Updated: 10/2/23
Pemphigus is a rare autoimmune disease that can cause uncomfortable and even painful blisters around the nose, mouth, throat, and other areas of your body. If you or someone you love has been diagnosed with pemphigus, it’s important to develop an effective management plan for controlling symptoms and minimizing flare-ups. Doing so may require some trial and error as well as enlisting the help of several healthcare professionals in order to create an individualized treatment plan best suited for your specific needs. In this blog post, we’ll discuss why getting support from others is key when battling pemphigus – both physically and mentally – along with strategies to get started on finding the right care team for you.
What is Pemphigus?
Pemphigus is a group of rare autoimmune disorders affecting the skin. An autoimmune disorder is a type of disorder or syndrome that causes the body’s immune system to attack healthy body cells as if they were invasive cells. In the case of pemphigus, the immune system attacks the skin and/or moist parts of the body, including the mouth, nose, throat and genitals.
The symptoms of pemphigus can be painful and often debilitating. The most common symptoms include the appearance of blisters and sores on the skin and mucous membranes, particularly in the mouth and throat. These blisters and sores can be painful, making it difficult to eat, drink, or talk. The sores can be small and manageable or large, open and raw. In some cases, they can also cause scarring. Other symptoms may include fever, fatigue, and loss of appetite. In some cases, pemphigus presents a life-threatening situation.
Several forms of it have been identified. Most affect middle aged and older adults. There currently is no cure for the condition, but treatments are available to help keep it under control. In addition, some dietary supplements can help with managing the side effects of treatment.
Types of Pemphigus and Symptoms
Location and characteristics of the blisters determine the type of pemphigus. The catabolic state (breakdown of muscle proteins) that can result from this condition makes it potentially very dangerous to one’s health. In addition, secondary bacterial or viral infections can occur, leading to sepsis.
Pemphigus Vulgaris
Pemphigus vulgaris accounts for about 70 percent of all pemphigus cases and is the most common in North America. The symptoms of this type include:
- Soft blisters that break easily
- Often starts at the mouth and then spreads to other areas of the body including the scalp, chest, neck, back and genitals
- Blisters are painful but not itchy
- Skin peels easily when touched or pinched
- Eating and drinking can be very painful
- Stuffy nose that discharges bloody mucous
- Hoarse voice due to sores in the throat
Pemphigus Foliaceus
Characteristics of this milder form of pemphigus include:
- Blisters that start on the scalp and face before spreading to the skin on the chest and back
- Does not cause blisters in the mouth or mucous membranes
- Blisters that itch, but do not usually cause pain
- Lesions that form crusts on the skin
- Skin peels easily when touched or pinched
Fogo selvagem is a sub-type of pemphigus foliaceus, mostly found in rural areas of South America like Brazil. This type can affect children and younger adults and is thought to be triggered by environmental factors such as insect bites or medication.
Pemphigus erythematosus is another sub-type of pemphigus foliaceus and is also known as Senear-Usher syndrome. With this type, a butterfly-shaped rash often develops on the face. Some researchers describe it as having immunilogical features of both pemphigus and lupus.
Pemphigus Vegetans
The rarest type (accounting for 1-2 percent of cases), pemphigus vegetans:
- Most often starts in the mouth before spreading to other areas of the body
- Can leave thick plaques on the skin, especially under the arms and around the groin
Paraneoplastic Pemphigus
Also a rare type, this form is more commonly found in people with cancer or a benign (non-cancerous) tumor. It presents with:
- Sores that develop on the mouth, lips, eyelids and/or esophagus
- It can affect the lungs, causing a life-threatening situation
- May be resolved partially or completely with removal of tumor
IgA Pemphigus
This is a rare type of pemphigus that affects the skin, mucous membranes, and other organs. It usually occurs in middle-aged or older adults, and the symptoms vary widely depending on the affected organ. The skin symptoms can range from blisters to crusted sores, while the liver symptoms can include abdominal pain, nausea, and jaundice. IgA pemphigus can also affect the lungs, kidneys, and nervous system.
Causes of Pemphigus
Pemphigus results from the body attacking its own healthy cells. Researchers still aren’t sure why this happens, but it appears to be a combination of genetic predisposition combined with exposure to something within a person’s internal or external environment.
Certain medications including penicillimine and ACE inhibitors may trigger the onset of pemphigus. In addition, such as in the case of paraneoplastic pemphigus, tumors are implicated in the onset of the disease.
Risk Factors
Pemphigus is not a contagious condition, which means that it cannot be transmitted from one person to another. Although the condition occurs worldwide, it is more common in specific groups including:
- People of Mediterranean descent
- People living in rural communities in Central and South America
- Middle aged or older adults
Pemphigus Diagnosis
The symptoms of pemphigus may resemble those of other skin diseases such as bullous pemphigoid or herpes. However, there are distinct differences between pemphigus and other skin disorders. For example, bullous pemphigoid also produces large, fluid-filled lesions, but the lesions do not open as easily as those of pemphigus. Herpes also has some symptoms that resemble those of pemphigus. However, herpes is not an autoimmune disorder.
Several tests can be performed to confirm pemphigus including:
Skin Examination
This involves using a cotton swab or finger to rub a patch of the skin that is free from blisters. If the skin peels off easily, then the patient may have pemphigus. However, a skin exam should be accompanied by additional tests.
Skin Biopsy
In this test, a doctor takes tissue samples from the blisters caused by what is suspected to be pemphigus for further analysis under a microscope.
Blood Tests
This involves checking the blood for desmogleins antibodies. The primary function of antibodies is to kill disease-causing pathogens. Patients with this disease have elevated levels of antibodies.
Direct Immunofluorescence (DIF)
DIF is another test to diagnose pemphigus. It involves taking a skin biopsy and staining it with fluorescent dyes that bind to autoantibodies. The biopsy requires examination under a microscope to look for the presence of autoantibodies. DIF is a highly reliable test and can be in conjunction with other tests to diagnose pemphigus.
Immunoblotting
Immunoblotting is a more special test that can confirm a pemphigus diagnosis. It involves separating out the proteins targeting by the autoantibodies and then using a technique, Western blot, to detect the specific proteins that are attacking by the autoantibodies.
Endoscopy
Doctors perform an endoscopy by using a small, flexible tube to examine the inner lining of the mouth and throat.
Coping with Pemphigus
Pemphigus does not only have physical effects, but it can also have severe psychological effects, including distress, fear and loneliness. To cope with the disorder, find out as much as you can about it. There are many resources on the internet as well as libraries that provide valuable information about the condition. You may also talk to your doctor for tips on how you can join support groups and cope with the disease. Other recommendations for dealing with pemphigus include:
- Avoid contact sports that could cause further damage to your skin
- Avoid hot, spicy foods
- Use a soft toothbrush
- Talk to your doctor to prescribe painkillers if needed
- Maintain good oral and skin hygiene
- Apply talcum powder on your skin to prevent it from sticking to your clothes and bedsheets
- Change and launder your towels, sheets and bed linens to prevent secondary re-infection
- See a physician as soon as you suspect pemphigus
Find a Support Network
Coping with pemphigus can be overwhelming. It’s important to have a support network you can turn to for help and encouragement. Consider joining a support group, either in person or online, where you can connect with others who are dealing with similar challenges. These groups provide a place where you can share experiences, advice, and emotional support.
Practice Good Self-Care Habits
Taking care of your body is crucial when you have pemphigus. This includes getting enough sleep, eating a healthy diet, and engaging in regular exercise. Additionally, make sure to take your prescribed medications as directed and keep up with any appointments your healthcare provider recommends.
Manage Stress
Stress can exacerbate pemphigus symptoms, so finding ways to manage stress is important. This may include exercise, meditation, deep breathing, or other relaxation techniques. Make sure to incorporate stress-management practices into your daily routine.
Educate Yourself
Learning as much as you can about pemphigus can help you feel more in control of your health. Talk to your healthcare provider about your condition and make sure to ask any questions you have. Additionally, do your own research to learn about the latest treatments and coping strategies.
Prioritize Activities That Bring You Joy
Living with pemphigus doesn’t mean you have to give up the things you love. Prioritize activities that bring you joy and find ways to modify them to accommodate your condition. Whether it’s spending time with loved ones, pursuing hobbies, or engaging in other activities, do what makes you happy.
Pemphigus Treatment
Although there is no cure, pemphigus can be managed through medication. Doctors can prescribe drugs to improve the symptoms of this condition.
Medications
One of the most common treatment options for pemphigus is immunosuppressive drugs, such as corticosteroids. These drugs work by suppressing the immune system, which can prevent the formation of blisters and sores. However, long-term use of these drugs can cause side effects such as weight gain, mood swings, and increased risk of infection. Therefore, it is important to work closely with a healthcare provider to manage the dosage and monitor for any potential side effects.
This is one of the most effective treatments. It is usually available in pill form and can relieve the symptoms of the condition in just a few weeks.
Immunosuppressants
These drugs prevent your immune system from attacking healthy cells and tissues. Immunosuppressant dosages for pemphigus is for the individual. The dosage and treatment plan of each patient depends on the severity and presentation of the disease at the time of diagnosis. However, they are with significant downsides, including increasing the risk of infection as the immune system suppresses, increasing susceptibility to infections.
Biological Therapy
Another type of medication used to treat pemphigus is biologic drugs. These drugs target specific cells or proteins that play a role in the formation of blisters and sores. Biologic drugs are effective in reducing symptoms, but they can be expensive and may require frequent injections. In this treatment, doctors inject a drug known as Rituxan to decrease antibody activities in the body.
IVIG
Intravenous immunoglobulin (IVIG) is a treatment option for pemphigus that involves infusing antibodies directly into the bloodstream. These antibodies can help fight the autoimmune response that leads to the formation of blisters and sores. IVIG is typically in severe cases of pemphigus or when other treatment options failed. However, it can be expensive and may cause side effects such as headache and nausea.
Plasmapheresis
This procedure is similar to dialysis, where filtering blood is to remove harmful antibodies. It is in severe cases of pemphigus where other treatments failed. Plasmapheresis can also be effective, but, like IVIG, it can have side effects such as low blood pressure, dizziness, and bleeding.
Complementary Therapies
Complementary therapies such as acupuncture, herbal remedies, and mindfulness meditation can help manage pain and reduce stress. These therapies can be alongside conventional treatments to improve overall well-being.
Supplements for Pemphigus
No dietary supplement can prevent or treat pemphigus. However, certain vitamins and minerals can help the body counteract some of the side effects of medication.
Vitamin D
People with pemphigus often need to avoid exposure to direct sunlight since it can irritate their skin. Researchers also found that people with pemphigus generally have lower vitamin D levels to begin with, which could make the condition worse. As a result, supplemental vitamin D is recommended. A doctor can test a person’s current vitamin D levels to check if they’re within healthy range. Too much vitamin D is toxic and can lead to a variety of serious side effects.
Calcium
To use vitamin D3 as a supplement, take 50 mg of this powder daily or as directed by a physician. Because of how concentrated this supplement is, it should be measured and consumed with care. Do not supplement this product if you do not possess an accurate milligram scale.
The main medication to treat pemphigus, corticosteroids, can reduce bone density. This makes bone fractures and falls more likely, causing physical pain and a loss of independence. As a result, those on steroid medications are advised to make sure they consume enough calcium through food and/or supplements. Supplemental calcium comes in many forms such as calcium carbonate, calcium citrate and calcium aspartate.
Ginger
Another side effect of steroid medications is the development of stomach ulcers. Ginger is well known for its stomach soothing effects and can easily be incorporated into the diet, made into a tea or taken as a supplement. As a dietary supplement, take 1,000 milligrams of ginger root extract powder once daily, or as directed by a physician. To avoid any heartburn, take with at least 8 ounces of water.
Curcumin
Curcumin is a powerful anti-inflammatory compound found in turmeric. It has shown to have immune-modulating effects that can benefit people with autoimmune diseases like pemphigus. Curcumin also has antioxidant properties that can help protect the skin from damage and promote healing. Take in supplement form or add to food as a spice.
Omega-3 Fatty Acids
Omega-3 fatty acids are known for their anti-inflammatory properties, which is important in alleviating the symptoms of Pemphigus. Patients with Pemphigus have increased inflammation, so Omega-3 fatty acids can help protect cells from inflammation and reducing inflammation throughout the body. Omega-3 fatty acids can be obtained through natural sources like fatty fish, nuts, and seeds or through supplementation.
Probiotics
Pemphigus is an auto-immune condition, which means that there’s a connection to your gut health. Thus, probiotics have been found to be beneficial in improving gut health, reducing inflammation, and boosting the immune system. Research has also found that probiotics can help improve the gut microbiome, which is critical to the overall healing process. If you’re struggling with Pemphigus, it’s important to consider a probiotic supplement to help improve gut health and overall immune function.
The Bottom Line
Living with pemphigus can be challenging, but with the right treatment and lifestyle changes, it is possible to manage the symptoms and improve quality of life. If you or someone you know is experiencing symptoms of pemphigus, it is important to seek medical attention to receive an accurate diagnosis and develop a personalized treatment plan. Remember, early detection and treatment can greatly improve the outlook and overall health of those living with pemphigus.
Understanding pemphigus and its symptoms is crucial to managing this rare autoimmune disease. While it can be challenging to diagnose and treat, early detection and management can lead to better outcomes. If you suspect you have any symptoms of pemphigus, schedule an appointment with an autoimmune specialist or dermatologist immediately. By taking preventative measures and working closely with a healthcare team, you can help manage the symptoms and improve your quality of life.
Managing Pemphigus can be challenging, but with the right supplements, it’s possible to improve one’s overall healing journey. Probiotics, Vitamin D, Zinc, Omega-3 fatty acids, and Curcumin are beneficial in treating Pemphigus. Although these supplements are not a cure, improving your overall gut and immune health can lead to a better quality of life. If you’re dealing with Pemphigus, it’s important to consider supplements as part of your treatment plan, but always speak to your health care provider before adding any supplements to your routine. Stay strong and keep powering your journey towards a healthier you!
These statements have not been evaluated by the Food and Drug Administration. These products are not intended to diagnose, treat, cure or prevent any disease
