What Is Pemphigus?

Pemphigus is a rare autoimmune disorder characterised by painful blistering of the skin and mucous membranes. The immune system incorrectly attacks healthy cells in these tissues, resulting in blisters and sores. Pemphigus is classified into three types: 

• Pemphigus vulgaris
• Pemphigus foliaceus
• Paraneoplastic pemphigus

If you want to learn more about pemphigus, continue reading. Understanding this condition will help you recognise its symptoms, seek appropriate medical care, and manage the disease's impact on your daily life.¹

Overview

Pemphigus is a rare autoimmune disorder that affects the skin and mucous membranes. In individuals with pemphigus, the immune system mistakenly attacks healthy cells in these tissues, resulting in painful blistering and sores. Pemphigus can affect people of any age, gender, or ethnicity, but it is most common in middle-aged or older adults. Although pemphigus is a rare disease, it can have a significant impact on people's lives by inducing pain, discomfort, and emotional distress.¹

The International Pemphigus and Pemphigoid Foundation states that pemphigus and pemphigoid are not contagious through any means, including blood or bodily fluids. Additionally, they are not genetic conditions. Pemphigus and pemphigoid are both rare autoimmune diseases that affect only a small percentage of the population. Pemphigus can be difficult to diagnose as the disease’s symptoms are similar to those of other skin conditions. However, early detection and treatment are critical for managing symptoms and avoiding further complications.²

Pemphigus and pemphigoid are distinguishable by their primary symptoms. Pemphigus is marked by the formation of shallow ulcers or delicate blisters that are prone to rupture easily, while pemphigoid is characterised by the development of stronger, "tense" blisters that are less likely to rupture on their own.^3,4

Types of pemphigus

Pemphigus is an autoimmune disorder that can manifest in several ways, with the two most prevalent forms being pemphigus vulgaris and pemphigus foliaceus. The layer of skin where blisters form and the location of these blisters on the body determines the type of pemphigus a patient is experiencing.

1. Pemphigus vulgaris- can cause blisters to form in the mouth, on other mucosal surfaces, and on the skin. These blisters form deep within the skin's epidermis layer and are frequently painful. In some cases, patients may develop pemphigus vegetans, a subtype of the disease in which blisters form primarily in the groin and under the arms
2. Pemphigus foliaceous- is uncommon and usually affects only the skin. This type of pemphigus causes blisters in the upper layers of the epidermis, which can be itchy or painful. While the two most common types of pemphigus are pemphigus vulgaris and pemphigus foliaceous, there are also less common forms, such as paraneoplastic pemphigus, which is often associated with underlying cancer and can affect multiple organs in the body, causing a variety of symptoms

Aside from the two major types of pemphigus, there are a few other uncommon forms of the disease. 

1. Paraneoplastic pemphigus- typically causes sores in the mouth and on the lips, but this form can also cause blisters or inflamed lesions on the skin and other mucosal surfaces. Paraneoplastic pemphigus is usually associated with an underlying tumour, and can also cause severe lung problems. In some cases, surgical removal of the tumour may be beneficial

2. IgA pemphigus- is caused by an antibody called IgA. Clusters or rings of blisters or pimple-like bumps may appear on the skin with this form

3. Drug-induced pemphigus- Some of the medications that can cause pemphigus-like blisters or sores include antibiotics, blood pressure medications, and drugs containing a chemical group known as a thiol. However, once the medication is stopped, the blisters and sores usually go away. It is critical for patients to discuss any potential side effects of their medications with their doctor¹

Causes of pemphigus

Pemphigus is an autoimmune disease, which means that the immune system attacks healthy cells in the body by mistake. Pemphigus is caused by the immune system producing antibodies that attack proteins in the skin that help skin cells stick together. As a result, the skin becomes fragile and blisters easily.⁵

Pemphigus can occur at any age, but it is more common in middle-aged and elderly people. According to the International Pemphigus and Pemphigoid Foundation, 1 in 25,000 to 50,000 people in the United States suffer from the disease. It has been found that people of Jewish or Mediterranean descent are more likely to contract the disease.⁶

While the precise cause of pemphigus remains unknown, there are several risk factors that can increase a person's risk of developing the condition. These are some examples:

1. Genetic factors: Some people may be predisposed to developing pemphigus due to a genetic predisposition. Certain genes may predispose a person to the disease

2. Environmental triggers: Some environmental triggers, such as medications, infections, or chemical exposure, can cause the immune system to attack healthy cells in the body

3. Age and gender: Pemphigus is more common in older people and women

4. Nationality: Pemphigus is more common in people of Jewish and Mediterranean ancestry

5. Other autoimmune diseases: People who have other autoimmune diseases, such as rheumatoid arthritis or lupus, are more likely to develop pemphigus

A physical exam, a review of the patient's medical history, and a skin biopsy to examine the affected skin tissue under a microscope are typically used to diagnose pemphigus. Blood tests can also be used to detect the presence of specific antibodies in the bloodstream.⁷

Signs and symptoms of pemphigus

Pemphigus symptoms vary depending on the type and severity of the disease. Among the most common symptoms are:

• Painful, shallow blisters or sores on the skin or mucous membranes, such as the mouth, nose, throat, genitals, or anus
• Skin that is itchy, burning, or painful
• Skin that is red, inflamed, or raw around the affected area
• Weakness or fatigue
• Quick weight loss
• Fever
• Dehydration or an imbalance in fluids

It should be noted that pemphigus is a serious and potentially life-threatening condition, and anyone experiencing any of these symptoms should seek medical attention as soon as possible.⁷

Management and treatment for pemphigus

The severity of the disease, the type of pemphigus, and the individual's overall health status all influence how pemphigus is managed and treated. The primary goal of treatment is to prevent complications while controlling blister formation and promoting the healing of existing lesions.

Some pemphigus management and treatment options include:

1. Corticosteroids, such as prednisone, are the most commonly used medications to treat pemphigus. They aid in the suppression of the immune system and the reduction of inflammation. Immunosuppressants and biologics, for example, may also be used.

2. Topical medications, such as corticosteroid creams, can be used to reduce inflammation and promote blister and lesion healing.

3. Wound care: Proper wound care is critical for infection prevention and healing. This may include wound cleaning and dressing, as well as the use of topical or oral antibiotics as needed.

4. Diet: A vitamin and mineral-rich diet may help support the immune system and promote healing.

Living with pemphigus can be stressful and can have an impact on an individual’s mental health. Psychological support, such as counselling or support groups, may assist individuals in coping with the disease's emotional impact.

Pemphigus cannot be prevented as it is caused by an autoimmune reaction. Early diagnosis and treatment can help prevent complications and improve outcomes. It is also critical to manage underlying conditions, such as infections or certain medications, that may contribute to the development or worsening of pemphigus.

It is essential to work closely with a medical professionalexperienced in treating pemphigus to develop a personalised treatment plan that is both safe and efficient for each patient.⁸

Summary

Pemphigus is a rare autoimmune condition that can result in stinging sores and blisters on the skin and mucous membranes. To manage symptoms and prevent complications, which can significantly affect a person's quality of life, early detection and treatment are crucial. Although the precise cause of pemphigus is unknown, a person's risk of developing the condition can be raised by genetic factors, environmental triggers, age, gender, and other autoimmune diseases. It is critical to seek medical attention from a dermatologist or other qualified healthcare provider if you think you may have pemphigus. Many pemphigus patients can control their symptoms and lead fulfilling lives with the right diagnosis and care.