Overview
Pemphigus is a disease that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. It is an autoimmune disorder where the body produces antibodies that damage cells of your skin and mucous membranes. Pemphigus can occur at any age but is most commonly seen in middle-aged or older individuals, and it tends to be a long-lasting (chronic) condition. Treatment with medication usually controls it. There are two common types: Pemphigus vulgaris, which typically begins with blisters in the mouth and then on the skin or genital mucous membranes, and Pemphigus foliaceus, which causes blisters on the chest, back, and shoulders but doesn't cause mouth blisters. It's important to see a doctor if you have blisters inside your mouth or on your skin that don't heal.
Understanding the disease
Symptoms
The symptoms of pemphigus include the development of blisters on the skin and mucous membranes, such as in the mouth or on the genitals. These blisters tend to rupture easily, leaving open sores that may ooze and become infected. Pemphigus vulgaris often begins with blisters in the mouth and then on the skin or genital mucous membranes, while Pemphigus foliaceus causes blisters on the chest, back, and shoulders. The blisters are generally painful, but not itchy. In some cases, pemphigus can be life-threatening if left untreated.
Risk Factors
Risk factors for developing pemphigus include being middle-aged or older, as the condition tends to be more common in this age group. Pemphigus is also more common in people of Middle Eastern or Jewish descent. The exact cause of the disease is unknown in most cases, but it can sometimes be triggered by the use of certain drugs such as angiotensin-converting enzyme inhibitors, penicillamine, and others.
Development
Pemphigus is an autoimmune disorder that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. It can occur at any age, but it's most often seen in people who are middle-aged or older. Pemphigus vulgaris and pemphigus foliaceus are two common types of pemphigus. Pemphigus vulgaris usually begins with blisters in your mouth and then on your skin or genital mucous membranes, while Pemphigus foliaceus causes blisters on the chest, back, and shoulders. Pemphigus isn't contagious, and in most cases, the cause is unknown. However, rarely, it may be triggered by the use of certain drugs, such as angiotensin-converting enzyme inhibitors, penicillamine, and other medications.
Assessment and Diagnosis
Triage
You should see a doctor if you have blisters inside your mouth or on your skin that don't heal, as this could be a sign of pemphigus. The symptoms listed are related to pemphigus vulgaris and pemphigus foliaceus, but if you suspect you or someone else may have pemphigus, it's important to consult a doctor for proper diagnosis and treatment.
Diagnosis
To diagnose pemphigus, your doctor will likely start by discussing your medical history, examining your skin and mouth, and asking questions about your symptoms. In addition to these initial steps, diagnostic tests may be conducted to confirm pemphigus. These tests may include a skin biopsy, blood tests to detect specific antibodies, and an endoscopy to check for sores in the throat. Your doctor may refer you to a dermatologist for further evaluation and specialized care.
Management and Treatment
Complications
Potential complications of pemphigus include skin infection, bloodstream infection (sepsis), malnutrition due to difficulty eating, medication side effects such as high blood pressure and infection, and death if left untreated.
Treatment
Treatment for pemphigus usually involves medication to control the condition. The most common medications used for pemphigus include:
- Corticosteroids: These powerful anti-inflammatory drugs help reduce inflammation and suppress the immune system's attack on healthy cells. Corticosteroids are often the first-line treatment for pemphigus.
- Immunosuppressants: These medications help regulate the immune system by reducing its activity. Immunosuppressants are typically used when corticosteroids are not effective or cannot be used due to side effects. Examples include mycophenolate mofetil, azathioprine, and cyclophosphamide.
- Biologics: For severe cases or when other treatments have failed, biologic medications may be prescribed. These medications target specific molecules in the immune system and have fewer side effects than traditional immunosuppressants. Examples include rituximab and eculizumab. It's important to work closely with a healthcare professional to determine the most appropriate treatment plan based on the severity of the condition and individual factors.
Preparing for medical consultation
When preparing for an appointment for pemphigus, it is important to:
- Be aware of any pre-appointment restrictions or instructions provided by the doctor.
- Write down all symptoms you are experiencing, including any unrelated ones.
- Note down key personal information, such as any major stresses or recent life changes.
- Make a list of all medications, vitamins, or supplements you are taking, including doses.
- Consider bringing a family member or friend along for support.
- Bring any previous scans, X-rays, or medical records relevant to the appointment. Remember to discuss your concerns and questions with your doctor during the appointment, and don't hesitate to ask for clarification if needed.