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Last updated Dec. 19, 2018

Approved by: Maulik P. Purohit MD, MPH

Pemphigus is a rare, benign, chronic skin disorder that commonly affects mid-to-elderly adults.

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

  • Pemphigus, NOS

What is Pemphigus? (Definition/Background Information)

  • Pemphigus is a rare, benign, chronic skin disorder that commonly affects mid-to-elderly adults
  • It is characterized by the formation of painful blisters on the surface of the skin and mucous membranes
  • The condition generally relates to the malfunctioning of the immune system when antibodies attack the healthy cells in the skin, instead of attacking harmful pathogens such as bacteria and viruses
  • The most commonly affected areas are the mouth, nose, throat, anus, and the genitals
  • Corticosteroids are the mainstay of treatment for Pemphigus. The use of corticosteroids and other medications can help patients recover completely from this skin disorder. Without treatment, Pemphigus may be life-threatening
  • There are no known preventive measures associated with the condition

The following are the types of Pemphigus:

  • Pemphigus vulgaris: It is the most common type of Pemphigus
  • Pemphigus vegetans
    • Pemphigus vegetans of Hallopeau
    • Pemphigus vegetans of Neumann     
  • Pemphigus foliaceus
    • Pemphigus erythematosus
    • Endemic Pemphigus         
  • Paraneoplastic Pemphigus
  • IgA Pemphigus
    • Sub-corneal pustular dermatosis
    • Intraepidermal neutrophilic lgA dermatosis       

Who gets Pemphigus? (Age and Sex Distribution)

  • Any individual irrespective of age and gender may be affected by Pemphigus. Though, middle-aged and older people in the 30-70 years age group are affected the most
  • Adolescent girls are more at risk of the condition than adolescent boys
  • Pemphigus is seen worldwide; but, people of Jewish, Indian, and Mediterranean origin have a higher risk

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

Common risk factors associated with Pemphigus are:

  • Age: Middle-aged and elderly adults face a higher risk
  • Adolescent girls
  • Descendants of Jewish, Indian, and Mediterranean origin are more susceptible to Pemphigus than other racial or ethnic group

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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? (Etiology)

  • Pemphigus is usually caused by dysregulation (impairment) of the immune system
  • Generally, antibodies are produced in the body to kill microorganisms, such as bacteria and virus, 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 may be triggered by many factors such as

  • Environmental factors such as:
    • Drugs such as penicillamine, captopril, and rifampicin
    • Burns
    • Stress
    • Hormones
    • Pregnancy         
    • Vaccinations
    • UV light, x-rays
    • Tumors
  • Genetic susceptibility could be another major cause for Pemphigus development

What are the Signs and Symptoms of Pemphigus?

The major signs and symptoms of Pemphigus is the appearance of blisters on the skin and mucous membrane. The symptoms may vary depending on the type of Pemphigus one is affected with.

The signs and symptoms of Pemphigus vulgaris include:

  • Painful blisters in the mouth, mucous membrane of the genitals
  • Difficulty in eating and swallowing

The signs and symptoms of Pemphigus foliaceus include:

  • Blisters usually appear on the face and scalp and spread to the chest and back
  • The blisters are usually not painful, but itchy

How is Pemphigus Diagnosed?

The diagnosis of Pemphigus may involve:

  • A thorough check up of the mouth and skin of the patient for the presence of blisters
  • Complete evaluation of medical history
  • Nikolsky sign: The physician may rub the skin near the blister and if the skin peels off, it is a sure sign of Pemphigus
  • Skin biopsy: A piece of the tissue from the blister is taken and sent to the lab for examination under a microscope. The pathologist studies the biopsy sample to arrive at a definite diagnosis
  • Immunofluorescence studies on the skin biopsy specimen
  • Blood tests: Blood tests are conducted to check the level of desmoglein antibodies in blood. An elevated level of this antibody will confirm the presence of Pemphigus

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?

Complications associated with Pemphigus include:

  • Skin infection
  • Sepsis: Infection in the blood stream
  • Psychological and emotional stress due to the skin condition
  • Sometimes, the use of some medications may create dangerous side effects

How is Pemphigus Treated?

Treatment options for Pemphigus include:

The use of medications:

  • Corticosteroids: Common drugs recommended by the physician include corticosteroids, such as prednisone, which is usually the mainstay of treatment
  • Immunosuppressants:
    • Azathioprine
    • Methotrexate
    • Mycophenolate mofetil           
  • Biological therapies: Rituximab is a new drug that controls the white blood cells responsible for producing Pemphigus antibodies
  • Other medications, such as dapsone and intravenous immunoglobulin, may also be used

Hospitalization is required when the symptoms are severe. The following procedures may be undertaken while in the hospital:

  • Fluids are given through vein to prevent dehydration: Electrolytes, such as  sodium, potassium, and calcium, are administered intravenously to maintain fluid and electrolyte balance in the body
  • Intravenous feeding is done when the patient suffers from painful sores in the mouth and is unable to eat through their mouth
  • Therapeutic plasmapheresis: In this procedure, the antibodies attacking the skin are removed through a device called a cell separator

How can Pemphigus be Prevented?

Currently, there are no known and definitive measures to prevent Pemphigus, NOS.

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

  • Without treatment, Pemphigus may be life-threatening. However, the mortality rate has significantly reduced with current treatment measures
  • The use of corticosteroids and other medications can help patients to recover completely from the condition
  • Individuals with Pemphigus may have emotional distress

Additional and Relevant Useful Information for Pemphigus:

Please check back for periodic updates to our ‘physician approved content’.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 27, 2015
Last updated: Dec. 19, 2018