Materiały źródłowe

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

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

  As the cause of pemphigus is unknown, there isnt a way to prevent the condition. […] Treatment helps manage symptoms and could be ongoing throughout your life. […] Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections.

• #2 Pemphigus in Dogs | PetMD

  https://www.petmd.com/dog/conditions/skin/pemphigus-dogs

  Pemphigus isnt a preventable condition. […] Most dogs with pemphigus should continue year-round parasite prevention. Fleas can significantly worsen your dogs discomfort by causing itchiness, secondary infections, and allergic reactions. Speak with your vet about the preventative you are using for your dog.

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

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

  Given that we know so little about what triggers pemphigus, it’s hard to suggest what you can do to avoid it if you have never had it before. […] Here are some self-help tips that can help: […] Treat skin injuries immediately. This may include topical antibiotics to prevent infection, icing wounds to reduce inflammation, or compression bandages to control swelling. […] Avoid excessive sun exposure. Wear appropriate clothing when outdoors and always wear high-SPF sunscreen. […] Maintain good oral health. This can aid in the healing of oral blisters and prevent infections that can reactivate the disease. […] Manage your stress. Explore stress-reduction techniques like meditation, yoga, tai chi, guided imagery, or progressive muscle relaxation (PMR) to unwind and gain better control of your emotions.

• #4 Management of pemphigus vulgaris: challenges and solutions

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4622091/

  The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. […] Subsequently, the real challenge is to prevent relapses in the long run and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. Such intent requires close clinical monitoring of efficacy and safety of treatment. […] The challenge is to minimize hospitalization and improve patients quality of life. […] The aim of pharmacologic therapy for PV is to reduce inflammatory response and autoantibody production. […] The EDF proposed a useful treatment algorithm for use after the consolidation phase. […] If oral CSs are given as monotherapy, add an immunosuppressant.

• #5 Management of pemphigus vulgaris: challenges and solutions

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4622091/

  Monitor the patient for adverse events, and remember that prolonged immunosuppressive therapy increases the risk of side effects. […] Finding the most effective steroid-sparing agent has formed much of the focus of recent research; however, evidence is inconclusive. […] Prophylactic infusion after complete remission does not seem to provide any additional benefit. […] Concomitant long-term antibiotic and prophylaxis for herpes virus has been shown to drastically reduce the rate of infections.

• #6 Multidrug Protocol May Cure Pemphigus, Pemphigoid – The Dermatology Digest

  https://thedermdigest.com/multidrug-protocol-may-cure-pemphigus-pemphigoid/

  Selective elimination of autoantibodies may lead to a rebound effect [or flare] due to stimulation of autoantibody producing cells. […] Outcomes are better with the multidrug protocol compared to treatment with only prednisone and rituximab because of the latter treatments inability to selectively suppress production of pathogenic autoantibodies, according to Dr. Grando. […] Maintenance with IVIG and an immunosuppressor should be continued for at least 2 years in expectation that the critical mass of autoreactive plasma cells dies off during this timeframe, according to Dr. Grando. […] If you keep patients lesion free for that long a period of time, there is scientific evidence to suggest that critical mass of autoreactive plasma cells will die off during this timeframe. […] And thats why the current approach with prednisone and rituximab is not really helpful [for a] real chance for cure.

• #7 Pemphigus: 10 tips for managing

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

  Try NOT to injure your skin. If you cut, bump, or injure your skin in any way, new blisters can form. […] Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. Many medicines taken to control the pemphigus also increase your risk of getting an infection because the medicines suppress your immune system. To decrease your risk of getting an infection, keep all wounds, including cuts and scrapes, clean. […] Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. If you’ve been diagnosed with pemphigus foliaceus or one of its subtypes, such as drug-induced pemphigus or fogo selvagem, you want to avoid being out in the sun. Stay away from heat. These can cause the blisters to spread, so you get blisters where you previously had none. […] Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works. No one treatment works for everyone. Your dermatologist can also help you manage possible side effects and answer questions about the disease.

• #8 Efficacy of Repeated Courses of Rituximab as Treatment for Pemphigus Vulgaris | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3649

  Rituximab serves as a disease-modifying agent, notably for patients with moderate-to-severe pemphigus vulgaris. […] With repeated cycles of treatment with rituximab, high efficacy is maintained and remission can be induced despite failures in previous cycles. […] The first and most important issue emerging from the current study is that rituximab is potentially a disease-modifying agent; i.e., substantial improvement was observed with each subsequent cycle, and this was associated with fewer flare-ups, longer remissions, and a crescendo effect. […] Given this finding as well as the beneficial effect of rituximab on the disease course, it is preferable to administer rituximab as the first-line adjuvant treatment (along with corticosteroids). […] The reduction in prednisone dose on completion of each rituximab cycle and the lower starting dose at the beginning of each cycle served as additional verifications of treatment efficacy, which is in agreement with the literature. […] Rituximab is an effective steroid-sparing agent and has a positive long-term clinical effect if used repeatedly during relapses. […] With each subsequent cycle, an improvement is observed with a substantially longer remission time, thus rendering rituximab a potent disease-modifying agent.

• #9 Pemphigus: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/pemphigus-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. […] Taking both a corticosteroid like prednisone, and an immunosuppressant medication like azathioprine to quiet the immune system, may deliver better results. […] To treat health problems that pemphigus can cause, some patients need to be hospitalized. 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.

• #10 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Treatment-and-Self-Help-Tips-for-Pemphigus-Vulgaris.aspx

  There is no cure for pemphigus vulgaris, but symptoms can be kept under control with the use of corticosteroids or immunosuppressants. […] Antibiotic and antifungal treatment, either for existing infections or as prophylaxis. […] These medications may also increase the risk of contracting infection, which is why certain precautions should be taken. […] Avoiding activities that could cause the skin to become damaged, such as contact sports. […] Maintaining good oral and skin hygiene. […] Seeking medical attention as soon as any symptoms of infection develop.

• #11 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

  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. […] Prevention of infections is a crucial factor in the overall clinical outcome. […] Patients should be encouraged not to pop or drain blisters since this could provide a route for infection. […] The ideal dressing material should provide a moist environment to the wound while shielding it from bacterial invasion.

• #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%. […] 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. […] It is advisable for pemphigus patients to reduce the consumption of foods and drinks with a high content of the above chemical compounds.

• #13

  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. […] 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. […] Overall tolerability of IVIg is very good and severe side effects are rare, but may occur as a result of aggregation, complement activation, and protein impurities. […] The new 10% IVIg preparation with a novel production process is a highly purified and thus well-tolerated product. […] 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.

• #14 Multidrug Protocol May Cure Pemphigus, Pemphigoid – The Dermatology Digest

  https://thedermdigest.com/multidrug-protocol-may-cure-pemphigus-pemphigoid/

  Dr. Sergei Grando shares his multidrug protocol shown to offer potential cure for the majority of pemphigus and pemphigoid patients. […] But steroids alone far from solve the problem, said Dr. Grando, who directs one of the largest centers of excellence in the U.S. treating patients with pemphigus and pemphigoid. […] Another problem is that prednisone, instead of being a friend, becomes a foe. […] Thats why, unfortunately, mortality to date, which is about 10% on average, stems from complications from treatment rather than from the disease itself, he said. […] The multidrug IVIG regimen achieved three principal treatment objectives: rapid control of pemphigus symptoms, stable disease remission, and prevention of flares, concluded Dr. Grando. […] Finally, the protocol prevents autoantibody production with the use of cytotoxic immunosuppression.

• #15 Bullous Pemphigoid: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/15855-bullous-pemphigoid

  Healthcare providers and medical researchers arent sure how to prevent bullous pemphigoid. […] Certain medications may trigger bullous pemphigoid, including the diabetes medication alogliptin. Ultraviolet (UV) light and ionizing radiation may also trigger or aggravate bullous pemphigoid.

• #16 Pemphigus (includes “pemphigus vulgaris” [“PV”], “pemphigus vegetans”, “pemphigus foliaceus”, “pemphigus erythematosus”, “paraneoplastic pemphigus”, and “drug-related pemphigus”)1 – CDHO

  https://cdho.org/factsheets/pemphigus/

  Is medical consult advised? […] Yes, if not already done, all patients/clients diagnosed with oral pemphigus lesions should be referred to a dermatologist, as well as an ophthalmologist for assessment of ocular lesions. […] Yes, if the disease is poorly controlled (as evidenced by severity and extent of mucous membrane and skin involvement). […] Yes, if after treatment and remission, new lesions are observed. The dental hygienist should ensure referral back to the specialist physician for re-evaluation and further treatment. […] Is medical consult advised? […] See above. Also, consultation may be appropriate for consideration of prophylactic prednisone prior to invasive procedures in order to reduce disease flares. […] Disease flares following dental work are fairly common. As a result, some authorities recommend prophylactic administration of oral prednisone prior to dental work (e.g., 20 mg/day, in addition to the patient/client’s normal requirement, for 5 to 7 days) for each dental procedure associated with trauma to the gingiva. […] Oral prophylaxis should ideally be performed prior to the initiation of systemic or topical therapy.

• #17 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. […] 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.

• #18 Modified Rheumatoid Arthritis Protocol for Rituximab in Pemphigus: A Retrospective Case Series | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/rituximab-pemphigus/

  A modified rheumatoid arthritis protocol for rituximab was shown to be effective and safe in treating patients with pemphigus. […] Clinicians should focus on adopting rituximab modified RA protocols in patients with pemphigus; however, further research should aim to delineate a more precise judgment.

• #19 Pemphigus Foliaceus: Causes, Symptoms and Images — DermNet

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

  The primary aim of treatment is to prevent new areas from developing bacterial infections and promote healing of affected areas. […] Topical treatment with corticosteroids is usually all that is necessary for mild pemphigus foliaceus. […] For more severe cases treatment is similar to that for pemphigus vulgaris. […] Oral antibiotics may be prescribed to treat secondary bacterial infection.

• #20 Pemphigus Vulgaris (PV) | Diagnosis, Treatment & Management

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

  PV is treatable and prompt intervention may prevent the development of more severe symptoms. […] Treatment aims: To prevent/strictly limit development of new blisters and erosions. […] The consensus group also provides recommendations on supportive treatment, such as proper dental care, intralesional injections of corticosteroids, topical treatment with potent corticosteroids, antiseptic baths, analgesics, local anesthetics and nutritional management, and prophylaxis against side effects in prolonged corticosteroid therapy.

• #21 Interventions for pemphigus vulgaris and pemphigus foliaceus | Cochrane

  https://www.cochrane.org/CD006263/SKIN_interventions-for-pemphigus-vulgaris-and-pemphigus-foliaceus

  There is inadequate information available at present to ascertain the optimal therapy for pemphigus vulgaris or pemphigus foliaceus. […] Further research is required, especially to assess the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve harm:benefit analyses. […] A range of interventions have been described for treatment of pemphigus, however the optimal therapeutic strategy has not been established. […] Multiple treatments are available for pemphigus vulgaris and pemphigus foliaceus and there is a variation in dosage plan and combination of drugs used, which makes choice of treatment schedule complex. […] Treatments need to be chosen after careful consideration of the potential benefits and side effects, in the context of the individual’s other medical conditions.

• #22 Interventions for pemphigus vulgaris and pemphigus foliaceus | Cochrane

  https://www.cochrane.org/CD006263/SKIN_interventions-for-pemphigus-vulgaris-and-pemphigus-foliaceus

  This review found insufficient information to conclude which is the most effective and safest treatment regimen. Further studies are required to determine the optimal treatment regimen, especially to assess the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve harm:benefit analyses.

Zobacz więcej