What is pemphigus vulgaris?
Pemphigus is a group of autoimmune skin disorders characterised by the formation of life-threatening blisters on the skin and mucous membrane. The immune system attacks the skin by producing autoantibodies that destroy proteins in the skin, causing blisters. There are four major types of pemphigus, the most common one being pemphigus Vulgaris, which accounts for 70% of all pemphigus cases worldwide. Pemphigus Vulgaris is rare, with only one case in 100,000 people in a year. It is more common in certain ethnicities namely the Jews, Indians and Mediterraneans. Pemphigus Vulgaris usually occurs in adults around 40 to 60 years old. Just like any other autoimmune disease, women are more likely to be affected than men. Pemphigus Vulgaris treatment aims to decrease blisters formation and prevent infection.
What are the symptoms of pemphigus Vulgaris?
The disease initially presents with lesions on the mucous membrane of the mouth and genitals. The blisters can cause painful, slow-healing ulcers that are widespread in the inside of the mouth, leading to eating and drinking difficulties. If the larynx is involved, it can cause painful swallowing and a hoarse voice when talking. Nose block and bleeding may occur if the lesions are in the mucosal of the nose. After a few weeks or months, blisters slowly develop on the skin, especially the upper chest, back, scalp and face. These blisters are filled with clear fluid and have thin walls that easily rupture, causing painful and itchy wounds. The skin around the nails may be red, swollen and painful. Moreover, the conjunctiva, oesophagus, parts of the genitals and anus may be affected. If left untreated, pemphigus can cause life-threatening complications including bacterial, viral and fungal infections, malnutrition due to difficulty eating and psychological impact.
How is pemphigus vulgaris diagnosed?
As many disorders may present with clinically similar features, confirmation of the diagnosis of pemphigus vulgaris requires a skin biopsy and blood tests to look for evidence of autoantibodies, especially immunoglobulin G (IgG) in the skin cells or in the blood. Drug-induced pemphigus should first be excluded.
Can pemphigus vulgaris be cured?
There is currently no cure for pemphigus vulgaris, but treatment helps to control the symptoms. Primary goals of treatments are to heal the blister and prevent new ones from forming, as well as preventing infections that can complicate the conditions. The mainstay of treatment of pemphigus Vulgaris is a systemic corticosteroid, such as prednisolone. Steroids work by suppressing the haywire immune system and reducing inflammation. Since their use, there has been a dramatic fall in the mortality rates, from 99% to 15%. In most cases, blisterings stop after 2 to 3 weeks, and it takes about 6 to 8 weeks to fully control the disease. Since there is no significant difference in terms of disease outcomes for lower and higher doses of steroids, experts recommend only to increase the dosage when lower doses show no improvement. This is because there are many side effects to long term usage of steroids such as osteoporosis and increased risk of infections. The ultimate goal is to stop all steroids while keeping the disease quiescent. Even though corticosteroid is not a cure, it greatly improves the quality of life by effectively controlling the disease. Topical steroids and emollients might be helpful.
An alternative medication is a biologic agent, which is an anti-CD20 antibody called rituximab. It suppresses the immune system by binding to the autoantibodies, disabling it to further attack the body. Besides having strong evidence of its effectiveness in treating pemphigus vulgaris, it benefits the individuals by avoiding steroid-related side effects. Rituximab is the next best medication for those who are not fit for steroid-related therapy. However, its high cost remains a huge limiting factor for long-term use.
Other immunosuppressive drugs that are off-label but are helpful to control the disease long term include azathioprine, mycophenolate mofetil (MMF) and cyclophosphamide.
Besides medications, general management of pemphigus vulgaris includes wound care, pain control with analgesics and lifestyle changes. Appropriate wound care is evidently crucial to prevent infections and promote healing. Strict aseptic techniques should be practised like wearing surgical gloves and using aseptic solutions when cleaning or changing dressings. Be gentle with the skin to avoid new blisters. During the active phase of the disease, minimise activities that can traumatise the skin and mucous membrane in the mouth, such as contact sports and eating spicy, acidic and crunchy food. Oral hygiene and dental care should not be neglected despite painful oral blisters. Gently brush the teeth with a soft toothbrush and mint-free toothpaste, and rinse with an antiseptic mouthwash.