What is the treatment for pemphigus vulgaris?

From the Guidelines

The treatment for pemphigus vulgaris should start with high-dose oral prednisolone, with a suggested initial dose of 1 mg/kg/day, and can be combined with adjuvant immunosuppressants such as azathioprine or mycophenolate mofetil. The goal of treatment is to rapidly control the disease, reduce antibody production, and prevent new blister formation. According to the British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017 1, the optimum corticosteroid dosing schedule is not known, and dosing schedules are largely empirical and based on practical experience.

First-Line Therapy

• Corticosteroids are the first-line therapy, with oral prednisolone being the preferred choice
• The initial dose of prednisolone can be 1 mg/kg/day, with adjustments made based on disease severity and response to treatment
• Adjuvant immunosuppressants such as azathioprine (2-3 mg/kg/day) or mycophenolate mofetil (2-3 g/day) can be added to reduce long-term corticosteroid use

Second-Line Therapy

• For patients who do not respond to first-line therapy, alternative immunosuppressants such as cyclophosphamide or rituximab can be considered
• Rituximab, an anti-CD20 monoclonal antibody, has been shown to be effective in treating pemphigus vulgaris, with a dosing regimen of 1000 mg IV given twice, two weeks apart, or 375 mg/m² weekly for four weeks 1

Maintenance Therapy

• Once the disease is under control, the goal is to taper the corticosteroid dose to the lowest effective level, with the aim of reducing the dose to 10 mg daily or less
• Adjuvant immunosuppressants can be continued for months to years to maintain remission
• Proper wound care, pain management, and antibiotics for secondary infections are also important aspects of maintenance therapy

The treatment approach should be individualized based on the patient's disease severity, response to treatment, and potential side effects of therapy. As noted in the guidelines 1, the DCP regimen, which combines intravenous cyclophosphamide with dexamethasone or methylprednisolone, has been shown to be effective in treating pemphigus vulgaris, but its use should be carefully considered due to potential side effects.

From the FDA Drug Label

Dermatologic Diseases Pemphigus, Bullous dermatitis herpetiformis, severe erythema multiforme (stevens-Johnson syndrome), exfoliative dermatitis, mycosis fungoides, severe psoriasis, severe seborrheic dermatitis The treatment for Pemphigus vulgaris includes prednisone (PO), as it is listed under Dermatologic Diseases as a treatment for Pemphigus 2.

• Key points:
  • Pemphigus is listed as a treatment indication
  • Prednisone (PO) is the specified drug
  • The label does not provide detailed treatment guidelines or dosing information for Pemphigus vulgaris.

From the Research

Treatment Options for Pemphigus Vulgaris

The treatment for pemphigus vulgaris typically involves the use of immunosuppressive medications to reduce the severity of the disease. Some of the treatment options include:

• Corticosteroids, such as prednisone, to reduce inflammation and suppress the immune system 3, 4, 5, 6
• Immunosuppressants, such as azathioprine, cyclophosphamide, and mycophenolate mofetil, to reduce the dose of corticosteroids required and minimize side effects 3, 5
• Gold salts as a steroid-sparing agent to reduce the dose of corticosteroids required 4
• Intravenous immunoglobulin (IVIg) therapy for patients who do not respond to conventional immunosuppressive treatment 7
• Cyclosporine, a potent immunosuppressive drug, in combination with prednisone to achieve clinical remission 6

Benefits and Drawbacks of Treatment Options

The benefits and drawbacks of each treatment option are:

• Corticosteroids: effective in reducing inflammation, but can have significant side effects, such as increased risk of infection and osteoporosis 3, 5
• Immunosuppressants: can reduce the dose of corticosteroids required, but can also have significant side effects, such as increased risk of infection and liver damage 3, 5
• Gold salts: can reduce the dose of corticosteroids required, but can also have significant side effects, such as kidney damage and blood disorders 4
• IVIg therapy: can be effective in patients who do not respond to conventional immunosuppressive treatment, but can be expensive and require frequent infusions 7
• Cyclosporine: can be effective in achieving clinical remission, but can also have significant side effects, such as kidney damage and increased risk of infection 6

Treatment Regimens

Different treatment regimens have been studied, including:

• Prednisolone alone 5
• Prednisolone plus azathioprine 5
• Prednisolone plus mycophenolate mofetil 5
• Prednisolone plus intravenous cyclophosphamide pulse therapy 5
• Combined treatment with cyclosporine and prednisone 6 The most efficacious treatment regimen may depend on the individual patient's response to treatment and the severity of their disease.