Pemphigus vulgaris is a rare autoimmune condition that causes painful blistering on the skin and mucous membranes. These blisters commonly appear in the mouth and are prone to bursting easily, which may leave sore patches.

The term “pemphigus” refers to a group of autoimmune skin conditions in which problems with the immune system cause sores, blisters, or fluid-filled bumps to form on the skin. The name comes from “pemphix,” the Greek word for “blister.” The term “vulgaris” refers to it being the most common type.

In this article, we will discuss pemphigus vulgaris, including its causes, symptoms, treatment, and more.

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Pemphigus is a rare group of autoimmune diseases that cause blisters. Pemphigus vulgaris is the most common of these skin conditions, accounting for roughly 70% of all pemphigus cases worldwide. It has the characteristic feature of severe blistering of the skin and the mucous membranes.

The frequency of pemphigus vulgaris varies by geographic location and ethnicity. A 2017 review suggests there are roughly 30,000 cases of pemphigus vulgaris in the United States, with an incidence of 1–10 new cases per 1 million people. The condition affects both sexes equally and typically begins between the ages of 40 and 60.

The characteristic feature of pemphigus vulgaris is soft blisters filled with clear fluid that appear on otherwise healthy-looking skin. These blisters often break easily.

An estimated 50–70% of people with pemphigus vulgaris develop blisters in their mouth before having skin blisters that may come and go. However, the condition may also affect other mucous membranes, including those in the:

  • eyes
  • nose
  • throat
  • genital area

Blistering typically results in severe pain, itching, burning, and stinging sensations. The pain from mouth blisters can be so severe that a person may stop eating solid foods and consume only liquids through a straw. Chewing and swallowing can worsen pain and lead to malnutrition and weight loss.

The ruptured skin blisters may also limit a person’s daily activities. Skin lesions commonly affect the following areas:

  • chest
  • scalp
  • back
  • face

Other potential symptoms of pemphigus conditions include:

  • nail damage and nail loss
  • light sensitivity
  • eye problems
  • fatigue
  • weakness

Another characteristic symptom in pemphigus conditions is the Nikolsky sign. This name derives from Dr. Pyotr Nikolsky and refers to when the top layers of the skin slip away from the lower layers in response to pressure. This sign is a useful diagnostic tool in identifying cases of blistering skin conditions.

Pemphigus vulgaris is an autoimmune blistering skin condition. This means that the immune system mistakenly attacks healthy tissues in the skin. Specifically, the condition results from autoantibodies attacking proteins that help layers of skin stick together. This causes a buildup of fluid between the skin cells, which leads to the formation of blisters.

Kerantinocytes are a type of skin cell that stick together at spots known as desmosomes. These sticky spots contain proteins that form a glue and help connect the skin cells. One of these proteins is called desmoglein. In pemphigus vulgaris, autoantibodies attack desmoglein proteins and prevent them from sticking skin cells together, resulting in blistering.

In most cases, the autoantibodies bind to a protein known as desmoglein 3. However, roughly half of individuals who experience pemphigus vulgaris also have autoantibodies for another protein, desmoglein 1. While researchers know that this attack on desmoglein results in pemphigus vulgaris, it is still not clear why some people develop these autoantibodies.

While the condition is not hereditary, genetic factors may play a role. Research highlights an association between pemphigus vulgaris and human leukocyte antigent (HLA) class II genes. As such, certain populations may have higher incidences of pemphigus vulgaris, including those who:

  • are from India
  • are from Southeast Europe
  • are from the Middle East
  • have Jewish ancestry, particularly Ashkenazi Jewish

Additionally, the following environmental factors may induce immune dysregulation, which can cause pemphigus vulgaris to flare up:

Certain medications may also induce pemphigus vulgaris, including:

To diagnose pemphigus vulgaris, a doctor will typically perform the following:

  • a medical history and review of symptoms
  • a physical exam, which may include looking for the Nikolsky sign
  • blood tests to determine the presence of autoantibodies
  • a skin biopsy to review a skin sample under a microscope

Treatments for pemphigus vulgaris primarily aim to reduce blister formation, promote the healing of blisters and sores, and prevent potential infections. Early and effective treatment is important, as it can prevent potentially serious complications.

After initial treatment, it may take 2–3 weeks for blister development to stop and 6–8 weeks for healing to occur. Treatment will then typically involve slowly decreasing medications to allow a person to manage the condition.

Usually, a doctor will begin treatment with corticosteroids to suppress the immune system. A doctor will first prescribe a high dose of corticosteroids to manage the condition and then slowly reduce the dose to minimize potential side effects.

As such, a doctor may begin prescribing steroid-sparing drugs to allow a person to reduce their dose. These typically include other immunosuppressant drugs such as:

A doctor may also prescribe medications to treat infections and topical therapy to help soothe the skin.

Additionally, they will advise a person on appropriate wound care. This may involve handling skin gently, cleansing with antiseptic solutions, avoiding activities that may irritate the skin, and following proper oral and dental hygiene guidelines.

Aside from the severe pain, extensive blistering may also lead to:

  • infections
  • life threatening fluid loss
  • significant skin damage
  • nail changes
  • pigment changes
  • sepsis

Pemphigus is a rare group of skin blistering conditions. Aside from pemphigus vulgaris, the following forms of pemphigus also exist:

Pemphigus foliaceus

This type of pemphigus often affects the skin but not the mucous membranes. In pemphigus foliaceus, the autoantibodies attack only the desmoglein 1 protein in the skin, not desmogein 3.

Blisters are usually small and scattered and characterized by painful and burning sensations. This type commonly affects the scalp, face, torso, and armpits.

IgA pemphigus

This condition causes grouped blisters with crusts in the epidermis. In IgA pemphigus, a person’s autoantibodies instead attack the protein desmocollin 1.

Pimple-like lesions may come with redness and herpes in a circular or ring-shaped pattern. This type commonly affects the trunk and nearby extremities. Like pemphigus foliaceus, it does not typically affect mucous membranes.

Paraneoplastic pemphigus

Paraneoplastic pemphigus is an autoimmune multi-organ syndrome associated with underlying malignant or benign tumors, most commonly non-Hodgkin lymphoma.

There is often severe and critical involvement of the mucous membranes, which leads to extensive inflammation of the mucous linings. The skin lesions vary but may include erosions, blisters, and lichen-type lesions. They may resemble skin lesions from other autoimmune skin diseases such as lichen planus and erythema multiforme.

Pemphigus vulgaris is a rare autoimmune skin blistering condition. The characteristic feature of this condition is blistering of the mucous membranes, most commonly within the mouth. It occurs when the immune system mistakenly attacks proteins in the skin, causing blisters to form.

Treatment options typically involve the use of immunosuppressants to reduce blister formation and help promote the healing of blisters and sores. A doctor may also prescribe medications to prevent potential infections and soothe the skin.