From the Guidelines
Pemphigus foliaceus is an autoimmune blistering skin disease characterized by superficial blisters that easily rupture, leaving crusted erosions, and is not directly addressed in the provided guideline evidence, which focuses on pemphigus vulgaris. However, based on general medical knowledge, it occurs when autoantibodies target desmoglein 1, a protein that helps cells stick together in the upper layers of the epidermis. The provided evidence 1 discusses the management of pemphigus vulgaris, which may share some similarities with pemphigus foliaceus, but it is essential to note that the two conditions have distinct characteristics and may require different treatment approaches.
Some key points from the guideline evidence for pemphigus vulgaris include:
- First-line therapy with corticosteroids, such as oral prednisolone, with an optimal dose not established but suggested to start with 1 mg/kg/day in most cases 1.
- The use of adjuvant immunosuppressants, such as azathioprine, mycophenolate mofetil, or rituximab, to reduce the dose of corticosteroids and maintain remission 1.
- The importance of monitoring for treatment side effects, including osteoporosis, and assessing the risk of osteoporosis immediately 1.
In the context of pemphigus foliaceus, treatment typically begins with high-potency topical corticosteroids for mild, limited disease, and oral prednisone at 0.5-1 mg/kg/day is often used for more extensive disease, sometimes combined with steroid-sparing immunosuppressants. The primary goal of treatment is to induce and maintain remission, reduce the risk of complications, and improve the patient's quality of life. Supportive care includes gentle skin cleansing, avoiding trauma to blisters, and treating secondary infections with appropriate antibiotics. Patients should be educated about sun protection, as UV exposure can trigger flares, and should be monitored for medication side effects, including regular blood tests to check liver function, blood counts, and glucose levels.
From the Research
Definition of Pemphigus Foliaceus
- Pemphigus foliaceus (PF) is a rare autoimmune bullous disorder that affects the skin and is characterized by the presence of autoantibodies against desmoglein 1 (Dsg1) 2.
- It is a type of pemphigus, which is a group of autoimmune mucocutaneous bullous diseases that can cause significant morbidity and mortality 3.
- PF differs from pemphigus vulgaris (PV) in that it only affects the skin, while PV can also affect the mucous membranes 4.
Characteristics of Pemphigus Foliaceus
- PF is usually milder than PV, but can still be resistant to treatment and require long-term management to control the disease 2.
- The disease is caused by the formation of autoantibodies against Dsg1, which leads to the separation of skin cells and the formation of blisters 2.
- PF can be characterized by the presence of skin lesions, which can be treated with various therapies, including corticosteroids, immunosuppressive agents, and biologics 5, 4.
Treatment of Pemphigus Foliaceus
- The treatment of PF typically involves the use of systemic corticosteroids, which can be effective in inducing remission, but can also have significant side effects 5, 6.
- Immunosuppressive agents, such as azathioprine and mycophenolate mofetil, can be used as adjuvant therapy to reduce the dose of corticosteroids and minimize side effects 5, 6.
- Biologics, such as rituximab and intravenous immunoglobulin, have also been shown to be effective in treating PF, particularly in patients who are nonresponsive to conventional immunosuppressive therapy 4.