Pemphigus

Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. These often break open, causing pain and leaving you vulnerable to infection. Pemphigus isn’t contagious. You can manage your symptoms with medicine to help your skin heal.

Causes of Pemphigus #

• The exact cause of pemphigus is unknown. Research suggests that genetics and environmental factors play a role in your diagnosis.

• Pemphigus is an autoimmune condition. This means that your body’s defense system (immune system antibodies) attacks your body’s healthy cells, mistaking them for foreign invaders.

• When your body attacks itself, you’ll notice symptoms of pemphigus in the form of blisters or sores on your skin.

• In rare cases, certain medications, including penicillin, an antibiotic, piroxicam, a nonsteroidal anti-inflammatory drug used for rheumatoid arthritis, and blood pressure medications can cause the condition.

• Some studies found that specific HLA genes, which are genes that build your immune system, predispose you to certain types of the condition

Symptoms of Pemphigus #

Symptoms vary based on the type of pemphigus you have but could include:

• Fluid-filled bump or bubble on your skin (blister).

• The skin around the blister is pink to red.

• Sores that have a crusty appearance.

• Blisters or sores leak clear fluid or bleed lightly.

• Your skin around the blisters is fragile and peels in layers or scales.

• Pain on or near your affected skin.

• Itchy skin.

Blisters and sores can easily become infected. Skin symptoms of an infection include:

• White or yellow pus fills the blister and leaks out if the blister breaks open.

• Pain or a burning sensation to the touch.

• Yellow crust forms on the blister if it breaks open.

• Skin doesn’t heal.

• The area on or around the blister swells or gets bigger.

Severe symptoms of pemphigus include:

• Fever.

• Fatigue.

• Muscle weakness.

• Vision problems and light sensitivity.

Types of Pemphigus #

Pemphigus vulgaris #

• This is the most common type in the U.S. Blisters always affect your mouth.

• Some people may have blisters on their skin and in other mucous membranes.

• These lesions develop in superficial layers of your skin. They can be painful and heal slowly.

• Pemphigus vulgaris blisters in the mouth, which appear as pink and white bumps and open red sores.

• Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth.

• Dark red and white pemphigus vulgaris blisters on the legs and groin.

• Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs.

Pemphigus vegetans #

This type is similar to pemphigus vulgaris but causes thicker lesions. These lesions usually form in areas of your body with skin folds such as your groin and armpit.

Drug-induced pemphigus #

Medications can cause blistering. Some drugs that cause this condition include antibiotics and blood pressure medication. Blisters can develop months after taking the medicine.

Pemphigus erythematosus (Senear-Usher syndrome) #

This type is an overlap syndrome with lupus that causes blisters to develop on your upper back, chest, cheeks and scalp. When lesions form, they’re usually red and scaly.

Pemphigus foliaceus #

This type causes blisters to develop on your scalp, face, neck and back. Lesions rarely appear in your mouth. This type affects your outermost skin layer only. Small blisters may break open easily to form crusty lesions that spread to cover large areas of skin.

Endemic pemphigus (fogo selvagem) #

This is a form of pemphigus foliaceus that occurs more often in South and Central America, particularly Brazil.

Paraneoplastic pemphigus #

This is the rarest type of pemphigus that develops in people diagnosed with cancer. Severe blisters form in your mouth. If your healthcare provider diagnoses paraneoplastic pemphigus, they’ll look for signs of cancer somewhere in your body.

Diagnosis of Pemphigus #

Biopsy #

They’ll take a small sample of your skin’s tissue and examine it under a microscope.

Blood tests #

They’ll examine a sample of your blood to look for antibodies that cause the condition

Treatment of Pemphigus #

Treatment is unique to each person diagnosed with pemphigus and could include:

• Taking medicine to prevent infections and help your skin heal.

• Stopping the use of medicines that cause your symptoms.

• Wound care for blisters and sores.

Medicines used to treat pemphigus include:

Corticosteroids #

Medication to reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (ointments or creams).

Immunosuppressive drugs #

Drugs that manage your body’s autoimmune response, which is what happens when your body attacks healthy cells.

Rituximab #

A monoclonal antibody that targets problematic B cells. Intravenous immunoglobulin: Healthy antibodies (proteins made by your immune system to attack foreign substances), given through a needle into your vein, to help reduce the antibodies that cause your diagnosis.

Antibiotics #

In some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe antibiotic medicine to treat the infection.