What’s Pemphigus? What Causes It?

Pemphigus is a rare autoimmune skin disease that causes blistering of the skin and mucous membranes. It is caused by antibodies that attack the desmoglein proteins, which are responsible for holding skin cells together. This leads to the formation of blisters that can break open and leave raw, painful sores.

There are two main types of pemphigus:

Pemphigus vulgaris: This is the most common type of pemphigus and affects the skin and mucous membranes of the mouth, nose, throat, and genitals.

Pemphigus foliaceus: This type of pemphigus only affects the skin and does not typically involve the mucous membranes.

Risk factors:

- Age: Pemphigus is more common in people over the age of 40.

- Gender: Women are more likely to develop pemphigus than men.

- Family history: People with a family history of pemphigus are more likely to develop the disease.

- Certain medical conditions: People with certain medical conditions, such as lupus, rheumatoid arthritis, and diabetes, are more likely to develop pemphigus.

- Medications: Some medications, such as penicillamine and captopril, can cause pemphigus.

Symptoms

The symptoms of pemphigus include:

Blisters: These are the hallmark of pemphigus and can occur anywhere on the body. They are typically round or oval and filled with clear fluid. The blisters can break open and leave raw, painful sores.

Itching: The skin around the blisters can be itchy.

Pain: The blisters can cause pain, especially if they are broken open.

Fever: A low-grade fever is common in people with pemphigus.

Chills: Some people with pemphigus also experience chills.

Weakness: Fatigue and weakness are common in people with pemphigus.

Weight loss: People with pemphigus may lose weight due to malnutrition or dehydration.

Diagnosis

Pemphigus is diagnosed based on the appearance of the blisters, a biopsy of the skin, and blood tests.

Treatment

There is no cure for pemphigus, but treatment can help to control the symptoms and prevent complications. Treatment options include:

Oral medications: Oral medications such as prednisone and other corticosteroids can help to reduce inflammation and the formation of blisters.

Immune-suppressing drugs: Immune-suppressing drugs such as azathioprine and methotrexate can help to decrease the production of antibodies that attack the skin.

Biologic agents: Biologic agents such as rituximab and infliximab are used to treat pemphigus in people who do not respond to other medications.

Other treatments: Other treatments that may be used to treat pemphigus include topical corticosteroids, antibiotics, and wound care.