Medicines for Pemphigus vulgaris

What it is

Pemphigus vulgaris is a rare, serious autoimmune condition in which the immune system mistakenly attacks the skin and the moist linings of the body (mucous membranes), particularly the mouth. In autoimmune conditions, the immune system produces antibodies against the body’s own tissues; in pemphigus vulgaris, these antibodies attack the proteins that hold skin cells together, causing the layers of the skin (and mucous membranes) to come apart and form blisters and raw, painful areas. The blisters are typically fragile and easily broken, leaving painful sores and raw areas that can be slow to heal. A characteristic feature is that the mouth is often affected — indeed, painful mouth ulcers and sores are frequently the first sign, and can occur before the skin is affected, making eating and drinking painful. Blisters and erosions can also affect the skin (anywhere on the body) and other mucous membranes (such as the throat, nose, eyes, or genitals). Pemphigus vulgaris usually affects adults, particularly in middle age or older. It is a serious condition because, before effective treatment was available, the widespread loss of skin and mucous membrane could lead to significant illness (through fluid loss, infection, and difficulty eating); however, it is now treatable, and the outlook has greatly improved with modern treatment. Because it is driven by the immune system, treatment involves medicines that reduce the immune attack and control the inflammation — such as steroids and other medicines that calm the immune system — which can bring the condition under control and heal the blisters and sores, along with supportive care for the skin and mouth. Pemphigus vulgaris is usually a long-term condition needing ongoing treatment and monitoring, guided by specialists. The key messages are that pemphigus vulgaris is a rare autoimmune blistering condition (often first affecting the mouth) causing painful blisters and raw areas, and that it is treatable with medicines that calm the immune system.

How it is treated

Pemphigus vulgaris is diagnosed and treated by specialists (dermatologists), and the mainstay of treatment is medicines that reduce the immune system’s attack and control the condition, along with supportive care; with modern treatment, the outlook has greatly improved. Because painful mouth ulcers and blisters or raw areas on the skin or mucous membranes can have various causes, reaching the diagnosis involves assessment and tests — including examining the blisters and sores, and taking a skin (and sometimes blood) sample for specialised tests that detect the autoimmune process (such as a biopsy with special staining, and blood tests for the relevant antibodies) — which confirm pemphigus vulgaris and distinguish it from other conditions. Once diagnosed, treatment focuses on reducing the immune attack and controlling the inflammation to heal the blisters and sores and prevent new ones: this usually starts with steroids (corticosteroids, often at higher doses initially to bring the condition under control), frequently alongside other medicines that calm or suppress the immune system (immunosuppressant or immune-modulating medicines) to control the disease and allow the steroid dose to be reduced over time; for many people, a targeted treatment (such as rituximab, a medicine that reduces the immune cells producing the harmful antibodies) is now an important and effective treatment, often used to bring the condition into remission. Treatment is tailored to the individual and the severity, and aims to bring the condition under control (remission) and then maintain it while minimising side effects. Supportive care is important: caring for the skin and wounds, good mouth care and pain relief (as the mouth is often affected and eating can be painful), preventing and treating infection (as the raw areas are prone to infection), and supporting nutrition and comfort. Because pemphigus vulgaris is usually a long-term condition, ongoing treatment, monitoring (of the disease and the treatment and its side effects), and specialist follow-up are needed, with treatment adjusted over time; many people achieve good control or remission with modern treatment. The reassuring messages are that pemphigus vulgaris, although a rare and serious autoimmune condition, is treatable — with medicines that calm the immune system (including targeted treatments) controlling the condition and healing the blisters and sores — that the outlook has greatly improved with modern treatment, and that specialist care and supportive measures (for the skin, mouth, and nutrition) help; so prompt diagnosis and specialist treatment are important, and persistent painful mouth ulcers or unexplained blistering should be assessed.