Pemphigus

What is Pemphigus?

Pemphigus is a group of rare skin disorders that cause your skin to blister. It can also affect the mucous membranes of your mouth or your genitals. However, it’s not contagious. 

There are two main types: pemphigus vulgaris and pemphigus foliaceus.

  • Pemphigus vulgaris. The most common form, pemphigus vulgaris usually begins with blisters in your mouth, which then erupt on your skin. Blisters can also break out on the mucous membranes of your genitals. The blisters typically are painful, but don't itch. Blisters in your mouth or throat may make it hard to swallow and to eat.

  • Pemphigus foliaceus. This type doesn't usually affect mucous membranes. The blisters, which usually begin on your face and scalp and later erupt on your chest and back, usually aren't painful. They tend to be crusty and itchy. Pemphigus can occur at any age, but often strikes people in middle age or older. It’s considered a chronic skin condition but it can be controlled with an early diagnosis and proper treatment that may include similar treatments as those used for burns. Pemphigus may also occur in the setting of cancer, known as paraneoplastic pemphigus.

Diagnosing pemphigus

We perform the following tests in order to properly diagnose you with pemphigus:

  • Skin biopsy. This will show the presence of a blister under the epidermis as well as inflammatory cells in the dermis.

  • Direct immunofluorescence. This is done to shows an immune-mediated disease process. Autoantibody immunoglobulin G (IgG) is deposited in a line along the dermoepidermal junction.

  • Indirect immunofluorescence. This is a blood test to reveals the presence of circulating IgG autoantibodies that target the skin basement membrane and type VII collagen.

 

Causes of pemphigus

Your immune system is your primary defense from foreign invaders, like viruses and bacteria. But in pemphigus, the immune system mistakenly produces antibodies that attack healthy cells in your skin and mucous membranes.

 

Sometimes, pemphigus develops as a side effect of certain medications, such as certain blood pressure drugs or chelating agents. This type of pemphigus usually improves when the medicine is stopped.

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