Bullous pemphigoid typically starts off with a very itchy rash that has an appearance similar to that of hives. This rash may recur for several months. Eventually, the rash evolves to areas that form blisters that then break and leave areas of sore, open skin. The blisters are tight and hard, and the skin between blisters may be red and swollen. The whole body can be affected.

Cicatricial pemphigoid is a blistering disease that causes scarring. Ocular pemphigoid, which affects the eyes, is a common form and can be associated with a feeling of dry, gritty eyes. It can lead to blindness. If the mouth is affected, there can be sore open areas of gums, cheeks, tongue and/or palate. If the throat is involved, swallowing and talking may be affected and be life-threatening if not treated. Body and scalp sores can also appear.

Pemphigus vulgaris may start with oral ulcers that can be mistaken for the more common aphthous ulcers (“canker sores”). These are very painful and interfere with eating and talking. Another early sign are scalp sores, which are like open wounds and can be confused with a local scalp infection. Skin sores are called blisters, but these are weak and break so easily that it looks more like the skin has been scraped, resulting in red and weepy areas. About one half of people with pemphigus vulgaris will develop blisters and sores on their skin. As it is rare, it is common for the condition to progress for months or years before a diagnosis is made.

Pemphigus foliaceus is a rash that has a more scaly appearance—like eczema—rather than like blisters. The face is a common site, but it can also occur on the scalp and body. The skin is very prone to infection and sometimes will be extremely itchy or painful.

Paraneoplastic pemphigus (pemphigus that is caused by an underlying cancer) usually presents with very sore and eroded lips, pain and redness of the eyes, and a widespread rash that has areas that blister and become sore, along with other areas that are itchy and hive-like.

Both pemphigoid and pemphigus are diagnosed through a microscopic examination and immune testing of a skin specimen for antibody deposits. A special immune test, known as direct immunofluorescence, is taken from a skin biopsy and is useful to confirm the diagnosis. Rarely, the antibody titre count is used to confirm the severity of the disease. The antibody titre count is a blood test that measures the level of the protein that is causing the condition.

There is no cure for either pemphigus or pemphigoid. However, once a specific condition is diagnosed, a doctor can determine the best treatment plan. Fortunately, a number of medications and therapies are available:

• Corticosteroids (e.g., prednisone) are most often used to get the disease under control. They are commonly used with other treatment options in order to reduce the dose of this medication. If used for prolonged periods of time, the side effects can be severe.
• Immunosuppessants, such as azathioprine, mycophenolate mofetil, mycophenolic acid, methotrexate, cyclophosphamide, and cyclosporine, help suppress the part of the immune system that triggers the production of auto-antibodies. While it takes time for these medications to work, they have been successful with many people.
• Other therapies used include intravenous immunoglobulin (IVIG), plasmapheresis (a blood-filtering technique that removes antibodies from the blood), and a select number of biologics—medications that address the immune system dysfunction—such as rituximab, infliximab, and etanercept.
• Other drugs that are used with varying degrees of success include dapsone, gold injections, methotrexate, colchicine, tetracycline, minocycline, doxycycline and niacinamide. 

To date, no single medication or combination of medications and therapies has proven to guarantee remission. (Remission is defined as a state in which a patient has had no lesions while off all therapies for at least two months.) What works for one person might not work for another. For this reason, people with these conditions are encouraged to learn about their options and work with their doctor to determine the most effective and appropriate treatment.

 *All information on medical treatments on this site is provided as an overview only. For a complete and up-to-date list of side effects, warnings and precautions, read the product’s package insert and consult your doctor or a pharmacist.
**If you are considering an alternative or complementary therapy, discuss it with your doctor first, and always be sure to keep your doctor up to date about any vitamins, supplements, or other forms of alternative treatment you are taking. Like any medication, alternative therapies can interact with other medications/treatments and, in some cases, have side effects of their own. Remember that “natural” does not necessarily mean “safe.”

In general, pemphigoid can be controlled by taking some daily medications. As a result, the lifestyles of those who live with this condition are largely unchanged. Pemphigus can have a greater impact, as the condition is chronic and can be life-threatening.

Regardless of the type, there are times when either condition can be very debilitating and cause lost time at work, loss of appetite, loss of sleep, hospitalization, and emotional distress. These effects are most often associated with the onset of the disease and during the search for a correct diagnosis.

The medications used in both types of conditions can affect a person’s quality of life. For example, much of the impact on lifestyle results from the side effects of prednisone:

• Many people experience emotional difficulties and mood changes. If these are continual and severe, other medications can be used to offset these side effects. 
• To control weight gain, a high-protein, low-carbohydrate, low-fat diet, combined with regular exercise, is recommended.
• Osteoporosis, glaucoma and cataracts are also known to occur, and regular check-ups with your doctor will help you counter these side effects with appropriate therapies.
• Type 2 diabetes (steroid-induced diabetes) is a common side effect of prednisone and creates a need for a modified diet. Generally, this type of diabetes will diminish as the dosage of prednisone is reduced and may even go away when prednisone is stopped.

When taking immunosuppressants such as CellCept, methotrexate, colchicine or Imuran, your immune system may become suppressed. As a result, you will need to avoid any unnecessary exposure to possible infections, and take care of your general health by reducing stress, getting sufficient rest and maintaining a nutritious diet. 

Anti-inflammatory antibiotics (tetracycline, erythromycin, dapsone) are often used in pemphigoid and may cause nausea or stomach upset. Sometimes taking these medications with ginger ale or crackers can be helpful.

People with both types of conditions may also need to become well-versed on the proper care of their lesions. Wound dressing and care takes time, but with a little practice, knowledge and the right materials, you will quickly learn how to manage your condition. Talk to your care provider for tips.

Psychologically, many people with both conditions report a direct relationship between increased stress and flares in disease activity. If you believe stress is related to an increased incidence of lesions, consider seeking counselling. Ask your doctor for a referral to a counsellor or psychologist to discuss your concerns.

Another source of support can be your family and friends.

Finally, if you’re looking for more information on either disease, visit the International Pemphigus & Pemphigoid Foundation. 

The information in this section has been gathered from existing peer-reviewed and other literature and has been reviewed by expert dermatologists on the CSPA Medical Advisory Board.

Pemphigoid and pemphigus are autoimmune diseases that cause severe blistering of mucosal surfaces and the skin. In an autoimmune disease, the immune system, which attacks foreign invaders, wrongly attacks a healthy part of the body. With pemphigoid and pemphigus, the skin and mucous membranes are the target. The causes of these conditions are unknown.

Pemphigoid has two major forms:

Bullous pemphigoid causes itching that may be painful as well as blisters that appear mostly on the abdomen, back, arms and legs. If left untreated, it can be severe and rarely life-threatening. 

Cicatricial pemphigoid is also known as mucosal membrane pemphigoid, as the lesions usually occur on mucous membrane surfaces. The gums are commonly affected—and epithelium of the eye (cornea and conjunctiva) is affected in up to one third of cases. Cicatricial pemphigoid can result in blindness if it involves the eyes, respiratory compromise if it involves the deeper parts of the throat, and infections if it leaves areas of open skin.

The type of pemphigoid one has depends on which antibodies are produced and in which layer of the skin the blisters occur.

Pemphigus has several types, including pemphigus vulgaris (the most common), pemphigus foliaceus and paraneoplastic pemphigus (the rarest). Blisters of varying sizes break out on the skin, the lining of the mouth, the thin covering of the penis, the vagina, and other mucous membranes. It is life-threatening if not treated.