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Pemphigus: Who gets and causes

Who gets pemphigus?

You’ll find pemphigus in countries around the world and in people of all races. About the same number of men and women get it.

Pemphigus is found in countries around the world

Although pemphigus is rare, it develops in people around the world.

Diverse group of people with map from around the world

Most types of pemphigus are rare in children. Middle-aged or older people are most likely to get pemphigus, including the most common type, pemphigus vulgaris. This type usually begins between 50 and 60 years of age.

Your risk of getting pemphigus vulgaris increases if you have:

  • Jewish ancestry, especially Ashkenazi Jewish heritage

  • Mediterranean ancestry

  • An autoimmune disease, especially myasthenia gravis

The risk of getting other types of pemphigus increases if you:

Take certain medicines: People who take penicillamine have a higher risk of getting pemphigus foliaceus or pemphigus vulgaris. People take this medicine to treat rheumatoid arthritis and Wilson’s disease. It’s also used to prevent kidney stones.

Some other medicines can also increase the risk of getting pemphigus. When a drug causes pemphigus, the person has drug-induced pemphigus.

Live in a rural, tropical area of Brazil or another country in Latin America: People in these areas have a higher risk of getting a type of pemphigus called fogo selvagem, which only occurs in these areas. Children and young adults are most likely to get fogo selvagem.

Researchers have found that when these areas become less rural, fewer people get fogo selvagem. When a rural area becomes a city, people no longer get this type of pemphigus.

Have a tumor growing inside your body, especially if it starts in one of these areas—lymph node, tonsil, spleen, or thymus gland: The tumor can be benign (non-cancerous), a pre-cancer, or cancer. Some people who have a tumor also get paraneoplastic pemphigus. This is the rarest type of pemphigus.

What causes pemphigus?

People get pemphigus when their body’s immune system attacks healthy cells in their:

  • Skin

  • Mouth, throat, or both

  • Moist tissues that lines the inside of the nose, eyelids, anus, genitals, or other areas

The body attacks these healthy cells because it mistakes them for something harmful like viruses or bacteria.

Scientists are still trying to find out why the body does this. We know that pemphigus is NOT contagious, so it’s not something you catch that causes these attacks.

Finding what causes pemphigus could lead to safer and more effective treatments.

Getty Images

Amagai M. “Pemphigus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:417-29.

Habif TP, Campbell, JL, et al. “Vesicular and bullous diseases.” In: Dermatology DDxDeck. Mosby Elsevier, China, 2006: Cards# 100 and 101.

James WD, Berger TG, et al. “Chronic blistering dermatoses.” In: James WD, Berger TG, et al. Andrews’ Diseases of the Skin (10th edition). Saunders Elsevier, Philadelphia, 2006:459-66.