From the Guidelines
Pemphigus vulgaris is a rare autoimmune skin disorder where your immune system mistakenly attacks proteins that hold your skin cells together, causing painful blisters and sores, which can be life-threatening if left untreated, but with proper treatment, most people can achieve remission and lead normal lives. Pemphigus vulgaris typically starts in the mouth and can spread to the skin, especially on the scalp, face, chest, and back, as noted in the British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017 1. The blisters are fragile and can easily rupture, leaving raw, painful areas that may become infected. This condition occurs when antibodies attack desmoglein proteins, which act like the "glue" between skin cells. Some key points to note about pemphigus vulgaris include:
- Clinical improvement may be seen within days of starting corticosteroids, and cessation of blistering takes 2–3 weeks, while full healing may take 3–8 weeks, as reported in the British Journal of Dermatology study 1
- The optimum corticosteroid dosing schedule is not known, but the British Association of Dermatologists suggests starting with prednisolone 1 mg kg-1 per day (or equivalent) in most cases, and increasing in 50–100% increments every 5–7 days if blistering continues 1
- Treatment usually involves medications that suppress the immune system, such as corticosteroids, along with steroid-sparing agents like azathioprine, mycophenolate mofetil, or rituximab, as outlined in the guidelines 1
- Topical treatments and antibiotics may also be needed for symptom management and to prevent infection
- The condition is chronic and requires ongoing medical care, but with proper treatment, most people can achieve remission, with one study reporting rates of complete remission off-therapy of 38%, 50%, and 75% achieved in 3,5, and 10 years from diagnosis, respectively 1
From the Research
Description of Pemphigus Vulgaris
Pemphigus vulgaris (PV) is a chronic, autoimmune, vesiculobullous disease that affects the skin and mucous membranes 2. It is characterized by the formation of blisters and erosions, which can be painful and debilitating. The disease is caused by autoantibodies that attack the cell-cell adhesion molecules in the skin, leading to a loss of cell adhesion and blister formation 3.
Symptoms and Clinical Features
The symptoms of pemphigus vulgaris can vary, but they often include:
- Blisters and erosions on the skin and mucous membranes
- Painful lesions in the mouth and throat
- Flaccid blisters on the skin that can rupture and leave open sores
- Lesions on other mucous membranes, such as the genitals and eyes The disease usually begins with lesions in the mouth and throat, and can then spread to other areas of the body 4.
Diagnosis and Treatment
Diagnosis of pemphigus vulgaris is based on clinical manifestations and confirmed with histological and immunochemical testing 3. The treatment of pemphigus vulgaris typically involves systemic corticosteroids and adjuvant therapies, such as immunosuppressive agents and intravenous immunoglobulin 2. Recent studies have also shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug 4.
Prognosis and Management
Pemphigus vulgaris is a chronic disease with a mortality rate of around 10%, and septicemia is the main cause of death 4. Patients with pemphigus vulgaris require long-term and multidisciplinary follow-up to manage their condition and prevent complications. Early diagnosis and treatment are critical to improving outcomes and reducing the risk of mortality 2.