From the Guidelines
Pemphigus vulgaris is a rare autoimmune blistering disorder affecting the skin and mucous membranes, characterized by the immune system's attack on desmogleins, leading to painful blisters and erosions. It is essential to understand that pemphigus vulgaris is a chronic condition that requires long-term management to prevent relapse and minimize morbidity and mortality. The disease occurs when the immune system mistakenly attacks proteins called desmogleins that help cells stick together, causing painful blisters and erosions 1.
Treatment Overview
Treatment typically begins with high-dose corticosteroids such as prednisone (1-2 mg/kg/day) to rapidly control the disease, followed by steroid-sparing immunosuppressants like azathioprine (1-3 mg/kg/day), mycophenolate mofetil (2-3 g/day), or rituximab (1000 mg IV on days 1 and 15, repeated as needed) 1. The British Association of Dermatologists' guidelines recommend starting with prednisolone 1 mg kg-1 per day (or equivalent) in most cases, with adjustments based on disease severity and response.
Key Considerations
Some key points to consider in the management of pemphigus vulgaris include:
- The importance of gentle skin cleansing and non-adherent dressings for erosions to promote healing and prevent infection.
- Pain management and monitoring for infections are crucial aspects of supportive care.
- Regular blood tests are necessary to monitor for medication side effects, particularly with immunosuppressive therapy.
- Patients should be followed closely by a dermatologist experienced in treating autoimmune blistering diseases.
Evidence-Based Recommendations
The most recent and highest quality study, the British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017 1, provides the foundation for current treatment recommendations. This guideline emphasizes the importance of individualized treatment approaches, considering factors such as disease severity, patient comorbidities, and potential side effects of therapies. Rituximab has emerged as a first-line therapy in many cases due to its effectiveness, although its approval status varies by country.
Long-Term Management
Without treatment, pemphigus vulgaris can be life-threatening, but with appropriate therapy, most patients achieve remission, though long-term maintenance therapy is often required to prevent relapse 1. The goal of treatment is to induce and maintain remission while minimizing the risk of treatment-related morbidity and mortality. The decision to withdraw treatment should be cautious and based on careful assessment of disease activity and the risk of relapse.
From the Research
Definition and Overview of Pemphigus Vulgaris
- Pemphigus vulgaris is a rare, chronic, autoimmune mucocutaneous blistering disease that can progress to involve the skin and multiple mucosae 2.
- It is characterized as a chronic, autoimmune, vesiculobullous disease 3.
- The disease can be life-threatening without proper treatment and may affect the skin and/or mucosa 4, 5.
Clinical Characteristics and Prognosis
- Pemphigus vulgaris can be associated with a high morbidity and significant mortality rate 2.
- Early diagnosis and early initiation of treatment are prognostic factors 3.
- The disease may have a long-term course, with some patients requiring treatment for about 5-10 years, while others may need shorter or longer courses 3.
Treatment Options
- Conventional therapy primarily consists of systemic corticosteroids and immunosuppressant agents 2, 5.
- Corticosteroids remain a first-line therapy for pemphigus, but guidelines vary in initial dose, tapering schedule, and management of relapse 4.
- Immunosuppressive agents, such as azathioprine and mycophenolate mofetil, are used as corticosteroid-sparing drugs 4, 5.
- Rituximab, a monoclonal antibody targeting CD20-positive B lymphocytes, is approved as a first-line therapy in moderate-to-severe pemphigus 4, 6.
- Other emerging treatment options include high-dose intravenous immunoglobulins, immunoadsorption, and combination therapy with rituximab and intravenous immune globulin 5, 6.