Pemphigus is a group of rare autoimmune disorders characterised by the formation of blistering lesions on the skin and mucous membranes.
It occurs when the immune system mistakenly produces antibodies that target proteins responsible for maintaining the integrity of skin cells. As a result, the immune system attacks these proteins, leading to the separation of skin layers and the formation of painful blisters that can easily rupture.
Pemphigus can cause significant discomfort, pain, and impairment of daily activities. It typically requires ongoing medical management and treatment to control symptoms and achieve remission.
The exact cause of pemphigus is not fully understood. However, it is believed to be an autoimmune disorder, which means that it occurs when the body’s immune system mistakenly attacks healthy cells and tissues.
In the case of pemphigus, the immune system produces antibodies that target proteins called desmogleins, which are responsible for holding skin cells together.
Genetic factors may play a role in predisposing certain individuals to develop pemphigus. It is thought that specific genetic variations may increase the risk of developing the condition. However, genetic factors alone are not sufficient to cause pemphigus, and additional triggers are usually involved.
Environmental factors and triggers may contribute to the onset or exacerbation of pemphigus. These triggers can vary from person to person and may include certain medications, infections, physical trauma or injury to the skin, exposure to ultraviolet (UV) radiation, and hormonal changes.
There are three main types of pemphigus:
This is the most common and severe form of pemphigus. It causes painful, fluid-filled blisters on the skin and mucous membranes, including the mouth and genitals. The blisters tend to be fragile and easily rupture, leading to the formation of raw, painful sores.
This type of pemphigus typically presents with superficial blisters that mainly affect the upper layers of the skin. The blisters are often itchy and can occur on the face, scalp, chest, and back. Unlike pemphigus vulgaris, the blisters in pemphigus foliaceus do not typically involve the mouth.
This is a rare form of pemphigus that is associated with an underlying cancer, such as lymphoma or leukaemia. It is characterised by severe blistering of the skin and mucous membranes, as well as involvement of other organs, such as the lungs and liver.
Although Pemphigus is rare, some people have an increased risk of suffering from this autoimmune disease. Common factors of increased risk include:
Signs and symptoms can show up quickly. Blisters can appear suddenly and spread. Widespread pemphigus can be life threatening. It can turn an otherwise healthy person into one who is extremely sick, incredibly tired, and in pain.
The following explains how pemphigus affects different areas of the body.
Skin
On the skin, blisters usually begin in one area. They can develop on normal-looking skin or on skin that looks inflamed. Either way, the blisters soon break open and ooze fluid. They then become sores, partly covered with crust.
The following pictures show what pemphigus can look like on the skin.
Pemphigus vulgaris
About 70% of people who get pemphigus have this type. The disease affects both skin and mucous membranes. The blisters on the skin burst leaving painful sores.
Pemphigus foliaceus
This type of pemphigus affects only the skin and often develops on the face, scalp, and upper body.
Drug-induced pemphigus
Some patients may get pemphigus after taking certain medicines.
Most sores tend to heal slowly, and some may not heal on their own. After healing, you may see a remnant dark spot. This is not a scar, but it often fades on its own.
Mouth and throat
Patients with pemphigus vulgaris, the most prevalent kind of pemphigus, frequently have painful mouth sores. Before blisters emerge on the skin, 50% to 70% of people with pemphigus vulgaris present with mouth sores.
Mouth sores start as blisters that break, generating the painful sores.
These sores can be so severe that some patients avoid eating solid foods and sip via straw. Talking can be uncomfortable if ulcers form in the throat.
Blisters can be seen spreading from the mouth to the lips and eventually to the skin in some people.
Nails
Nail problems develop in some people who have severe pemphigus. An infection may develop in the skin around the nail, as shown here. Some people see their nails slowly disappear.
With treatment, most people can recover their lost nails.
Moist tissues
Painful sores can develop in the tissue lining the inside of the eyes and nose, the genitals, the anus, and other areas of the body. Even the oesophagus (the tube that connects the throat to the stomach) can develop blisters and sores in rare cases.
Other symptoms of pemphigus include:
If sudden blisters appear on your skin or mouth, consult a certified dermatologist immediately to deal with the problem.
Living with pemphigus can be bothersome. It can take a toll on an individual’s life, both physically and emotionally. The painful blisters and sores can cause discomfort, disrupt daily activities, and even affect self-esteem. Addressing pemphigus is crucial to improve quality of life and prevent further complications.
DermaSure Skin & Hair Clinic, a trusted and renowned skin clinic in Delhi, specialises in providing effective treatment options for pemphigus. With a team of experienced dermatologists and a patient-centred approach, DermaSure aims to help individuals manage their condition and regain control over their skin health.
The diagnosis of pemphigus involves a comprehensive evaluation by a dermatologist or specialist in autoimmune disorders. The process typically includes the following steps:
Information about your symptoms, their duration, and any potential triggers or family history of autoimmune disorders is taken into account.
A thorough examination of the skin and mucous membranes is conducted to assess the presence of blistering lesions, their characteristics, and their distribution.
A small sample of skin tissue (biopsy) will be taken from a blister or affected area to look for characteristic changes in the skin layers and the presence of immune cells.
A sample of the skin biopsy is treated with fluorescent dyes that detect the presence of antibodies and immune proteins. This helps confirm the autoimmune nature of the condition.
Conducted to measure the levels of specific antibodies in the blood, such as anti-desmoglein antibodies, which are commonly associated with pemphigus.
Accurate diagnosis is essential for appropriate management and treatment of pemphigus. At DermaSure Skin & Hair Clinic, your treatment is guided by one of the best dermatologists in Delhi and we address pemphigus through a comprehensive and personalised treatment plan.
If you have mild pemphigus, a corticosteroid may be recommended. Many people need stronger medicines like prednisone or methylprednisolone. These corticosteroids work throughout the body. A corticosteroid can clear the blisters and sores.
This medication quiets (or suppresses) the immune system. Either azathioprine or mycophenolate mofetil is often used to treat pemphigus. These can stop the body from creating new blisters.
Rituximab is a FDA approved drug for the treatment of pemphigus which is now being used as a first line agent in its treatment. It is given intravenously as 2 doses of 1 gm each at a gap of 15 days.
If you have an infection, this type of medicine will be part of your treatment plan.
Your dermatologist may include baths and wound dressings in your treatment plan. This can help heal blisters and sores.
Don’t let Pemphigus plague you – consult the top dermatology centre in Delhi/NCR, At DermaSure, we are committed to helping you manage pemphigus and reclaim confidence in your skin health.
While there is currently no known cure for pemphigus, with appropriate treatment and management, many individuals with pemphigus can achieve remission and control the symptoms effectively. It is crucial to work closely with healthcare professionals to develop a personalised treatment plan.
Common symptoms of pemphigus include painful blisters and erosions on the skin and mucous membranes, often accompanied by itching, burning, and discomfort. The blisters are fragile and can rupture easily.
Treatment for pemphigus aims to suppress the autoimmune response and manage symptoms. It typically involves the use of systemic corticosteroids, immunosuppressive medications, and sometimes intravenous immunoglobulins. Close monitoring and regular follow-ups with a dermatologist or specialist are important for effective management of the condition.
Pemphigus is an immune system clutter. Ordinarily, your immune system produces antibodies to battle off hurtful intruders, such infections and microbes. But in pemphigus, the body produces antibodies that harm cells of your skin and mucous films. Pemphigus isn’t infectious.
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