What are the other names for this Condition? (Also known as/Synonyms)

• PV (Pemphigus Vulgaris)

What is Pemphigus Vulgaris? (Definition/Background Information)

• Pemphigus Vulgaris (PV) is a disorder characterised by bullous (blister-like) eruption on the skin and mucous membranes. It is the most common type of pemphigus, a rare and serious, but non-malignant chronic skin disorder. Pemphigus Vulgaris accounts for 70% of all cases of pemphigus
• It is caused by an autoimmune process in which antibodies are formed against the healthy cells in the skin and mouth. However, what actually triggers the immune system to form the antibodies is currently not well-understood
• The most common symptom of Pemphigus Vulgaris is the eruption of painful blisters in the mouth and skin. This can result in secondary infections of the blisters, which is the most common complication associated with PV. A diagnosis of the condition can be generally made through a physical examination and skin biopsy
• Steroids form the mainstay of treatment for Pemphigus Vulgaris. It is a life-threatening condition with a mortality rate of 5-15%. However, before the availability of modern medications, the mortality rate was almost 100%

Who gets Pemphigus Vulgaris? (Age and Sex Distribution)

• The mean age of onset of Pemphigus Vulgaris is 50-60 years
• The incidence rate is equal in both male and female
• All races and ethnic groups can be affected
• The incidence of Pemphigus Vulgaris ranges from 0.5 to 3 cases per 100,000 population. An increased incidence is noted in Ashkenazi Jews and in people of Mediterranean origin

What are the Risk Factors for Pemphigus Vulgaris? (Predisposing Factors)

The risk factors associated with Pemphigus Vulgaris include:

• Family history of the disorder
• Descendants of Jewish, Indian, and Mediterranean origin are more susceptible than individuals of other racial or ethnic group
• Use of certain medications such as penicillamine and ACE inhibitors (captopril); in such cases, the condition is termed Drug-Induced Pemphigus Vulgaris

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pemphigus Vulgaris? (Etiology)

Pemphigus Vulgaris is an autoimmune disorder characterized by the formation of antibodies against healthy cells in the skin and mucous membranes.

• Generally, antibodies are produced in the body to kill microorganisms, such as bacteria and viruses, which are harmful to the body. But, in pemphigus, the antibodies attack the healthy skin and mucous membranes
• These antibodies act against the proteins in the skin, known as desmogleins, which play a role in skin cell adhesion. When these proteins are attacked, the skin cells get separated from each other, causing fluid accumulation between the skin layers. This leads to the formation of blisters
• Pemphigus Vulgaris is considered to be a type II hypersensitivity reaction

The exact mechanism of ‘disorder trigger’ is unknown; but it may be triggered by environmental factors such as:

• Drugs such as penicillamine, captopril, and rifampicin
• Burns
• Stress
• Hormones
• Pregnancy
• Vaccinations
• UV light and X-rays
• Tumors

Genetic susceptibility could be another major cause for pemphigus development.

What are the Signs and Symptoms of Pemphigus Vulgaris?

The severity of the signs and symptoms varies from individual to individual. The signs and symptoms associated with Pemphigus Vulgaris may include:

• Painful blisters in the mouth and skin
• Blisters rupture easily with draining, oozing, and crusting of the lesions
• Swallowing and eating difficulties due to blisters in the mouth

How is Pemphigus Vulgaris Diagnosed?

Diagnosis of Pemphigus Vulgaris includes:

• Complete evaluation of medical history along with a thorough physical exam
• Nikolsky sign: When the surface of unaffected skin close to the blister is rubbed with a finger or cotton swab, it separates easily. This sign is quite specific to Pemphigus Vulgaris, but may be seen in other conditions too
• Blood test to detect the presence of pemphigus antibodies
• Skin biopsy: A small piece of skin is taken and is sent to the laboratory for examination under microscope by the pathologist. After putting together clinical findings, special studies on tissues (if needed), and with microscope findings, the pathologist arrives at a definitive diagnosis
• Skin biopsy with special preparation to search for antibodies directed against skin structures. This is the ‘gold standard’ for the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pemphigus Vulgaris?

Complications associated with Pemphigus Vulgaris include:

• Severe discomfort and stress
• Secondary infection of the blisters
• Scarring of the healed blisters can cause emotional distress
• Oral erosions causing difficulty in eating and swallowing
• Overall decreased quality of life

Complications associated with chronic use of high dose steroids, which includes Cushing syndrome, osteoporosis, hypertension, and diabetes.

How is Pemphigus Vulgaris Treated?

The treatment goal of Pemphigus Vulgaris is to treat the blisters, prevent the formation of new blisters, and to slow down the disorder process.

• Steroids form the first line of treatment
• Antibiotics are given to treat the secondary infection, as well as for their intrinsic anti-inflammatory effect
• Pain-relievers
• Mouth lozenges to reduce mouth ulcer pain
• Medications used (other than steroids) include:
  • Dapsone: An anti-inflammatory antibiotic
  • Immunosuppressive drugs such as methotrexate, azathioprine, cyclosporine, mycophenolate mofetil, and rituximab
  • Intravenous immunoglobulins
• Plasmapheresis: It is a procedure in which autoantibodies containing plasma is removed from the body and is replaced with donor plasma

How can Pemphigus Vulgaris be Prevented?

Currently, there are no effective methods to prevent Pemphigus Vulgaris.

What is the Prognosis of Pemphigus Vulgaris? (Outcomes/Resolutions)

Pemphigus Vulgaris is a potentially life-threatening autoimmune disorder with mortality of 5-15%. Infection is the most common cause of death in Pemphigus Vulgaris.

• The prognosis is much worse in individuals with extensive skin disorder and in older patients
• With appropriate treatment, the skin condition usually heal without scarring; however, the disorder process tends to be chronic
• Side effects of the medications (steroids) used for the treatment are also typically severe and disabling and add to the mortality

Additional and Relevant Useful Information for Pemphigus Vulgaris:

• The name pemphigus is derived from a Greek word ‘pemphix’ meaning bubble or blister
• Pemphigus was the name given to any blister lesion of the skin and mucous membrane by Wichman, in 1791