PEMPHIGUS VULGARIS
This section covers the following:
- Introduction
- How is pemphigus vulgaris diagnosed?
- How long will my pemphigus vulgaris last?
- How is pemphigus vulgaris treated?
- What drugs might I be offered to reduce the impact of pemphigus vulgaris?
- Where can I find out more information?
What is pemphigus vulgaris?
Pemphigus vulgaris is a rare autoimmune disease that is characterised by painful blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. Pemphigus vulgaris accounts for 70% of all pemphigus cases worldwide although it is extremely rare in New Zealand (about one case per million of the population).
How is pemphigus vulgaris diagnosed?
Often it will be your GP or dentist who will make the initial visual diagnosis and offer a referral to a specialist depending on the part of the body affected. The most common departments are Dermatology, Ear, Nose and Throat (ENT), Maxillo-Facial (Max-Fax) or to a dental hospital.
A biopsy of a blister is usually taken to confirm the diagnosis. The biopsy sample will be processed in the laboratory and examined under a microscope. Part of the biopsy sample will be examined using a technique known as direct immunofluorescence to demonstrate the presence of PV autoantibodies in the skin. It is not unusual to have more than one biopsy taken, especially if both the skin and mucous membranes are involved.
Pemphigus vulgaris autoantibodies measured in the blood (by a specialist technique known as ELISA) can be a useful test to monitor how active the disease is and can help determine if a change of treatment is appropriate.
How long will my pemphigus vulgaris last?
PV is a chronic autoimmune disease with no known cure. Suppressing your immune system is necessary to keep the disease under control and make remission possible. The initial goal of treatment is to clear existing blisters and help prevent relapses. Treatment typically depends on the severity and stage of your disease, with early diagnosis and treatment beneficial to getting your condition under control. Unfortunately, this disease can have unpredictable episodes where it flares for a variety of reasons such as stress or illness in which case treatment may need to be adjusted.
How long does PV last?
Before the advent of corticosteroids, PV had a high fatality rate, with approximately 70+% of patients dying within a year. Nowadays, it is very rarely a cause of death. With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus usually resolves after several months-years. Most patients treated for PV will enter a partial remission within 2 to 5 years.
How does Pemphigus Vulgaris treated?
What is the treatment of pemphigus vulgaris?
The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions [4,5]. Systemic corticosteroids are the mainstay of medical treatment for controlling the disease, usually in the form of moderate to high doses of oral prednisone or prednisolone, or as pulsed intravenous methylprednisolone. Since their use, many deaths from pemphigus vulgaris have been prevented (the mortality rate dropped from 99% to 5–15%). Corticosteroids are not a cure for the disease but improve the patient's quality of life by reducing disease activity. The doses of corticosteroids needed to control pemphigus vulgaris and the length of time on treatment may result in serious side effects and risks.
What drugs might I be offered to reduce the impact of pemphigus vulgaris?
Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. These are most often:
- Azathioprine
- Mycophenolate mofetil
- Cyclophosphamide
Rituximab, which is now approved by the Food and Drug Administration (FDA) in the US.
Other medications that are sometimes used in pemphigus (often in combination) include:
- Dapsone
- Methotrexate
- Tetracyclines
- Nicotinamide
- Plasmapheresis
- Intravenous immunoglobulin
- Extracorporeal photopheresis
- Immunoadsorption
- The TNFα inhibitor
- infliximab.
At optimal therapy, patients may continue to experience mild disease activity.
Other considerations include vaccination (live vaccines are contraindicated), bone prophylaxis, baseline ophthalmological assessment, and psychological support if needed.
Topical therapy
Topical therapy for cutaneous pemphigus vulgaris may include topical steroids and emollients.Treatment of mucosal pemphigus vulgaris may include various formulations of a topical steroid, intralesional steroid, topical tacrolimus, or topical ciclosporin.
General management
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions.
- Handle skin very gently to avoid causing new blisters and erosions.
- Wear surgical gloves and use aseptic technique when changing dressings.
- Analgesics may be needed especially for dressing changes.
- Gently cleanse with an antiseptic solutionor take a bleach bath.
- Drain intact blisters, but leave the blister roof in place.
- Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing.
- Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic.
- An absorbent dressing may be applied over the primary dressing if the erosions are oozing.
- Be vigilant in identifying and treating any infection.
- Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. These include activities such as contact sports and eating or drinking food that may irritate or damage the inside of the mouth (spicy, acidic, hard and crunchy foods).
Oral hygiene and proper dental care are essential.
- Use a soft toothbrush and mint-free toothpaste to gently and thoroughly brush teeth twice daily.
- Rinse using an antiseptic or anti-inflammatory mouthwash.
- Treat oral candidiasis, if present.
Future prospects for treatment
There is future hope that future treatment for pemphigus will be more specific with fewer side effects. The CD20 antagonist ofatumumab has been reported to be effective. Investigators have engineered specific chimeric auto-antibody receptor T-cells to eliminate desmoglein-3-specific B cells in mice. The use of anti-neonatal Fc receptor is under investigation in combination with rituximab.
What are the complications of pemphigus vulgaris?
Pemphigus vulgaris can cause very extensive, life-threatening erosions, especially if the diagnosis is delayed. Other potentially severe complications may include:
- Secondary bacterial infection
- Fungal infection, especially candida
- Viral infection, especially herpes simplex
- Nutritional deficiencies due to difficulty eating
- Complications of systemic steroids especially infections and osteoporosis
- Complications of immune suppressive treatments
- The psychological effects of severe skin disease and its treatment (anxiety and depression)
- An elevated risk of cardiovascular disease (atherosclerosis heart failure, arrhythmia, thromboembolism, and cardiovascular death).
How is the severity of pemphigus vulgaris assessed?
The severity of pemphigus can be scored using various scoring systems.
- PDAI: Pemphigus Disease Area Index
- PAAS: Pemphigus Area and Activity Score
- PAS: Pemphigus Activity Score
- ABSDIS: Autoimmune Bullous Skin Disorder Intensity Score
- Saraswat oral pemphigus scoring
- Pemphigus vulgaris lesion severity score
- Harman pemphigus grading
- Kumar scoring system
- Mahajan scoring system
Where can I find out more information?
We recommend the British Association of Dermatologists Patient Leaflet, the New Zealand DermNetNZ website and the International Pemphigus and Pemphigus Foundation.