Materiały źródłowe

• #1 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. Long-term use this type of medicine comes with the risk of skin thinning and easy bruising. Your healthcare professional might also suggest a steroid medicine taken by mouth. Oral steroids come with the risk of harmful side effects, such as weak bones, diabetes, stomach ulcers and eye problems. […] Depending on how you respond to the first medicines you try, your healthcare professional may suggest something other than steroids.

• #1 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  If the pemphigoid is very widespread, hospital admission may be arranged to dress blisters and erosions. […] Medical treatment involves: ultrapotent topical steroids to treat limited disease cream, moderate potency topical steroids and emollients to relieve itch and dryness, systemic steroids (eg, prednisone) they are however associated with an increased risk of adverse and potentially fatal side effects, tetracycline antibiotics, usually doxycycline 200 mg/day; doxycycline has fewer adverse effects than oral corticosteroids and is effective on its own for mild disease, other steroid-sparing medications on their own or in combination with steroids, antibiotics for secondary bacterial infection, pain relief. […] Most patients with bullous pemphigoid are treated with steroid tablets, either prednisone or prednisolone at an initial dose of 0.5 mg/kg/day. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced once there are fewer than 3 significant blisters, over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5-10 mg prednisone daily). These other medications may include doxycycline or: dapsone, nicotinamide, methotrexate, azathioprine, mycophenolate, intravenous immunoglobulin, rituximab, dupilumab, omalizumab.

• #1 Bullous pemphigoid: Diagnosis and treatment

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

  Other medications are also prescribed to treat bullous pemphigoid. […] Tetracycline, doxycycline, and dapsone are antibiotics, which can reduce the inflammation inside your body. […] Some patients take a medication called an immunosuppressant. This medication helps to calm the immune system. […] Wound care: Open blisters and raw skin can become infected. Wound care can prevent infection, relieve pain, and speed up healing. […] Your dermatologist will create a wound care plan that meets your individual needs. […] The amount of time you need to treat bullous pemphigoid will depend on how severe the disease is and your response to treatment. […] Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. […] Following a self-care plan for bullous pemphigoid can improve the results you get from treatment and how well you feel.

• #1 Biological treatment for bullous pemphigoid

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10172582/

  Bullous pemphigoid (BP) is the most common autoimmune subepidermal bullous disease. Topical or systemic corticosteroids are often used as the first-line treatment. However, long-term corticosteroid use may lead to significant side effects. Therefore, various adjuvant immunosuppressant therapies are used as steroid-sparing agents, with accumulating reports of biological treatments for severely recalcitrant BP. […] We identified nine eligible patients treated with rituximab (seven), omalizumab (three), or dupilumab (one). The mean age at diagnosis was 60.4 years, the average BP duration before biologic initiation was 1.9 years, and the average previous treatment failure was 2.11 therapies. The mean follow-up period from the first biological treatment to the last visit was 29.3 months. Satisfactory response, defined as clinical improvement, was achieved in 78% (7) of the patients, and total BP clearance was achieved in 55% (5) of the patients at the last follow-up visit.

• #2 Bullous pemphigoid: Diagnosis and treatment

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

  If the medical tests confirm that you have bullous pemphigoid, your dermatologist will create a personalized treatment plan. […] Treatment can help heal your skin so that bullous pemphigoid goes into remission. […] Your dermatologist will tailor your treatment plan to your needs. The goals of treatment are to: Stop (or reduce) new blisters, Heal existing blisters and sores, Treat an infection if it has developed, Relieve any itch and pain. […] A personalized treatment plan usually includes medication and wound care. […] Medication: Your dermatologist may prescribe creams and ointments called corticosteroids. These help to heal your skin, prevent new blisters from appearing, and relieve the itch. […] If you have severe bullous pemphigoid, your dermatologist may prescribe corticosteroid pills.

• #2 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Dermovate cream (clobetasol) is a very effective treatment for localised disease. It can also be tried BD in more extensive disease, as the side effects are very much less then when compared to systemic steroids. […] Systemic steroids are the mainstay of treatment. The initial dose is as follows: 20 mg / day in localised-mild disease, 40 mg / day in moderate disease, 50-70 mg / day in severe disease. The dose of steroid can usually be reduced over a matter of weeks to 15-20 mg / day, and then more slowly after that. It is felt prudent to reduce the daily prednisolone dose by 1 mg / month once the dose is below 10 mg / day, if blisters start to break through the dose can be increased, and then after a period of stabilisation gradually reduced again. The additional use of Dermovate cream may enable a lower dose of prednisolone. […] Immunosuppressive drugs such as methotrexate and mycophenolate mofetil are generally only considered if the steroid dose cannot be reduced to an acceptable level. The role of biologic therapies remains unclear.

• #2 Bullous pemphigoid – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/bullous-pemphigoid/

  The goal of therapy is to decrease blister formation, promote healing of blisters and erosions, and achieve the minimal dose necessary to control the disease process. Therapy must be individualized for each patient, keeping in mind the pre-existing conditions and other patient-specific factors. […] Localized BP often can be treated successfully with topical steroids alone. More extensive disease, which is often more difficult to control, is usually treated with systemic anti-inflammatory and immunosuppressive agents, oral corticosteroids being the mainstay of treatment. Oral prednisone/prednisolone doses range from 0.3 to 1.25 mg/kg body weight/day, which usually controls disease within 1-2 weeks; the dose is then progressively tapered. […] Several studies have suggested the use of concomitant immunosuppressive agents to achieve a corticosteroid-sparing effect.

• #2 Treatments for bullous pemphigoid | Cochrane

  https://www.cochrane.org/CD002292/SKIN_treatments-bullous-pemphigoid

  Treatment with a lower amount of clobetasol propionate cream (10 to 30 grams per day, decreased over 4 months) is equally effective and safe. […] Prednisolone, an oral corticosteroid, in the dose of 0.5 mg/kg/day, may be adequate to control disease in most people and reduces adverse effects compared to higher doses of oral corticosteroid. […] Initiating treatment with 200 mg/day of doxycycline leads to acceptable blister control compared to oral prednisolone (0.5 mg/kg/day) and is safer. […] A study with 20 participants suggests that nicotinamide (a form of vitamin B3) and tetracycline (an antibiotic used to treat a wide variety of infections) may be an effective alternative to prednisone and may decrease treatment-associated death. […] Adding azathioprine, a drug which suppresses the immune system, to an oral corticosteroid does not improve disease control; it may lead to a reduced need for oral corticosteroid.

• #2 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. Long-term use this type of medicine comes with the risk of skin thinning and easy bruising. Your healthcare professional might also suggest a steroid medicine taken by mouth. Oral steroids come with the risk of harmful side effects, such as weak bones, diabetes, stomach ulcers and eye problems. […] Depending on how you respond to the first medicines you try, your healthcare professional may suggest something other than steroids.

• #2 Bullous pemphigoid: Diagnosis and treatment

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

  Other medications are also prescribed to treat bullous pemphigoid. […] Tetracycline, doxycycline, and dapsone are antibiotics, which can reduce the inflammation inside your body. […] Some patients take a medication called an immunosuppressant. This medication helps to calm the immune system. […] Wound care: Open blisters and raw skin can become infected. Wound care can prevent infection, relieve pain, and speed up healing. […] Your dermatologist will create a wound care plan that meets your individual needs. […] The amount of time you need to treat bullous pemphigoid will depend on how severe the disease is and your response to treatment. […] Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. […] Following a self-care plan for bullous pemphigoid can improve the results you get from treatment and how well you feel.

• #2

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Bullous pemphigoid (BP) is a common autoimmune bullous disease affecting mainly the elderly, with rising incidence due to increased life expectancy. […] This review focuses on novel treatment options for BP, exploring therapies targeting different immune pathways. Rituximab, a CD20 monoclonal antibody, depletes B-lymphocytes and has shown efficacy in severe cases. Dupilumab, targeting interleukin (IL)-4 receptor and thus blocking IL-4 and IL-13, downregulates type 2 helper (Th2) responses and has demonstrated promising results. […] These novel therapies offer promising alternatives for managing BP, with potential to improve outcomes and reduce high cumulative doses of systemic corticosteroids and related toxicities. Further research, including controlled clinical trials, is needed to establish their efficacy, safety, and optimal dosing regimens for BP management.

• #3 Bullous Pemphigoid Treatment & Management: Medical Care, Complications, Diet

  https://emedicine.medscape.com/article/1062391-treatment

  As in other autoimmune bullous diseases, the general aims of therapy are 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 individualized for each patient, keeping in mind preexisting conditions and other patient-specific factors. […] More specifically, treatment is directed at reducing inflammatory response and autoantibody production. Although target-specific therapy is the „Holy Grail” for immunodermatologists, nontargeted treatments are used at present. The medications most often given are anti-inflammatory agents (eg, corticosteroids, tetracyclines, and dapsone) and immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, and cyclophosphamide). Strong topical corticosteroid treatment may achieve disease control while avoiding the adverse effects of systemic corticosteroids.

• #3 Treatments for bullous pemphigoid | Cochrane

  https://www.cochrane.org/CD002292/SKIN_treatments-bullous-pemphigoid

  Treatment with a lower amount of clobetasol propionate cream (10 to 30 grams per day, decreased over 4 months) is equally effective and safe. […] Prednisolone, an oral corticosteroid, in the dose of 0.5 mg/kg/day, may be adequate to control disease in most people and reduces adverse effects compared to higher doses of oral corticosteroid. […] Initiating treatment with 200 mg/day of doxycycline leads to acceptable blister control compared to oral prednisolone (0.5 mg/kg/day) and is safer. […] A study with 20 participants suggests that nicotinamide (a form of vitamin B3) and tetracycline (an antibiotic used to treat a wide variety of infections) may be an effective alternative to prednisone and may decrease treatment-associated death. […] Adding azathioprine, a drug which suppresses the immune system, to an oral corticosteroid does not improve disease control; it may lead to a reduced need for oral corticosteroid.

• #3 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Dermovate cream (clobetasol) is a very effective treatment for localised disease. It can also be tried BD in more extensive disease, as the side effects are very much less then when compared to systemic steroids. […] Systemic steroids are the mainstay of treatment. The initial dose is as follows: 20 mg / day in localised-mild disease, 40 mg / day in moderate disease, 50-70 mg / day in severe disease. The dose of steroid can usually be reduced over a matter of weeks to 15-20 mg / day, and then more slowly after that. It is felt prudent to reduce the daily prednisolone dose by 1 mg / month once the dose is below 10 mg / day, if blisters start to break through the dose can be increased, and then after a period of stabilisation gradually reduced again. The additional use of Dermovate cream may enable a lower dose of prednisolone. […] Immunosuppressive drugs such as methotrexate and mycophenolate mofetil are generally only considered if the steroid dose cannot be reduced to an acceptable level. The role of biologic therapies remains unclear.

• #3 Bullous pemphigoid – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/bullous-pemphigoid/

  The overall response rate to dapsone, when given either alone or in combination with corticosteroids or immunosuppressive agents, is about 81% in BP. […] Up to 24% of patients with BP do not respond to conventional therapy. Other drugs for treating BP include biologicals (anti-TNF drugs, rituximab), IVIg and plasma exchange. […] IVIg appears to be an effective alternative in treating patients with severe BP whose disease is nonresponsive to conventional therapy or who are at risk of experiencing serious or potentially fatal side effects from conventional immunosuppressive therapy. […] Among all the treatment modalities in India, the most commonly used is steroids, either oral or in the form of pulse therapy. Non-steroidal immunosuppressive drugs are added as adjuvants to increase the efficacy and to have a steroid sparing effect.

• #3 Treatment of Bullous Pemphigoid With Rituximab: Critical Analysis of the Current Literature – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/treatment-of-bullous-pemphigoid-with-rituximab-critical-analysis-of-the-current-literature-S1545961613P0672X

  The objective of this review was to critically analyze the currently available literature on the use of rituximab to treat patients with bullous pemphigoid (BP). […] Rituximab is a useful option for BP patients who are recalcitrant to conventional therapy. […] A specific protocol for the use of rituximab to treat BP patients is not yet available. […] Ten out of 16 patients received rituximab based on the Lymphoma Protocol, which included 4 once-weekly infusions at a dose of 375 mg/m2 per infusion as a single cycle. […] Four patients were treated with a modified Lymphoma Protocol. […] Two patients were treated with the Rheumatoid Arthritis Protocol that consists of a single cycle of 1gm rituximab, given on days 1 and 15.

• #4 Treatments Of Choice For Bullous Pemphigoid

  https://www.skintherapyletter.com/dermatology/bullous-pemphigoid/

  Topical superpotent steroids, e.g., clobetasol proprionate 0.05% have a role in treating localized and mild-to-moderate generalized disease. […] Antibiotics and niacinamide (nicotinamide) are useful first line treatments that may spare some patients from immunosuppressant therapy. […] The combination of tetracycline and niacinamide may confer some additional benefit to tetracycline alone. […] Azathioprine (up to 2.5mg/kg/d) in combination with systemic steroids has a steroid sparing effect, but is associated with increased morbidity and mortality. […] Experience with cyclophosphamide, methotrexate, ciclosporin, mycophenolate mofetil and chlorambucil is very limited. […] It is important that patients with bullous pemphigoid are followed regularly while being treated. […] Treatment must be regularly reviewed and attempts made to reduce, and ultimately withdraw all treatment if possible.

• #4 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Dermovate cream (clobetasol) is a very effective treatment for localised disease. It can also be tried BD in more extensive disease, as the side effects are very much less then when compared to systemic steroids. […] Systemic steroids are the mainstay of treatment. The initial dose is as follows: 20 mg / day in localised-mild disease, 40 mg / day in moderate disease, 50-70 mg / day in severe disease. The dose of steroid can usually be reduced over a matter of weeks to 15-20 mg / day, and then more slowly after that. It is felt prudent to reduce the daily prednisolone dose by 1 mg / month once the dose is below 10 mg / day, if blisters start to break through the dose can be increased, and then after a period of stabilisation gradually reduced again. The additional use of Dermovate cream may enable a lower dose of prednisolone. […] Immunosuppressive drugs such as methotrexate and mycophenolate mofetil are generally only considered if the steroid dose cannot be reduced to an acceptable level. The role of biologic therapies remains unclear.

• #4 Bullous Pemphigoid Treatment & Management: Medical Care, Complications, Diet

  https://emedicine.medscape.com/article/1062391-treatment

  Several studies found initial treatment with doxycycline to be effective and to be associated with a lower incidence of adverse effects as compared with prednisolone. […] Proper treatment of bullous pemphigoid depends on the severity of the disease. For localized disease, a topical steroid plus a systemic anti-inflammatory (eg, tetracycline or nicotinamide) may be sufficient. The effects of monotherapy with nicotinamide are unknown. For more severe cases, systemic steroids along with immunosuppressants may be needed for control. If the disease proves difficult to control, it is worthwhile to consider treatment with the anti-CD20 antibody rituximab, which is relatively specific in targeting the antibody-producing B cells. […] Some case reports have suggested that Janus kinase (JAK) inhibitors (eg, tofacitinib, baricitinib, upadacitinib, and abrocitinib) may prove useful for treatment of bullous pemphigoid.

• #4 Bullous Pemphigoid: Causes, Symptoms & Treatment

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

  For mild cases of bullous pemphigoid, the best treatments are topical corticosteroid creams or ointments that you rub directly on the affected areas. […] For moderate-to-severe cases of bullous pemphigoid, your healthcare provider may prescribe an oral corticosteroid, like prednisone, in addition to a steroid-sparing immunomodulatory agent such as dapsone, mycophenolate mofetil, azathioprine, methotrexate or chlorambucil. […] If you cant take cant corticosteroids or other immunomodulatory agents, your healthcare provider may prescribe oral tetracycline or doxycycline pills. […] For refractory cases of bullous pemphigoid, your healthcare provider may prescribe rituximab or IVIG infusions. […] You should start to feel better a few days after starting treatment.

• #4 Biological treatment for bullous pemphigoid

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10172582/

  Efficient and safe novel therapies can be considered in recalcitrant steroid-dependent BP non-responsive to conventional immunosuppressant therapies. […] Cumulative data on biological therapies, such as rituximab (RTX) and omalizumab, indicate their clinical benefits in patients with BP. In addition, emerging data on BP treatment with dupilumab (DUPI) indicate that it may be an additional immunomodulatory treatment that efficiently controls disease while maintaining a steroid-sparing effect, further expanding the limited existing armamentarium. […] Patients were treated non-exclusively with various biologic therapies, including RTX, omalizumab, and DUPI, without any enforced washout period. […] Clinical response was assessed using the degree of skin lesions and pruritus, according to the recommendations of an international panel of experts for outcome measures in bullous pemphigoid.

• #5 Bullous Pemphigoid Treatment & Management: Medical Care, Complications, Diet

  https://emedicine.medscape.com/article/1062391-treatment

  As in other autoimmune bullous diseases, the general aims of therapy are 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 individualized for each patient, keeping in mind preexisting conditions and other patient-specific factors. […] More specifically, treatment is directed at reducing inflammatory response and autoantibody production. Although target-specific therapy is the „Holy Grail” for immunodermatologists, nontargeted treatments are used at present. The medications most often given are anti-inflammatory agents (eg, corticosteroids, tetracyclines, and dapsone) and immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, and cyclophosphamide). Strong topical corticosteroid treatment may achieve disease control while avoiding the adverse effects of systemic corticosteroids.

• #5 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  If the pemphigoid is very widespread, hospital admission may be arranged to dress blisters and erosions. […] Medical treatment involves: ultrapotent topical steroids to treat limited disease cream, moderate potency topical steroids and emollients to relieve itch and dryness, systemic steroids (eg, prednisone) they are however associated with an increased risk of adverse and potentially fatal side effects, tetracycline antibiotics, usually doxycycline 200 mg/day; doxycycline has fewer adverse effects than oral corticosteroids and is effective on its own for mild disease, other steroid-sparing medications on their own or in combination with steroids, antibiotics for secondary bacterial infection, pain relief. […] Most patients with bullous pemphigoid are treated with steroid tablets, either prednisone or prednisolone at an initial dose of 0.5 mg/kg/day. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced once there are fewer than 3 significant blisters, over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5-10 mg prednisone daily). These other medications may include doxycycline or: dapsone, nicotinamide, methotrexate, azathioprine, mycophenolate, intravenous immunoglobulin, rituximab, dupilumab, omalizumab.

• #5 Treatments for bullous pemphigoid | Cochrane

  https://www.cochrane.org/CD002292/SKIN_treatments-bullous-pemphigoid

  Treatment with a lower amount of clobetasol propionate cream (10 to 30 grams per day, decreased over 4 months) is equally effective and safe. […] Prednisolone, an oral corticosteroid, in the dose of 0.5 mg/kg/day, may be adequate to control disease in most people and reduces adverse effects compared to higher doses of oral corticosteroid. […] Initiating treatment with 200 mg/day of doxycycline leads to acceptable blister control compared to oral prednisolone (0.5 mg/kg/day) and is safer. […] A study with 20 participants suggests that nicotinamide (a form of vitamin B3) and tetracycline (an antibiotic used to treat a wide variety of infections) may be an effective alternative to prednisone and may decrease treatment-associated death. […] Adding azathioprine, a drug which suppresses the immune system, to an oral corticosteroid does not improve disease control; it may lead to a reduced need for oral corticosteroid.

• #5 Treatments Of Choice For Bullous Pemphigoid

  https://www.skintherapyletter.com/dermatology/bullous-pemphigoid/

  Topical superpotent steroids, e.g., clobetasol proprionate 0.05% have a role in treating localized and mild-to-moderate generalized disease. […] Antibiotics and niacinamide (nicotinamide) are useful first line treatments that may spare some patients from immunosuppressant therapy. […] The combination of tetracycline and niacinamide may confer some additional benefit to tetracycline alone. […] Azathioprine (up to 2.5mg/kg/d) in combination with systemic steroids has a steroid sparing effect, but is associated with increased morbidity and mortality. […] Experience with cyclophosphamide, methotrexate, ciclosporin, mycophenolate mofetil and chlorambucil is very limited. […] It is important that patients with bullous pemphigoid are followed regularly while being treated. […] Treatment must be regularly reviewed and attempts made to reduce, and ultimately withdraw all treatment if possible.

• #5

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  BP treatment can be very challenging as BP is a chronic disease requiring long-term management. Glucocorticoids are the cornerstone of BP treatment. High-dose systemic corticosteroids are used to induce remission, followed by gradual tapering. […] Corticosteroid-sparing adjuvant agents are prescribed when monotherapy with glucocorticoids is inadequate to achieve disease control or when there is a relapse during corticosteroid tapering. […] The aim of this review was to summarize and discuss the novel targeted BP treatment options that have been developed and tested in clinical trials in recent years, based on the complex pathophysiology of BP. […] Rituximab therapy usually results in a major reduction in circulating BP180-specific B lymphocytes, as well as a dramatic reduction of anti-BP180 antibodies, and decreases expression of proinflammatory IL-15 and IL-6, leading to an improvement of BP skin manifestations. […] Dupilumab may further improve pruritus in BP by directly reducing IL-13 and by indirectly downregulating IL-31 production by eosinophils. […] A case series involving 13 BP patients from five academic centers receiving dupilumab, 92.3% (12/13) of the patients achieved disease clearance or satisfactory response.

• #6 Bullous Pemphigoid – Dermatologic Disorders – Merck Manual Professional Edition

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

  Corticosteroids, topical or oral […] High-potency topical corticosteroids (eg, clobetasol 0.05% cream) should be used for localized disease and may reduce the required dose of systemic drugs. […] Patients with generalized disease often require systemic treatment with prednisone 0.5 mg/kg orally once a day, which can be tapered to a maintenance level of 0.1 mg/kg/day after several weeks. […] Bullous pemphigoid occasionally responds to the anti-inflammatory activity of certain drugs, such as the combination of tetracycline or minocycline and nicotinamide. Other treatment options include monotherapy with dapsone, sulfapyridine, or erythromycin. IV immune globulin has been used occasionally. […] For patients with generalized and recalcitrant disease, and sometimes to decrease corticosteroid dose in chronic disease, immunosuppressants such as methotrexate, azathioprine, mycophenolate mofetil, and cyclosporine may be used. Among the biologics, rituximab, dupilumab, omalizumab, and intravenous immunoglobulins may be used.

• #6 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  If the pemphigoid is very widespread, hospital admission may be arranged to dress blisters and erosions. […] Medical treatment involves: ultrapotent topical steroids to treat limited disease cream, moderate potency topical steroids and emollients to relieve itch and dryness, systemic steroids (eg, prednisone) they are however associated with an increased risk of adverse and potentially fatal side effects, tetracycline antibiotics, usually doxycycline 200 mg/day; doxycycline has fewer adverse effects than oral corticosteroids and is effective on its own for mild disease, other steroid-sparing medications on their own or in combination with steroids, antibiotics for secondary bacterial infection, pain relief. […] Most patients with bullous pemphigoid are treated with steroid tablets, either prednisone or prednisolone at an initial dose of 0.5 mg/kg/day. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced once there are fewer than 3 significant blisters, over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5-10 mg prednisone daily). These other medications may include doxycycline or: dapsone, nicotinamide, methotrexate, azathioprine, mycophenolate, intravenous immunoglobulin, rituximab, dupilumab, omalizumab.

• #6

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Eosinophil infiltration is a common characteristic in BP skin lesions, and these cells are attracted by various chemokines present in blister fluid, such as IL-5, eotaxin, and galectin-9. […] Targeting eosinophils holds promise as a potential treatment strategy for BP. […] Bertilimumab is a humanized monoclonal antibody targeting eotaxin-1 (CCL-11). […] The molecule was also well tolerated, indicating that bertilimumab is a well tolerated and efficacious treatment for BP. […] Omalizumab is a high affinity monoclonal antibody against the C3 domain of IgE, blocking its interaction with the specific FcRI receptor, leading to a reduction of total IgE levels and eosinophilia. […] Multiple case reports have shown that omalizumab effectively reduced the severity of BP by diminishing itching and blister counts. […] A recent multicenter, retrospective study conducted in France involved 100 patients with BP who were treated with omalizumab after failing one or multiple lines of treatment. The study revealed that 77% of patients achieved complete remission.

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

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

  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.

• #7 Biological treatment for bullous pemphigoid

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10172582/

  Efficient and safe novel therapies can be considered in recalcitrant steroid-dependent BP non-responsive to conventional immunosuppressant therapies. […] Cumulative data on biological therapies, such as rituximab (RTX) and omalizumab, indicate their clinical benefits in patients with BP. In addition, emerging data on BP treatment with dupilumab (DUPI) indicate that it may be an additional immunomodulatory treatment that efficiently controls disease while maintaining a steroid-sparing effect, further expanding the limited existing armamentarium. […] Patients were treated non-exclusively with various biologic therapies, including RTX, omalizumab, and DUPI, without any enforced washout period. […] Clinical response was assessed using the degree of skin lesions and pruritus, according to the recommendations of an international panel of experts for outcome measures in bullous pemphigoid.

• #8 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  If the pemphigoid is very widespread, hospital admission may be arranged to dress blisters and erosions. […] Medical treatment involves: ultrapotent topical steroids to treat limited disease cream, moderate potency topical steroids and emollients to relieve itch and dryness, systemic steroids (eg, prednisone) they are however associated with an increased risk of adverse and potentially fatal side effects, tetracycline antibiotics, usually doxycycline 200 mg/day; doxycycline has fewer adverse effects than oral corticosteroids and is effective on its own for mild disease, other steroid-sparing medications on their own or in combination with steroids, antibiotics for secondary bacterial infection, pain relief. […] Most patients with bullous pemphigoid are treated with steroid tablets, either prednisone or prednisolone at an initial dose of 0.5 mg/kg/day. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced once there are fewer than 3 significant blisters, over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5-10 mg prednisone daily). These other medications may include doxycycline or: dapsone, nicotinamide, methotrexate, azathioprine, mycophenolate, intravenous immunoglobulin, rituximab, dupilumab, omalizumab.

• #8

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Eosinophil infiltration is a common characteristic in BP skin lesions, and these cells are attracted by various chemokines present in blister fluid, such as IL-5, eotaxin, and galectin-9. […] Targeting eosinophils holds promise as a potential treatment strategy for BP. […] Bertilimumab is a humanized monoclonal antibody targeting eotaxin-1 (CCL-11). […] The molecule was also well tolerated, indicating that bertilimumab is a well tolerated and efficacious treatment for BP. […] Omalizumab is a high affinity monoclonal antibody against the C3 domain of IgE, blocking its interaction with the specific FcRI receptor, leading to a reduction of total IgE levels and eosinophilia. […] Multiple case reports have shown that omalizumab effectively reduced the severity of BP by diminishing itching and blister counts. […] A recent multicenter, retrospective study conducted in France involved 100 patients with BP who were treated with omalizumab after failing one or multiple lines of treatment. The study revealed that 77% of patients achieved complete remission.

• #9 Can Methotrexate Be Employed as Monotherapy for Bullous Pemphigoid? Analysis of Efficiency and Tolerance of Methotrexate Treatment in Patients with Bullous Pemphigoid

  https://www.mdpi.com/2077-0383/12/4/1638

  The European Academy of Dermatology and Venerology (EADV) consensus states that the treatment of choice for bullous pemphigoid is systemic glucocorticosteroid therapy. […] An effective and safer treatment regimen for these patients is still being sought. […] The treatment of bullous pemphigoid with methotrexate in monotherapy is an effective and safe therapeutic method for elderly patients. […] Methotrexate is an antimetabolic drug that acts as an antagonist of folate. […] Methotrexate therapy seems well tolerated, Kjellman et al. suggest increasing the dosage as a safer alternative to systemic steroid therapy. […] Our findings indicate that patients with BP should be treated with MTX in monotherapy. […] MTX therapy is effective and safe, both when applied alone and with low doses of steroids.

• #9

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  BP treatment can be very challenging as BP is a chronic disease requiring long-term management. Glucocorticoids are the cornerstone of BP treatment. High-dose systemic corticosteroids are used to induce remission, followed by gradual tapering. […] Corticosteroid-sparing adjuvant agents are prescribed when monotherapy with glucocorticoids is inadequate to achieve disease control or when there is a relapse during corticosteroid tapering. […] The aim of this review was to summarize and discuss the novel targeted BP treatment options that have been developed and tested in clinical trials in recent years, based on the complex pathophysiology of BP. […] Rituximab therapy usually results in a major reduction in circulating BP180-specific B lymphocytes, as well as a dramatic reduction of anti-BP180 antibodies, and decreases expression of proinflammatory IL-15 and IL-6, leading to an improvement of BP skin manifestations. […] Dupilumab may further improve pruritus in BP by directly reducing IL-13 and by indirectly downregulating IL-31 production by eosinophils. […] A case series involving 13 BP patients from five academic centers receiving dupilumab, 92.3% (12/13) of the patients achieved disease clearance or satisfactory response.

• #10 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  If the pemphigoid is very widespread, hospital admission may be arranged to dress blisters and erosions. […] Medical treatment involves: ultrapotent topical steroids to treat limited disease cream, moderate potency topical steroids and emollients to relieve itch and dryness, systemic steroids (eg, prednisone) they are however associated with an increased risk of adverse and potentially fatal side effects, tetracycline antibiotics, usually doxycycline 200 mg/day; doxycycline has fewer adverse effects than oral corticosteroids and is effective on its own for mild disease, other steroid-sparing medications on their own or in combination with steroids, antibiotics for secondary bacterial infection, pain relief. […] Most patients with bullous pemphigoid are treated with steroid tablets, either prednisone or prednisolone at an initial dose of 0.5 mg/kg/day. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced once there are fewer than 3 significant blisters, over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5-10 mg prednisone daily). These other medications may include doxycycline or: dapsone, nicotinamide, methotrexate, azathioprine, mycophenolate, intravenous immunoglobulin, rituximab, dupilumab, omalizumab.

• #10 Treatment of Bullous Pemphigoid With Dupilumab: A Case Series of 30 Patients – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/treatment-of-bullous-pemphigoid-with-dupilumab-a-case-series-of-30-patients-S1545961624Pe144X/

  Bullous pemphigoid is often difficult to treat with the limited therapies available. Here, we describe clinical outcomes among 30 adults with bullous pemphigoid patients treated with dupilumab. Patients received a loading dose of dupilumab 600 mg, followed by 300 mg maintenance dose with varying administration frequency tailored to individual patient response. All patients experienced at least some improvement in blister formation and pruritus, with 23 (76.7%) of patients demonstrating either complete clearance of blistering or marked response. Complete clearance of pruritus or marked response was noted in 25 (83.3%) of patients. Eight patients were effectively maintained solely on dupilumab. […] Dupilumab should be considered as a therapeutic option in patients with bullous pemphigoid given its ability to induce sustained blistering and pruritus response in both typical and refractory cases while maintaining a favorable safety profile.

• #11 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Bullous pemphigoid (BP) is an autoimmune blistering disease in which autoantibodies are directed against components of the basement membrane. […] In recent years, advances in clinical practice have led to a better understanding and improved management of this disorder. […] This article is an updated review of the scientific literature on the treatment of BP. It focuses primarily on evidence-based recommendations and is written from a practical standpoint based on experience in the routine management of this disease. […] Three types of drugs, with distinct mechanisms of action, are used to treat BP: […] Anti-inflammatory drugs such as topical corticosteroids, sulfone, sulfamides, or antibiotics with anti-inflammatory properties such as tetracyclines […] Drugs designed to reduce the production of pathogenic antibodies, such as systemic corticosteroids, azathioprine, mycophenolate, cyclophosphamide, methotrexate, ciclosporin, and rituximab

• #11 Treatment of Bullous Pemphigoid With Dupilumab: A Case Series of 30 Patients – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/treatment-of-bullous-pemphigoid-with-dupilumab-a-case-series-of-30-patients-S1545961624Pe144X/

  Bullous pemphigoid is often difficult to treat with the limited therapies available. Here, we describe clinical outcomes among 30 adults with bullous pemphigoid patients treated with dupilumab. Patients received a loading dose of dupilumab 600 mg, followed by 300 mg maintenance dose with varying administration frequency tailored to individual patient response. All patients experienced at least some improvement in blister formation and pruritus, with 23 (76.7%) of patients demonstrating either complete clearance of blistering or marked response. Complete clearance of pruritus or marked response was noted in 25 (83.3%) of patients. Eight patients were effectively maintained solely on dupilumab. […] Dupilumab should be considered as a therapeutic option in patients with bullous pemphigoid given its ability to induce sustained blistering and pruritus response in both typical and refractory cases while maintaining a favorable safety profile.

• #12 Bullous pemphigoid – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/bullous-pemphigoid/

  The goal of therapy is to decrease blister formation, promote healing of blisters and erosions, and achieve the minimal dose necessary to control the disease process. Therapy must be individualized for each patient, keeping in mind the pre-existing conditions and other patient-specific factors. […] Localized BP often can be treated successfully with topical steroids alone. More extensive disease, which is often more difficult to control, is usually treated with systemic anti-inflammatory and immunosuppressive agents, oral corticosteroids being the mainstay of treatment. Oral prednisone/prednisolone doses range from 0.3 to 1.25 mg/kg body weight/day, which usually controls disease within 1-2 weeks; the dose is then progressively tapered. […] Several studies have suggested the use of concomitant immunosuppressive agents to achieve a corticosteroid-sparing effect.

• #12 Dupilumab Successfully Treated Bullous Pemphigoid in Retrospective Study

  https://www.dermatologytimes.com/view/dupilumab-successfully-treated-bullous-pemphigoid-in-retrospective-study

  Few adverse events resulted from treatment with dupilumab in 64 weeks of observation. […] In a retrospective cohort study of patients with bullous pemphigoid (BP), researchers found that dupilumab resulted in a reduction of symptoms within 4 weeks. […] The patients were treated with an initial 600 mg dose of dupilumab, followed by 300 mg of dupilumab every 2 weeks. […] After 4 weeks of treatment with dupilumab, 87% achieved disease control and 74.7% of those occurred within 2 weeks. […] Complete remission was achieved by 35.6% of patients during the observation period. […] Researchers concluded that dupilumab was an effective treatment for BP with minimal AEs.

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

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

  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.

• #13 Bullous pemphigoid successfully treated with dupilumab | Anais Brasileiros de Dermatologia

  https://www.anaisdedermatologia.org.br/en-bullous-pemphigoid-successfully-treated-with-articulo-S0365059624001156

  Bullous pemphigoid (BP), a chronic autoimmune cutaneous blistering disorder affecting predominantly the elderly, is characterized by skin tense bullae formation and pruritus symptoms. At present, the main treatment options are represented by corticosteroids and immunosuppressant drugs. Steroids often need to be administered in high doses, with subsequent adverse events and safety issues. Safer treatment modalities are therefore needed. Dupilumab is a biologic agent used to treat BP in recent years. Here, we describe an elderly patient with recalcitrant BP successfully treated with dupilumab. […] Dupilumab is a recently developed monoclonal antibody that blocks the signaling of IL-4 and IL-13, both of which are crucial cytokines in the T2 response. For this reason, the hypothesis is that the reduction in disease activity obtained in the cases reported so far may be related to the reduction in Th2-type responses induced by the inhibition of IL-4 and IL-13 signal transduction induced by dupilumab. The European Academy of Dermatology and Venereology already considers dupilumab as an optional treatment for refractory BP.

• #14

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Complement activation plays a crucial role in the pathogenesis of BP. […] Nomacopan (rVA576) is a C5 and leukotriene B4 inhibitor. […] An ongoing, randomized, double blind, placebo-controlled, phase III trial aims at enrolling 148 patients to evaluate the efficacy of nomacopan in BP. […] In previous in vitro studies, IL-17 enhanced eosinophilia. […] The administration of secukinumab has been found to decrease circulating anti-BP180 antibodies. […] A case report of an 85-year-old female patient with severe BP, adjuvant secukinumab therapy in addition to standard therapy with prednisolone led to a long lasting and complete remission. […] Janus kinase (JAK) inhibitors are presently employed in the treatment of conditions such as rheumatoid arthritis and atopic dermatitis, and they hold promise for managing various other inflammatory skin conditions. […] A recent case report highlighted a 33-year-old man diagnosed with BP and concurrent psoriasis vulgaris who was treated with tofacitinib, a JAK inhibitor.

• #15

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Complement activation plays a crucial role in the pathogenesis of BP. […] Nomacopan (rVA576) is a C5 and leukotriene B4 inhibitor. […] An ongoing, randomized, double blind, placebo-controlled, phase III trial aims at enrolling 148 patients to evaluate the efficacy of nomacopan in BP. […] In previous in vitro studies, IL-17 enhanced eosinophilia. […] The administration of secukinumab has been found to decrease circulating anti-BP180 antibodies. […] A case report of an 85-year-old female patient with severe BP, adjuvant secukinumab therapy in addition to standard therapy with prednisolone led to a long lasting and complete remission. […] Janus kinase (JAK) inhibitors are presently employed in the treatment of conditions such as rheumatoid arthritis and atopic dermatitis, and they hold promise for managing various other inflammatory skin conditions. […] A recent case report highlighted a 33-year-old man diagnosed with BP and concurrent psoriasis vulgaris who was treated with tofacitinib, a JAK inhibitor.

• #16 Successful induction treatment of bullous pemphigoid using reslizumab: a case report | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-021-00619-1

  Bullous pemphigoid (BP) is a potentially life-threatening autoimmune blistering disease which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. […] Topical and systemic steroid (methylprednisolone 2 mg/kg/day) treatment was started, and his skin symptoms worsened repeatedly, whenever systemic steroid were reduced. […] On admission day 29, reslizumab (anti-interleukin-5) 3.5 mg/kg was administered intravenously to the patients. The bullous skin lesion began to improve rapidly, and methylprednisolone (8 mg/day) was reduced without any worsening of symptoms during two doses of reslizumab. […] We report a case of successful treatment response to reslizumab administration in a patient with BP. Further studies are needed to confirm the role of anti-interleukin-5 as a treatment for BP in the future.

• #17 Successful induction treatment of bullous pemphigoid using reslizumab: a case report | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-021-00619-1

  Topical and systemic corticosteroid treatment is the mainstay for management of BP. Adjuvant immunosuppressive drugs such as methotrexate, azathioprine and cyclophosphamide are indicated for patients who need high doses corticosteroids to control disease or who have adverse effects on corticosteroid treatment. […] In recent years, biologics such as rituximab and omalizumab may be effective to BP with better safety profile than classical immunosuppressive drugs. […] The patient in this case had BP that was refractory to high-dose systemic steroid therapy and methotrexate, and there were the potential risks of infections for using classical immunosuppressive adjuvant drugs. […] However, blood eosinophilia and the patients symptoms improved rapidly from the day after reslizumab administration, and re-exacerbation of the skin lesion was noted after discontinuation of reslizumab. […] This is a case of successful treatment of BP using anti-IL-5 antibody (reslizumab). Future research is warranted to confirm the role of biologics such as anti-IL5 as a therapeutic option for BP, particularly with regard to the steroid-sparing effect.

• #18

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Eosinophil infiltration is a common characteristic in BP skin lesions, and these cells are attracted by various chemokines present in blister fluid, such as IL-5, eotaxin, and galectin-9. […] Targeting eosinophils holds promise as a potential treatment strategy for BP. […] Bertilimumab is a humanized monoclonal antibody targeting eotaxin-1 (CCL-11). […] The molecule was also well tolerated, indicating that bertilimumab is a well tolerated and efficacious treatment for BP. […] Omalizumab is a high affinity monoclonal antibody against the C3 domain of IgE, blocking its interaction with the specific FcRI receptor, leading to a reduction of total IgE levels and eosinophilia. […] Multiple case reports have shown that omalizumab effectively reduced the severity of BP by diminishing itching and blister counts. […] A recent multicenter, retrospective study conducted in France involved 100 patients with BP who were treated with omalizumab after failing one or multiple lines of treatment. The study revealed that 77% of patients achieved complete remission.

• #19

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Complement activation plays a crucial role in the pathogenesis of BP. […] Nomacopan (rVA576) is a C5 and leukotriene B4 inhibitor. […] An ongoing, randomized, double blind, placebo-controlled, phase III trial aims at enrolling 148 patients to evaluate the efficacy of nomacopan in BP. […] In previous in vitro studies, IL-17 enhanced eosinophilia. […] The administration of secukinumab has been found to decrease circulating anti-BP180 antibodies. […] A case report of an 85-year-old female patient with severe BP, adjuvant secukinumab therapy in addition to standard therapy with prednisolone led to a long lasting and complete remission. […] Janus kinase (JAK) inhibitors are presently employed in the treatment of conditions such as rheumatoid arthritis and atopic dermatitis, and they hold promise for managing various other inflammatory skin conditions. […] A recent case report highlighted a 33-year-old man diagnosed with BP and concurrent psoriasis vulgaris who was treated with tofacitinib, a JAK inhibitor.

• #20 Bullous Pemphigoid Treatment & Management: Medical Care, Complications, Diet

  https://emedicine.medscape.com/article/1062391-treatment

  Several studies found initial treatment with doxycycline to be effective and to be associated with a lower incidence of adverse effects as compared with prednisolone. […] Proper treatment of bullous pemphigoid depends on the severity of the disease. For localized disease, a topical steroid plus a systemic anti-inflammatory (eg, tetracycline or nicotinamide) may be sufficient. The effects of monotherapy with nicotinamide are unknown. For more severe cases, systemic steroids along with immunosuppressants may be needed for control. If the disease proves difficult to control, it is worthwhile to consider treatment with the anti-CD20 antibody rituximab, which is relatively specific in targeting the antibody-producing B cells. […] Some case reports have suggested that Janus kinase (JAK) inhibitors (eg, tofacitinib, baricitinib, upadacitinib, and abrocitinib) may prove useful for treatment of bullous pemphigoid.

• #21

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  Complement activation plays a crucial role in the pathogenesis of BP. […] Nomacopan (rVA576) is a C5 and leukotriene B4 inhibitor. […] An ongoing, randomized, double blind, placebo-controlled, phase III trial aims at enrolling 148 patients to evaluate the efficacy of nomacopan in BP. […] In previous in vitro studies, IL-17 enhanced eosinophilia. […] The administration of secukinumab has been found to decrease circulating anti-BP180 antibodies. […] A case report of an 85-year-old female patient with severe BP, adjuvant secukinumab therapy in addition to standard therapy with prednisolone led to a long lasting and complete remission. […] Janus kinase (JAK) inhibitors are presently employed in the treatment of conditions such as rheumatoid arthritis and atopic dermatitis, and they hold promise for managing various other inflammatory skin conditions. […] A recent case report highlighted a 33-year-old man diagnosed with BP and concurrent psoriasis vulgaris who was treated with tofacitinib, a JAK inhibitor.

• #22

  https://journals.lww.com/ders/fulltext/2023/41030/successful_treatment_of_bullous_pemphigoid_with.10.aspx

  A 64-year-old Taiwanese male, nonsmoker, with a history of hypertension and type 2 diabetes mellitus has been regularly taking metformin for glycemic control. The patient was previously diagnosed with biopsy-proven bullous pemphigoid after experiencing multiple itchy erythema and blisters all over the whole body for 3 months. He was treated with prednisolone 10 mg three times daily and azathioprine 25 mg daily. Lesions showed improvement during the first 2-week follow-up. However, while the prednisolone dose was tapered down to 10 mg daily, the disease flared up again. […] Initially, we treated the patient with intravenous methylprednisolone 4 mg/day, doxycycline 100 mg twice daily, systemic antihistamines, and topical steroids. However, we observed hyperglycemia, with poor-controlled blood sugar levels increasing up to 480 mg/dL. After discussing the options with the patient, we prescribed oral baricitinib 4 mg/day. Subsequently, we initiated the tapering of the systemic steroid in 2 days. At 1-week follow-up, the erythema on the body has disappeared and no new blisters have formed. The pruritus has also subsided, leaving only the wounds from the ruptured blisters.

• #23

  https://journals.lww.com/ders/fulltext/2023/41030/successful_treatment_of_bullous_pemphigoid_with.10.aspx

  A 64-year-old Taiwanese male, nonsmoker, with a history of hypertension and type 2 diabetes mellitus has been regularly taking metformin for glycemic control. The patient was previously diagnosed with biopsy-proven bullous pemphigoid after experiencing multiple itchy erythema and blisters all over the whole body for 3 months. He was treated with prednisolone 10 mg three times daily and azathioprine 25 mg daily. Lesions showed improvement during the first 2-week follow-up. However, while the prednisolone dose was tapered down to 10 mg daily, the disease flared up again. […] Initially, we treated the patient with intravenous methylprednisolone 4 mg/day, doxycycline 100 mg twice daily, systemic antihistamines, and topical steroids. However, we observed hyperglycemia, with poor-controlled blood sugar levels increasing up to 480 mg/dL. After discussing the options with the patient, we prescribed oral baricitinib 4 mg/day. Subsequently, we initiated the tapering of the systemic steroid in 2 days. At 1-week follow-up, the erythema on the body has disappeared and no new blisters have formed. The pruritus has also subsided, leaving only the wounds from the ruptured blisters.

• #24 Bullous pemphigoid – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/bullous-pemphigoid/

  The overall response rate to dapsone, when given either alone or in combination with corticosteroids or immunosuppressive agents, is about 81% in BP. […] Up to 24% of patients with BP do not respond to conventional therapy. Other drugs for treating BP include biologicals (anti-TNF drugs, rituximab), IVIg and plasma exchange. […] IVIg appears to be an effective alternative in treating patients with severe BP whose disease is nonresponsive to conventional therapy or who are at risk of experiencing serious or potentially fatal side effects from conventional immunosuppressive therapy. […] Among all the treatment modalities in India, the most commonly used is steroids, either oral or in the form of pulse therapy. Non-steroidal immunosuppressive drugs are added as adjuvants to increase the efficacy and to have a steroid sparing effect.

• #25 Successful Treatment of Bullous Pemphigoid Lesions by Berberine Stamp Therapy: A Case Report and Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9283775/

  Mild BP should be treated with topical corticosteroids alone, while moderate BP should be treated with other adjuvant therapies in combination with topical corticosteroids, and severe BP should be treated with systemic corticosteroids. […] In addition to hormones, drugs such as tetracycline and nicotinamide, azathioprine, doxycycline, mycophenolate mofetil, and dapsone are often used as adjuvant or alternative treatments. […] Furthermore, according to Engineer et al., intravenous immunoglobulin is an alternative hormone therapy worth investigating because it is effective in the clinic, particularly in resistant BP, with minimal adverse reactions. […] In addition to Western medicine treatment, traditional Chinese medicine treatment is currently receiving increasing attention, and its efficacy is considerable. […] In this case, we chose berberine primarily based on traditional Chinese medicines basic theory and syndrome differentiation for patients. […] The advantages of traditional Chinese medicine and external treatment to treat skin diseases are becoming increasingly apparent.

• #26 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  The efficacy of systemic corticosteroids has been demonstrated in several studies and is supported by extensive clinical experience. […] In patients who respond only partially to corticosteroid therapy or who develop adverse effects, nonimmunosuppressive agents such as tetracyclines, erythromycin, nicotinamide, and sulfone can be used as adjuvant therapy, especially in patients with mild or moderate disease. […] Immunosuppressive agents are indicated for patients with moderate or severe disease in whom high doses of corticosteroids fail to control the disease or in whom effective doses cannot be maintained due to adverse effects. […] Rituximab must be administered in an inpatient or day-hospital setting. Its toxicity and associated mortality are not negligible, particularly in relation to the risk of serious opportunistic or other infections. […] Therapeutic plasma exchange is a costly technique that is not available in most hospitals. […] The technique involves substituting a considerable volume of the patient’s blood with replacement plasma.

• #27 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Therapeutic plasma exchange is thus an exceptional treatment that is rarely used in BP. It should be reserved for patients with acute, severe disease who have high levels of circulating antibodies and contraindications for other treatments. […] The latest update on interventions for BP published in The Cochrane library in 2010 included 10 randomized controlled studies of the treatment of BP.

• #28

  https://www.healio.com/news/dermatology/20240906/lowdose-il2-promising-in-bullous-pemphigoid-treatment

  Patients with bullous pemphigoid treated with IL-2 had fewer days to disease control vs. those on corticosteroids alone. […] A low dose of interleukin-2 therapy showed positive results in the treatment of bullous pemphigoid with an early onset of response, according to a study. […] This perspective, comparator-controlled clinical trial evaluated if a low dose of IL-2 (Recombinant Human Interleukin-2 Injection, Beijing Sihuan Biopharmaceutical Co.) was efficacious in the treatment of BP. […] Disease control was achieved in 7.6 3 days in the treatment group, compared with 10.43 3.06 days in the control group (P = .008). […] After 1 week of treatment, 60.9% of patients achieved disease control compared with 17.6% of those in the control group (P = .01), and a greater reduction in Bullous Pemphigoid Disease Area Index scores was recorded in the IL-2 treatment group (P = .003).

• #29

  https://www.healio.com/news/dermatology/20240906/lowdose-il2-promising-in-bullous-pemphigoid-treatment

  The treatment group also needed a smaller dose of corticosteroids to reach disease control. […] Our results suggest that low-dose IL-2 therapy is promising for its early onset of response, especially in the first 2 weeks, the authors wrote. We speculate that the rapid increase of Treg cells in the first week was beneficial for disease control and early steroids reduction.

• #30 Successful Treatment of Bullous Pemphigoid Lesions by Berberine Stamp Therapy: A Case Report and Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9283775/

  Bullous pemphigoid (BP) is a life-threatening autoimmune disease of the skin that is mainly characterized by a large range of tension blisters and intense itching of the skin. […] The patient was successfully treated with hormones combined with topical berberine, and 95% of the patients lesions healed completely after 1 month. […] Based on clinical experience, we used steroids combined with topical berberine and achieved highly positive outcomes. […] Berberine is primarily derived from Coptis chinensis Franch and is typically administered in clinical practice as berberine hydrochloride tablets. […] The usage of stamp therapy for wound care also protected the patients large area of blisters and ulcerating, and eroding wounds from serious infection and complications. […] At present, the treatment of BP is mainly glucocorticoids supplemented by antibiotics or steroids.

• #31 Successful Treatment of Bullous Pemphigoid Lesions by Berberine Stamp Therapy: A Case Report and Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9283775/

  Mild BP should be treated with topical corticosteroids alone, while moderate BP should be treated with other adjuvant therapies in combination with topical corticosteroids, and severe BP should be treated with systemic corticosteroids. […] In addition to hormones, drugs such as tetracycline and nicotinamide, azathioprine, doxycycline, mycophenolate mofetil, and dapsone are often used as adjuvant or alternative treatments. […] Furthermore, according to Engineer et al., intravenous immunoglobulin is an alternative hormone therapy worth investigating because it is effective in the clinic, particularly in resistant BP, with minimal adverse reactions. […] In addition to Western medicine treatment, traditional Chinese medicine treatment is currently receiving increasing attention, and its efficacy is considerable. […] In this case, we chose berberine primarily based on traditional Chinese medicines basic theory and syndrome differentiation for patients. […] The advantages of traditional Chinese medicine and external treatment to treat skin diseases are becoming increasingly apparent.

• #32 Bullous pemphigoid: Diagnosis and treatment

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

  Other medications are also prescribed to treat bullous pemphigoid. […] Tetracycline, doxycycline, and dapsone are antibiotics, which can reduce the inflammation inside your body. […] Some patients take a medication called an immunosuppressant. This medication helps to calm the immune system. […] Wound care: Open blisters and raw skin can become infected. Wound care can prevent infection, relieve pain, and speed up healing. […] Your dermatologist will create a wound care plan that meets your individual needs. […] The amount of time you need to treat bullous pemphigoid will depend on how severe the disease is and your response to treatment. […] Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. […] Following a self-care plan for bullous pemphigoid can improve the results you get from treatment and how well you feel.

• #33 Bullous Pemphigoid: Know the Facts

  https://www.webmd.com/skin-problems-and-treatments/what-is-bullous-pemphigoid

  Blisters and open wounds must be taken care of to prevent infection. Ask your doctor how best to do this. Many products are on the market to use as dressings to protect the wounds, ranging from gauze that has petroleum jelly in it (to prevent sticking) to special foam dressings. The important thing is not to use a dry dressing that will stick to the wound or blister when it gets dry. It will become hard to remove and could cause more injury to the skin. […] If the blisters are widespread and severe enough, you might need specialized wound care in a hospital burn unit, where the staff has special expertise in wound care. […] It can take several years for bullous pemphigoid to go away completely. In the meantime, your doctor will monitor your progress to see how the treatment is working, and may switch drugs if needed. Some medications can have serious side effects, so report anything unusual to your doctor. You might need regular tests, like a bone density (DEXA) scan, blood pressure monitoring, or blood tests to make sure that you aren’t having any hidden side effects.

• #34 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Bullous pemphigoid is an uncommon blistering condition of the elderly, which often starts with itch and urticated and erythematous lesions. Later, large tense blisters develop on both erythematous and on normal skin and there may be mucosal involvement with blisters and erosions. The blisters are subepidermal. Bullous pemphigoid is the most commonly seen autoimmune blistering disease in the West with a reported incidence in the UK of 4.3 per 100,000 persons per year. […] The vast majority of patients need urgent referral to Secondary Care, and those with widespread blistering may require admission. A few patients, eg those with localised disease, may be suitable for management in Primary Care. Provide a patient information leaflet on pemphigoid. Supportive measures include wound care and reducing the risk of secondary infection by using antiseptic regimes, eg Dermol 500 lotion as a wash and / or topical emollient.

• #35 Bullous Pemphigoid: Symptoms and Treatment | Doctor

  https://patient.info/doctor/bullous-pemphigoid-pro

  Available data suggest that rituximab may provide clinical benefits for patients with refractory bullous pemphigoid. […] Bacterial superinfection of erosions should be treated with local antiseptics. Wound dressings should be considered for large wounds. Sterile puncture of large blisters is recommended.

• #36 Bullous pemphigoid Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/bullous-pemphigoid

  Anti-inflammatory medicines called corticosteroids may be prescribed. They may be taken by mouth or applied to the skin. More powerful medicines may be used to help suppress the immune system if steroids do not work, or to allow lower steroid doses to be used. […] Antibiotics in the tetracycline family may be useful. Niacin (a B complex vitamin) is sometimes given along with tetracycline. […] Your provider may suggest self-care measures. These may include: […] Applying anti-inflammatory creams to the skin […] Using mild soaps and applying moisturizer to the skin after bathing […] Protecting the affected skin from sun exposure and injury. […] Bullous pemphigoid usually responds well to treatment. The medicine can often be stopped after several years. The disease sometimes returns after treatment is stopped.

• #37 Bullous pemphigoid: Self-care

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

  Taking good care of yourself at home may help blisters caused by bullous pemphigoid to clear more quickly. Good care can also prevent a serious infection. Heres what dermatologists recommend for their patients who have bullous pemphigoid. […] It can take several weeks for blisters to start drying up and new blisters to stop forming. When you dont see results right away, you may be tempted to stop following your treatment plan. Continue with your treatment plan, making sure that you: […] For more information about treatment, go to Bullous pemphigoid: Diagnosis and treatment. […] If your skin looks infected, immediately call your dermatologist. Treating an infection early can prevent it from spreading and causing a life-threatening illness.

• #38 Bullous Pemphigoid: Causes, Symptoms, and Treatment

  https://patient.info/skin-conditions/bullous-pemphigoid-leaflet

  Bullous pemphigoid is a skin disease that can cause a rash, itching and blisters. Treatment usually works well to control symptoms. Treatment is usually with steroid creams or medicines, but sometimes other medicines may be used. […] Treatment aims to improve your symptoms and avoid side effects. Treatments used for bullous pemphigoid are: […] Steroids are the main treatment for bullous pemphigoid. High-strength steroid creams, also called topical steroids (for example, clobetasol) will normally be needed. Steroid tablets such as prednisolone are commonly used with or without steroid creams, especially if the rash is on more than one body area, or there are practical problems applying the cream. Steroids reduce inflammation and suppress the immune system. […] Other drugs to calm the immune system may be used if you cannot use steroids or they are not working to control your condition. […] Bullous pemphigoid usually goes away after 3-6 years. Treatment can then be stopped. In some people the condition unfortunately persists.

• #39 Bullous pemphigoid – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/symptoms-causes/syc-20350414

  Bullous pemphigoid often goes away on its own in a few months, but it may take as many as five years to go away for good. Treatment usually helps heal the blisters and prevent new ones from forming. […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Possible complications of bullous pemphigoid include side effects from the medicine used to treat bullous pemphigoid.

• #40 Bullous pemphigoid: Diagnosis and treatment

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

  Other medications are also prescribed to treat bullous pemphigoid. […] Tetracycline, doxycycline, and dapsone are antibiotics, which can reduce the inflammation inside your body. […] Some patients take a medication called an immunosuppressant. This medication helps to calm the immune system. […] Wound care: Open blisters and raw skin can become infected. Wound care can prevent infection, relieve pain, and speed up healing. […] Your dermatologist will create a wound care plan that meets your individual needs. […] The amount of time you need to treat bullous pemphigoid will depend on how severe the disease is and your response to treatment. […] Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. […] Following a self-care plan for bullous pemphigoid can improve the results you get from treatment and how well you feel.

• #41 Bullous Pemphigoid: What It Is and How to Treat It | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/patient-education/bullous-pemphigoid-what-it-and-how-treat-it

  Your healthcare provider may also suggest applying dressings. They will tell you how to care for your open skin. […] If steroids dont help your symptoms, your healthcare provider may treat you with other medications. They include Dapsone, Sulfonamides (BactrimTM), or Rituximab (Rituxan ) for more severe cases. These medications are not used as often because they have more side effects. […] With treatment, bullous pemphigoid often goes away in 1 to 5 years. Its possible to get it again, but its usually less severe.

• #42 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Treatments that increase the elimination of pathogenic antibodies from the serum of patients, such as high-dose intravenous immunoglobulin (IVIG) therapy and therapeutic plasma exchange. […] Although there are no consensus guidelines on the management of bullous pemphigoid, when deciding on a treatment approach, it is important to remember that BP is associated with high morbidity and affects mainly elderly patients. […] It is generally preferable to choose drugs with the lowest toxicity possible and to target the inflammatory component of the disease. […] In the case of localized lesions, high-potency topical corticosteroids may be sufficient to control the disease and would therefore be treatment of choice. […] High-potency topical corticosteroids are particularly indicated in patients with mild or moderate symptoms.

• #43 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Treatments that increase the elimination of pathogenic antibodies from the serum of patients, such as high-dose intravenous immunoglobulin (IVIG) therapy and therapeutic plasma exchange. […] Although there are no consensus guidelines on the management of bullous pemphigoid, when deciding on a treatment approach, it is important to remember that BP is associated with high morbidity and affects mainly elderly patients. […] It is generally preferable to choose drugs with the lowest toxicity possible and to target the inflammatory component of the disease. […] In the case of localized lesions, high-potency topical corticosteroids may be sufficient to control the disease and would therefore be treatment of choice. […] Topical or systemic corticosteroids are generally the first line of treatment in BP. High-potency topical corticosteroids are particularly indicated in patients with mild or moderate symptoms.

• #44

  https://link.springer.com/article/10.1007/s40257-023-00832-1

  The complex pathophysiology of BP indicates that the antibody-antigen binding activates simultaneously different molecular pathways, rather than a single cascade. […] The number of available therapeutic options is rapidly increasing, allowing us to move past the era of high-dose systemic corticosteroids or corticosteroid-sparing agents for the treatment of BP.

• #45 Pemphigus and Pemphigoid Signs & Symptoms | Rush

  https://www.rush.edu/conditions/pemphigus-and-pemphigoid

  When you choose Rush for pemphigus or pemphigoid care, you’ll receive customized treatments that follow the latest scientific guidelines and fit your personal needs and goals. For example, research shows we should give certain patients a high dose of steroids right away, instead of several rounds of lower-dose steroids over time. This helps us get the disease under control (and taper you off steroids) more quickly. And it can prevent the side effects that may occur if you take steroids over a long period of time. We also work with you to make sure your treatments fit your lifestyle. For example, if your schedule makes it hard to have infusion treatments several times a month, we can find other medicines that are equally (or nearly as) effective.

• #46 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Treatments that increase the elimination of pathogenic antibodies from the serum of patients, such as high-dose intravenous immunoglobulin (IVIG) therapy and therapeutic plasma exchange. […] Although there are no consensus guidelines on the management of bullous pemphigoid, when deciding on a treatment approach, it is important to remember that BP is associated with high morbidity and affects mainly elderly patients. […] It is generally preferable to choose drugs with the lowest toxicity possible and to target the inflammatory component of the disease. […] In the case of localized lesions, high-potency topical corticosteroids may be sufficient to control the disease and would therefore be treatment of choice. […] High-potency topical corticosteroids are particularly indicated in patients with mild or moderate symptoms.

• #47 Bullous pemphigoid – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/bullous-pemphigoid/

  The goal of therapy is to decrease blister formation, promote healing of blisters and erosions, and achieve the minimal dose necessary to control the disease process. Therapy must be individualized for each patient, keeping in mind the pre-existing conditions and other patient-specific factors. […] Localized BP often can be treated successfully with topical steroids alone. More extensive disease, which is often more difficult to control, is usually treated with systemic anti-inflammatory and immunosuppressive agents, oral corticosteroids being the mainstay of treatment. Oral prednisone/prednisolone doses range from 0.3 to 1.25 mg/kg body weight/day, which usually controls disease within 1-2 weeks; the dose is then progressively tapered. […] Several studies have suggested the use of concomitant immunosuppressive agents to achieve a corticosteroid-sparing effect.

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