] These problems can be managed with help from your dermatologist and other doctors.

• #12 Treatment of pemphigus – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/treatment-of-pemphigus/

  Systemic corticosteroids remain the mainstay of therapy for pemphigus. Their use has transformed what was almost invariably a fatal illness into one whose mortality is now below 10%. […] Treatment regimens are dictated by the age of the patient, the degree of involvement, the rate of disease progression and the subtype of pemphigus. Most patients who die of pemphigus at present die of complications of therapy. This can be prevented to an extend by the use of various adjuvant therapies mostly immunosuppressives which can reduce the need for steroids. […] The rationale to use drugs other than corticosteroids to treat pemphigus is that they may reduce the need for corticosteroids and hence their side effects and may result in better control of the disease. This includes immunosuppressive drugs like cyclophosphamide, azathioprine, cyclosporine, methotrexate and recently mycophenolate mofetil.

• #12 Treatment of pemphigus – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/treatment-of-pemphigus/

  Immunosuppressives are usually started after prednisolone has been tapered to 40mg daily. […] Dexamethasone-Cyclophosphamide pulse therapy has been used recently for pemphigus unresponsive to high doses of oral corticosteroids. […] Mycophenolate mofetil has recently been used 2 g daily with prednisolone 2 mg/kg daily with good success. […] Other adjuvant treatment in pemphigus includes immuno modulatory procedures like plasmapheresis and extracorporeal photopheresis. […] At present plasmapheresis appears to be worthwhile in patients with severe pemphigus unresponsive to conventional therapy.

• #13 Insights into Pathophysiology and Treatment of Pemphigus | AAFP

  https://www.aafp.org/pubs/afp/issues/2000/0201/p820.html

  Pemphigus occurs in three major types, all characterized by blistering and ulceration caused by the action of autoantibodies against adhesion molecules that anchor keratinocytes to one another and to the underlying basement membrane. […] Treatment is aimed at reducing the synthesis of these autoantibodies. Combination systemic therapy with corticosteroids and immunosuppressive agents is usually required, although the iatrogenic effects of these potent medications may contribute to morbidity. Prednisone alone in dosages up to 1 mg per kg may control some cases. If the dosage cannot be tapered to 5 to 10 mg on alternate days within a year of treatment, azathioprine or cyclophosphamide must be added. In fulminant cases, plasmapheresis has been used to remove autoantibodies from the circulation. […] The same therapeutic agents are used, but treatment is usually less aggressive and the prognosis better than in patients with pemphigus vulgaris. […] Combination therapy with steroids and cyclosporine may alleviate symptoms.

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. […] Treatment is with corticosteroids and sometimes other immunosuppressive therapies. […] Referral to a dermatologist with expertise in treating this disorder is recommended. Hospitalization is required initially for all but the most minor cases. […] Treatment of pemphigus vulgaris is aimed at decreasing production of pathogenic autoantibodies. One mainstay of treatment is systemic corticosteroids. […] For mild pemphigus vulgaris, first-line treatment is typically rituximab or prednisone with or without azathioprine or mycophenolate mofetil. […] For moderate or severe pemphigus vulgaris, treatment is typically rituximab and higher doses of prednisone with or without azathioprine or mycophenolate mofetil.

• #15 Pemphigus: Updated Review and Emerging Therapies

  https://www.dermatologytimes.com/view/pemphigus-updated-review-and-emerging-therapies

  Systemic corticosteroids represent the first-line treatment in all forms of pemphigus. Adjuvant medications include azathioprine and mycophenolate compounds. Rituximab, an anti-CD20 monoclonal antibody that induces B-cell depletion, has been shown to improve patient survival. Currently, rituximab in conjunction with corticosteroids is first-line for treatment of moderate to severe pemphigus, thus allowing for a major corticosteroid-sparing effect and decreasing the risk of steroid-associated adverse events. […] As in all forms of pemphigus, corticosteroids are first-line in the treatment of PV. There is an accepted algorithm for the management of PV developed by international experts, which resulted from a Delphi consensus based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines.

• #15 Pemphigus: Updated Review and Emerging Therapies

  https://www.dermatologytimes.com/view/pemphigus-updated-review-and-emerging-therapies

  As in all forms of pemphigus, systemic corticosteroids are first-line in the treatment of PF. The treatment algorithm for PF is similar to that of PV. However, in localized forms of PF with a limited number of lesions, topical or intralesional corticosteroids can be used. […] There are many new treatments currently being tested for pemphigus. Second and third generation anti-CD20 monoclonal antibodies, such as veltuzumab, ocrelizumab, and obinutuzumab, have higher binding affinities and induce a more robust B-cell depletion compared to rituximab. The development of these next generation anti-CD20 monoclonal antibodies could lead to major progress in the treatment of pemphigus. Bruton tyrosine kinase (BTK) inhibitors, which downregulate several B-cell functions, and efgartigimod, an engineered Fc fragment derived from human IgG1, are also being studied for use in pemphigus treatment. Recently, researchers have engineered chimeric autoantibody receptor (CAAR)-T cells that recognize Dsg domains to selectively target autoreactive B-cells, which could become a precise therapy for pemphigus in the future. Other novel, potential therapies include cytokine inhibitors, anti-CD25 monoclonal antibodies, autologous hematopoietic stem cell transplantation, and polyclonal regulatory T cell (PolyTreg) therapy. Hopefully, one or more of these emerging therapies will soon be shown to be safe and effective in the treatment of pemphigus, and ultimately improve survival and quality of life for these patients.

• #16

  https://link.springer.com/article/10.1007/s13555-024-01191-3

  Pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis (AIBD) characterized by painful blistering of the skin and mucosa caused by autoantibodies that lead to loss of adhesion in the epidermis. Standard therapy for PV is corticosteroids, either alone or in combination with steroid-sparing immunosuppressants or infusions with rituximab. According to the published European guideline, high-dose intravenous immunoglobulin (IVIg) therapy with a dosage of 2 g per kg body weight distributed over 25 days every 4 weeks is a promising treatment option, especially for severe or refractory disease. […] This report describes a 73-year-old female patient with severe and recurrent disease who achieved stabilization with IVIg treatment. […] Hence, she was transitioned to a new IVIg preparation with a new manufacturing process, resulting in fewer side effects and an improved quality of life.

• #16

  https://link.springer.com/article/10.1007/s13555-024-01191-3

  Intravenous immunoglobulin (IVIg) therapy is a promising treatment option for severe pemphigus vulgaris (PV), particularly in the case of failure on standard treatment with steroids and additional immunosuppressants. […] Our case presents a patient with successful disease control under long-term IVIg therapy. Her quality of life improved owing to a switch of IVIg preparationa subject that has not yet been sufficiently addressed in the literature. […] The new 10% IVIg preparation with a novel production process is a highly purified and thus well-tolerated product. […] Nowadays, IVIg are used for the treatment of a wide range of diseases, and numerous clinical studies have shown their efficacy and tolerability. […] For treatment of PV, IVIg may be used as a secondary or tertiary therapeutical option in patients with particularly severe and treatment-refractory disease, as the patient presented above.

• #17

• #18 Successful Treatment of Pemphigus Vulgaris With Etanercept in Four Patients – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/successful-treatment-of-pemphigus-vulgaris-with-etanercept-in-four-patients-S1545961609P0940X/

  Pemphigus vulgaris is an autoimmune disease characterized by intraepidermal blister formation. The treatment of pemphigus vulgaris is generally regarded as difficult. Corticosteroids, the drug class of first choice, often must be combined with steroid-sparing agents to prevent hazardous, long-term side effects. The authors describe four patients with severe pemphigus vulgaris who were treated with the tumor necrosis factor (TNF)- antagonist, etanercept, twice weekly. In all four cases, the addition of etanercept produced dramatic clinical improvement and facilitated the reduction of corticosteroids necessary to maintain symptom control. Thus, etanercept may be an effective therapeutic agent for pemphigus vulgaris and should be considered as an alternative treatment option for patients presenting with recalcitrant disease.

• #19 Bullous pemphigoid – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/diagnosis-treatment/drc-20350419

  Treatment of bullous pemphigoid is aimed at healing the skin, relieving itching and pain, and preventing new blisters. Your healthcare professional will likely prescribe one or a combination of medicines: […] The main treatment for bullous pemphigoid is corticosteroid medicine applied to the affected area. Usually a strong steroid cream such as clobetasol propionate is used. […] Depending on how you respond to the first medicines you try, your healthcare professional may suggest something other than steroids. […] The oral medicines dapsone and doxycycline help control blisters. […] Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Azasan, Imuran), rituximab (Rituxan), mycophenolate (CellCept) and methotrexate (Trexall). These medicines also have a risk of harmful side effects, including infection. People who take these medicines need close follow-up and, at times, repeat blood tests to monitor for side effects.

• #20 Refractory pemphigus vulgaris: Treatment options – Journal of Skin and Sexually Transmitted Diseases

  https://jsstd.org/refractory-pemphigus-vulgaris-treatment-options/

  Additional doses of rituximab are found effective in relapsed cases or in patients who fail to achieve remission after a single cycle of rituximab. […] Intravenous immunoglobulin is effective for refractory pemphigus, it is used as adjuvant therapy to systemic corticosteroids and immunosuppressive adjuvants. […] Immunoadsorption is a therapeutic option for pemphigus. […] Plasmapheresis is a commonly used therapy. […] Infliximab is a chimeric monoclonal antibody against TNF alpha (Tumor Necrosis Factor alpha). […] Other therapies for pemphigus includes: topical tacrolimus and pimecrolimus, sulfasalazine with pentoxifylline, gold, tetracyclines with or without nicotinamide, chlorambucil, mizoribine, subcutaneous veltuzumab. […] Meticulous research on the immunology and cell biology of pemphigus vulgaris should be a top priority since safer and more effective treatment options are the need of the hour for this potentially fatal autoimmune disease.

• #21 Management of chronic wounds in patients with pemphigus | CWCMR

  https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR

  Rituximab can also be considered as first-line therapy. […] Following successful therapy, cutaneous manifestations of pemphigus tend to resolve in lighter skin phototypes, whereas post-inflammatory pigmentary changes often occur in patients with darker skin phototypes. […] The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. […] Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. […] When the disease becomes severe with extensive skin involvement, patients are typically admitted to the burn unit.

• #21 Management of chronic wounds in patients with pemphigus | CWCMR

  https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR

  Superimposed infections are often a significant cause of morbidity and mortality in pemphigus, either secondary to the breakdown of the skin barrier or immune suppression related to treatment. […] The most frequently reported cause of death in pemphigus is septicemia which is usually secondary to cutaneous Staphylococcus aureus infection. […] An ideal dressing should decrease the microbial burden without causing cytotoxic damage. […] Nutrition is a crucial aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage. […] Systemic corticosteroids are typically used for treatment initiation, however, due to the need for prolonged treatment and high morbidity of long corticosteroid courses, the early introduction of corticosteroid-sparing immunosuppressive agents is a key principle of treatment. […] Dressing selection is best guided by both the wound characteristics and dressing special features. The ideal dressing material should provide a moist environment to the wound while shielding it from bacterial invasion.

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

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

  Pemphigus may require hospitalization and involve many of the same interventions used in burn centers. […] The mainstay of pemphigus treatment is oral corticosteroids, usually prednisone. This typically requires extremely high doses, which can be dangerous for some, causing intestinal perforation and sepsis. […] If oral corticosteroids cannot be used, other options may be considered, including: local steroid injections, immunosuppressant drugs, like CellCept (mycophenolic acid), intravenous gamma globulin (IVIG), usually reserved for severe paraneoplastic pemphigus, biologic drugs, like Rituxan (rituximab) if other drug treatments fail. […] Plasmapheresis, a technique similar to dialysis used to clean blood, may be considered if Rituxan cannot be used. Tetracycline antibiotics may also be prescribed to prevent secondary infections, while talcum powder can prevent bedsheets and clothing from sticking to the lesions. Many people get better with treatment, although it can sometimes take years to fully recover. Others will need to take medications permanently to prevent a recurrence.

• #23 Pemphigus Vulgaris – UF Health

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

  Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: […] Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes: […] Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used. […] Plasmapheresis may be used along with systemic medicines to reduce the amount of antibodies in the blood. Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. […] Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.

• #24

  https://insight.jci.org/articles/view/92021

  Taken together, the basic, translational, and clinical research studies in the field have considerably expanded our understanding of PV pathogenesis and positioned PV to be a model autoimmune disease for the development of targeted therapeutic strategies that can avoid systemic immunosuppression. Below, we discuss strategies to therapeutically target various elements of the PV autoimmune response without affecting the immune system as a whole, which would ideally prevent the severe side effects and deaths associated with current PV therapy. […] The DSG3 CAAR approach offers several therapeutic advantages and fewer toxicities compared with antibody-based B cell depletion or CART therapy of cancer. First, CAAR T cells can expand and persist indefinitely in vivo to eliminate DSG3-autoreactive B cells, presumably with superior potency and durability compared with antibody-mediated B cell depletion. Second, antigen-specific B cell depletion avoids the risks of generalized immune suppression associated with pan-B cell depletion.

• #25 Oral Jak Inhibitor Upadacitinib Use in Treatment of Pemphigus Foliaceus | SKIN The Journal of Cutaneous Medicine

  https://skin.dermsquared.com/skin/article/view/2097

  Pemphigus foliaceus (PF) is a rare, blistering autoimmune condition that occurs when desmoglein-1 autoantibodies target and lead to loss of intercellular connections, resulting in blister formation on the skin. Current standard of care consists of highly immunosuppressive therapies such as prednisone, rituximab, and mycophenolate mofetil. A 43-year-old male with new-onset PF was treated with upadacitinib, a JAK inhibitor. He saw resolution of his blisters within 12 weeks of treatment and remains in remission from his PF. Our case demonstrates that JAK inhibition may prove to be an effective strategy in preventing dsg-1-triggered blisters. […] JAK1 inhibitors also may prove to be a safer, less immunosuppressive alternative to the highly immunosuppressive agents available today. Larger studies will be required to study the drugs efficacy in others with PF.

• #26 Pemphigus Foliaceus: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23029-pemphigus-foliaceus

  Symptoms can reduce with topical creams, ointments or drugs. […] Treatment for pemphigus foliaceus is unique to each person diagnosed with the condition and could include: […] Using corticosteroids (topical creams or oral medicines) or topical calcineurin inhibitors to reduce blistering. […] Taking immunosuppressive drugs like rituximab, methotrexate, mycophenolate or azathioprine. […] Every treatment option has different risks, so discuss the side effects with your provider before beginning your treatment. […] Once treatment begins, your provider will follow up with you a couple of weeks after your diagnosis to make sure treatment is effective. […] On average, you might notice new blisters stop forming after two weeks and the sores on your skin will start to heal. […] There is no cure for pemphigus foliaceus. […] Your provider will help you manage your symptoms and prevent infections.

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

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

  Treatments include corticosteroids given topically or orally. […] The standard initial treatment is corticosteroids, which are administered topically and orally. […] So we also use other medications, injections and infusions that suppress the body’s immune response and deplete the antibodies. Mycophenolate mofetil and rituximab are two common ones. […] The majority of patients with these disorders will see very significant improvement after three months of treatment. Some patients go into a long-term remission after that, while others will need a second treatment a year later. Most will require ongoing treatment. […] Patients are not cured, per se, but the symptoms can be controlled, skin will heal if cared for properly, and, over the long term, there is a good likelihood that the disorders will go away.

• #28 Rituximab: Revolutionizing the Treatment of Pemphigus | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-rituximab-revolutionizing-treatment-pemphigus-articulo-S1578219017304286

  Rituximab has achieved complete clinical response rates of 75% in clinical trials and case series. […] Rituximab (RTX) is an anti-CD20 monoclonal antibody that has been used off-label to treat pemphigus for several decades. It is used particularly to treat patients with refractory disease or who are unable to tolerate conventional treatment. […] The results of this trial suggest that RTX could revolutionize the treatment of pemphigus, replacing corticosteroid therapy as the first-line treatment option.

• #29 Treatment of pemphigus: An Indian perspective – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/treatment-of-pemphigus-an-indian-perspective/

  We follow a three tier approach in the management of our pemphigus patients. […] Patients with mild disease, who may not require long term, high dose corticosteroids are managed by conventional modalities. […] The major change in the therapeutics of pemphigus was the concept of pulse therapy introduced by Pasricha and Gupta. […] We prefer DCP pulse therapy in patients with moderate to severe disease or patients with mild disease who fail conventional treatment and have no contraindication for pulse therapy. […] With the availability of novel targeted therapeutic agents, patients who fail DCP/conventional treatment, and those in whom they are contraindicated or cause severe adverse effects can now be offered IVIG and/or rituximab. […] Though we are following a three tier approach, we offer rituximab treatment to all pemphigus patients irrespective of severity.

• #29 Treatment of pemphigus: An Indian perspective – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/treatment-of-pemphigus-an-indian-perspective/

  Our own experience and evidence in the literature suggests that treatment with rituximab results in early remission, fewer relapses, and an overall better prognosis. […] Treating pemphigus is akin to walking a tightrope. The therapeutic efficacy of any drug has to be balanced against its potential toxicity and systemic immunosuppression. No individual drug is uniformly effective in all patients. Effective therapy for pemphigus lies in combining various agents to maximize efficacy and minimize side effects.

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