What are the symptoms of Pemphigus (a group of chronic blistering skin diseases)?

From the Guidelines

Pemphigus symptoms can be effectively managed with corticosteroids as the first-line therapy, with oral prednisolone being the preferred initial treatment, starting with a dose of 1 mg kg-1 per day, as recommended by the British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017 1. The symptoms of pemphigus include painful blisters and erosions on the skin and mucous membranes, with the main symptoms being fragile blisters that easily rupture, leaving raw, painful areas that may crust over and become infected. These blisters often begin in the mouth before spreading to the skin, particularly on the face, scalp, chest, back, and groin. Patients may experience pain when eating or swallowing if oral lesions are present. Some key points to consider in the management of pemphigus symptoms include:

• Clinical improvement may be seen within days of starting corticosteroids, with cessation of blistering taking 2–3 weeks and full healing taking 3–8 weeks 1.
• The Nikolsky sign, where gentle pressure on normal-appearing skin causes it to separate, is a distinctive feature of pemphigus.
• Other symptoms include itching, burning sensations, and fluid-filled blisters that may ooze and form crusts.
• Pemphigus can also cause weight loss, fatigue, and malnutrition due to painful oral lesions that interfere with eating.
• The treatment should be cautious and not done prematurely, as relapse rates are high initially, with 47% of successfully treated patients relapsing in one trial when treatment was stopped after 1 year 1. The British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017 recommend the following treatment approach:
• First-line therapy: Corticosteroids, with oral prednisolone being the preferred initial treatment, starting with a dose of 1 mg kg-1 per day 1.
• Second-line therapy: Consider switching to alternate corticosteroid-sparing agent if treatment failure with first-line adjuvant drug (azathioprine, mycophenolate mofetil or rituximab) or mycophenolic acid 720–1080 mg twice daily if gastrointestinal symptoms from mycophenolate mofetil 1.
• Third-line therapy: Consider choice of additional treatment options based on assessment of individual patient need and consensus of multidisciplinary team, including cyclophosphamide, immunoadsorption, intravenous immunoglobulin, methotrexate, and plasmapheresis or plasma exchange 1.

From the Research

Pemphigus Symptoms

Pemphigus is a severe autoimmune bullous dermatosis that affects the skin and/or mucosa, and it may be life-threatening without proper treatment 2. The symptoms of pemphigus include blisters and erosions on the oral mucosa and skin, which are caused by autoantibodies reacting with the cell-cell adhesion structures, desmosomes 3.

Treatment Options

The primary treatment modality for pemphigus is oral corticosteroids, while other therapeutic options, such as steroid pulse therapy, immunosuppressants, intravenous immunoglobulins, plasmapheresis, and anti-CD20 monoclonal antibody therapy, are occasionally employed 3. Some key points about treatment options include:

• Corticosteroids remain a first-line therapy for pemphigus, but there are many differences in initial dose, tapering schedule, and management of relapse between different guidelines 2.
• Immunosuppressive agents, such as azathioprine (AZA) and mycophenolate mofetil (MMF), are also widely used as corticosteroid-sparing drugs 2, 4, 5.
• Rituximab is a monoclonal antibody targeting CD20-positive B lymphocytes that is approved as a first-line therapy in moderate-to-severe pemphigus 2.
• MMF demonstrates a shorter time to achieve complete remission (CR) on therapy and has a significantly higher steroid-sparing effect compared to AZA 4.

Disease Management

Some key points about disease management include:

• The guidelines and/or consensus statements for treatment vary widely between groups 2.
• The treatment response can be evaluated using early and late endpoints, such as complete remission (CR) on therapy, CR off therapy, and immunological remission 4.
• Cumulative steroid use, relapse rate, and adverse events should be compared between different treatment groups 4.