Understanding Pemphigus: Symptoms, Types, Causes, Diagnosis, and Treatment

Pemphigus is a group of rare, non-contagious autoimmune diseases that cause the body's immune system to attack healthy skin and mucous membranes, resulting in painful blisters. Specifically, the immune system produces antibodies against desmogleins, which are the proteins that help skin cells bond together; hence, skin cells separate and form fragile blisters. While not contagious, pemphigus can be a serious condition if left untreated. In this article, we'll explore the types, symptoms, causes, diagnosis, and treatment options for pemphigus.

Types of Pemphigus and Their Symptoms

There are several types of pemphigus, each with its own set of symptoms:

• Pemphigus vulgaris: The most common type, affecting the mouth, genitals, and skin with painful blisters that peel easily.

• Pemphigus foliaceus: Causes itchy, crusty blisters on the chest, back, and shoulders.

• Endemic pemphigus (fogo selvagem): A rare type of pemphigus foliaceus that often affects multiple family members in South America.

• Pemphigus vegetans: Similar to pemphigus vulgaris but with thick, wart-like lesions in areas where skin rubs together.

• Drug-induced pemphigus: Triggered by certain medications, with blisters appearing up to 6 months after taking the drug.

• Pemphigus erythematosus (Senear-Usher syndrome): Causes blisters on the scalp, cheeks, upper back, and chest that can turn into red, crusty lesions.

• Paraneoplastic pemphigus: The rarest type, affecting people with cancer and causing difficult-to-treat mouth blisters.

Causes and Risk Factors

Pemphigus is an autoimmune disorder, meaning the immune system mistakenly attacks the body's own cells. In rare cases, certain medications, such as penicillin and some rheumatoid arthritis drugs, can trigger the condition. However, the exact cause is often unknown.

Some people are more likely to develop pemphigus, including those who are:

• Of Jewish, Indian, Middle Eastern, or Southeastern European descent

• 40 years old or older

• Diagnosed with other autoimmune diseases, particularly myasthenia gravis

Diagnosing Pemphigus

Diagnosing pemphigus can be challenging, as several conditions can cause blisters. To ensure an accurate diagnosis, your doctor may perform the following tests:

• Medical history: Reviewing your medications, as some drugs can cause pemphigus.

• Skin exam: Rubbing a patch of skin not covered by a blister to see if it peels easily.

• Skin biopsy: Examining a tissue sample from a blister under a microscope.

• Blood tests: Checking for elevated levels of antibodies called desmogleins.

• Endoscopy: Using a thin, flexible tube to examine blisters in the mouth and throat.

Treatment Options for Pemphigus

Treatment for pemphigus depends on the type and severity of the condition. Options may include:

• Corticosteroids: Often the first line of treatment, taken orally to relieve symptoms within a few weeks.

• Immunosuppressants: Medications that prevent the immune system from attacking healthy tissues.

• Biological therapies: Injections of rituximab (Rituxan) to decrease the number of antibodies attacking the body.

• Antibiotics, antivirals, and antifungal medications: Used to fight or prevent infections.

• Wound care: Topical antibiotics and dressings to heal blisters.

• Plasmapheresis: A process that removes plasma from the blood and replaces it with donor plasma, used when other treatments are ineffective.

Without proper treatment, pemphigus can lead to serious complications, such as skin infections, sepsis, malnutrition, and even death. Additionally, medications used to treat pemphigus may cause side effects like infections, rashes, high blood pressure, and osteoporosis.

With appropriate treatment, at least 75% of people with pemphigus will experience complete remission within 10 years. Some individuals may need to continue taking medications indefinitely to prevent symptoms from recurring.

If you suspect you may have pemphigus, it's essential to consult a healthcare professional for an accurate diagnosis and proper treatment. For more information on pemphigus and other autoimmune diseases, visit the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the International Pemphigus & Pemphigoid Foundation, or the National Organization for Rare Disorders.

The Bottom Line

Early recognition and aggressive treatment are crucial since untreated cases historically had 90% mortality rates, though modern therapy achieves excellent outcomes. The key is prompt diagnosis through specialized testing when persistent oral or skin blisters develop. If you're experiencing unexplained blistering that won't heal, Doctronic can help connect you with dermatology specialists for proper evaluation.