Pemphigoid is a group of autoimmune blistering diseases. In an autoimmune disease, the body's immune system attacks its own organs or tissues by producing autoantibodies (antibodies against the self).
A properly functioning immune system produces antibodies. Like little soldiers, they attack hostile, foreign viruses and bacteria. In a person with pemphigoid, however, the immune system erroneously perceives as foreign one or more proteins that naturally occur in the skin and/or mucous membranes. The immune system responds by producing autoantibodies that attack these proteins.
These proteins are the glue that attach adjacent skin or epidermal cells, keeping the skin intact. When these proteins are attacked, they are damaged, and the cells become separated from each other, i.e., the skin becomes unglued. This causes blisters that do not heal by themselves. In some cases, these blisters can cover a significant area of the skin.
There are two major types of Pemphigoid, and early diagnosis is important. Though there may be a genetic predisposition and some groups with a higher incidence of the disease, the disease appears to affect people across racial and cultural lines, so it's not possible to say who may get Pemphigoid or why. Treatment is available, as is help for living with Pemphigoid, including information on nutrition and caregiving.
There are two major forms of pemphigoid:
- Bullous pemphigoid (BP)
- Mucous Membrane pemphigoid/Cictricial Pemphigoid (MMP/CP)
The type of pemphigoid one has depends on which autoantibodies are produced, and in which layer of the skin the blister occurs.
Bullous Pemphigoid
Patients with bullous pemphigoid develop blisters as a result of autoantibodies directed against skin proteins at the junction between the epidermis and the dermis. BP patients usually experience significant itching (and sometimes pain), and may require treatment with corticosteroids or immunosuppressants. Up to 70% of patients with BP will experience a remission within five years of initial diagnosis, although some patients may relapse.
BP lesions should heal without scarring unless secondary infection occurs. Appropriate wound care is important to promote healing and prevent infection and scarring.
Mucous Membrane/Cicatricial Pemphigoid
MMP/CP has been considered predominantly a disease of the elderly with a peak incidence between 60 and 80 years, it is rarely seen in young adults. However, the incidence in adults 40+ has most recently been noted. Lesions can arise on any mucous membrane surface including the nose, mouth, eyes (OCP), esophagus, larynx, urethra and anal mucosal. Recurrent lesions will produce scarring which can be dangerous on the mucosal surface. The gums are commonly involved and can cause gingivitis. Eye epithelium is affected in about 2/3 of the cases of OCP and it can be cause for considerable concern.
Skin lesions occur in more than 20% of patients and are usually transient. When the lesions are present, they consist of small intact blisters or erosions, usually in the head and neck areas.
Immunoflourescent testing (testing for circulating antibodies) for MMP is highly unreliable and is negative in the majority of cases. It is important to obtain positive direct immunoflourescence results (biopsies), even if it requires repeat biopsies because it can mimic other diseases such as lichen planus.
Spontaneous remissions are rare. The organs involved dictate treatment. This disease is progressive and not highly steroid responsive. If the mouth and nose are the only involvement, treatment should be limited to topical steroids, intralesional steroid injections, or occasional short bursts of oral corticosteroids.
If only the gums are involved, topical therapy applied with flexible dental trays (similar to the disposable molds used to deliver fluoride treatments to the teeth).
A different situation exists if the eyes, esophagus or larynx are involved. In such cases, involvement could include blindness and asphyxiation (due to scarring), and aggressive systemic therapy is warranted. Systemic steroids may not adequately control progression; most other therapies only slow the scarring process. The treatment of choice is oral cyclophosphamide (Cytoxan). About 3/4 of the patients treated with this regimen tolerate the drug. Most of these patients will have complete clinical remission after all drugs are withdrawn.
Occasionally, patients respond to oral dapsone. Those that respond well do so quickly, but unfortunately, these patients are in the minority. Azathioprine and Mycophenolate Mofetil is an alternative for patients who cannot tolerate Cytoxan. Cyclosporine is not recommended for use in MMP/CP. Recently, the biologics have been noted as being an alternative treatment for MMP/CP - specifically, enteracept and infliximab.
Because it is so rare, pemphigoid is often the last disease considered during diagnosis. A dermatologist should be consulted if there are any persistent skin, mouth or eye lesions. Early diagnosis may permit successful treatment with only low levels of medication.
There are three criteria that must be met for a definite diagnosis:
- Proper clinical presentation — visual examination of skin lesions.
- Lesion biopsy — A sample of the blistered skin is removed and examined under the microscope to determine if the cells are separated in the manner characteristic of pemphigoid. Additionally, the layer of skin in which cell to cell separation occurs can be determined.
- Direct immunoflourescence — The biopsy skin sample is treated to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigoid.
In addition to the above, another diagnostic test that may be used is called indirect immunofluorescence or antibody titer test. This measures desmoglein autoantibodies in the blood serum. It may be used to obtain a more complete understanding of the course of the disease. In addition, a serum assay for desmoglein antibodies, known as ELISA, is also available. It is the most accurate but, unfortunately, is not available in all clinical labs.
Use our checklist of items for newly diagnosed patients to discuss with a physician.
Research suggests a genetic predisposition to the disease. While several genetic markers are associated with pemphigoid, exact trigger or triggers are not known. It is unknown why only some people with these markers develop the disease.
Pemphigoid is not contagious. It cannot be transmitted by person-to-person contact or by exchange of blood products or other bodily fluids. Two people in the same family are very rarely affected by it. Pemphigoid strikes men and women equally. Although the onset usually occurs in the elderly (60 and older), pemphigoid can affect young adults and has even been documented in children as young as several months old.
No race or culture is free from pemphigoid. Definitive statistics on the incidence of pemphigoid are not available.
For patients with localized disease, topical steroids or intralesional steroids may be used. For patients with more severe or widespread disease, systemic corticosteroids, such as prednisone, are commonly used. Several other drugs are often used in combination with prednisone.
Oral corticosteroids
Prompt and sufficient doses of steroids, usually prednisone or prednisolone, are required to get pemphigoid under control. Once controlled, the medications are reduced slowly to minimize side effects. A large number of patients experience remission; however, a maintenance dose is often required to keep the disease under control.
Antibiotics
- Cycline drugs — tetracycline, minocycline, or doxycycline in combination with niacinamide
- Erythromycin
- Dapsone®
Immunosuppressants
- Azathioprine (Imuran®)
- Mycophenolate mofetil (CellCept®)
- Cyclophosphamide (Cytoxan®)
- Cyclosporine
Other Drugs & Treaments
- Methotrexate (cell division inhibitor)
- Enbrel® - eteracept
- Remicade® - infliximab
Treatment should always be addressed according to the disease activity that is clinically apparent. An indirect immunofluorescence test (antibody titer count) will generally show a high count when the disease is more active, and will be low or undetectable when the disease is in remission. However, this is not always true. The antibody titer test may be most useful with patients on maintenance doses of drugs. If a titer count is low, then it could be reassuring that the flare is controllable and short. A high titer might indicate the need for further treatment.
To date, no studies have shown that alternative, homeopathic, or any other non-traditional method has been successful in treating these diseases. For the best possible results, it is imperative that traditional treatments be administered.
However, once the disease is under control, alternative therapies may be useful to help reduce drug side effects.
In general, pemphigoid can be controlled to the degree that aside from taking some daily medications, the lifestyles of those who live with these diseases are largely unchanged. There are, however, cases where pemphigoid can be very debilitating and cause lost time at work, loss of appetite, inability to eat normally, weight loss, loss of sleep, hospitalization, emotional distress, etc. These effects are most often associated with the onset of the disease during the search for a correct diagnosis. If an early diagnosis is made and treatment initiated, it is very common for the patient to return to a fairly normal lifestyle.
Much of the impact on lifestyle comes as a result of side effects caused by prednisone. Type 2 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 goes away when prednisone is stopped. Many people on prednisone also experience emotional difficulties and mood changes. If these are continual and severe, other medications are often used to help mitigate these side effects. Another commonly reported side effect of prednisone is weight gain. A high protein, low carbohydrate, low fat diet, as well as a regular exercise program is recommended for those taking prednisone. Osteoporosis, glaucoma, and cataracts are also known side effects of prednisone, and regular checkups with your health care providers will enable most people on prednisone to effectively counter these side effects with appropriate therapies and attention.
It is very important to make certain that all physicians, doctors, and specialists involved with a treatment regimen are in contact with one another to avoid conflicting medications and to be sure that each doctor's treatments are working in harmony with the others'. Also, all lab test results should automatically be given to all physicians on a particular case.
Stress
The possible effect of stress on the onset or subsequent flare of pemphigoid is not clearly understood. Many people who live with pemphigoid report a direct relationship between increased stress and flares in disease activity. Others seem to be unaffected by increased stress.
If you believe stress is related to increased incidence of lesions, it is wise to address and resolve those stress issues. Obviously, many causes of stress are not a matter of choice, but the manner in which an individual deals with the various sources of stress can be modified to minimize the neagative impacts of stress. One of the most common and effective ways to help reduce stress is to openly and honestly discuss it with a spouse, friend, or therapist. Having someone in your corner helps.
Another way is take a few irons out of the fire in order to regroup and recover. Focus on getting yourself back to health before you again devote time and energy to other pursuits. Sometimes it's necessary to pull back in some areas and go back to them after you are more able to handle some "extras."
As with lifestyle concerns, many of the nutrition concerns are associated with prednisone. In order to control an outbreak of pemphigoid, a prompt response with a large dose of Prednisone is generally prescribed. This a glucocorticoid steroid drug which requires a diet high in protein, low in carbohydrates, low in salt, low in fat, with special attention paid to calcium and potassium levels. Calcium with vitamin D supplements are routinely ordered.
Eating a healthy diet is obviously important to health, but it is especially important for pemphigoid patients to mitigate the effects of medications and to give the body all it needs to fight the disease and rebuild the body. Acidophilus, a beneficial bacteria found in yogurt, as well as in dietary supplements, is recommended for those who frequently use antibiotics. It also helps prevent yeast infections.
Be sure to discuss your present diet, medications, and lifestyle with your physician and/or dietician before making changes to your diet.
It has been documented that some some patients have a sensitivity to garlic, onions and leeks (the Alium food group). These foods and possibly others may trigger or worsen flair-ups. A member of the IPPF Medical Advisory Board, Dr. Sarah Brenner of Israel, is doing research in this area. Read Foods That Eat You.
Be sensitive to foods that can help or hurt you. Test yourself. If you suspect that eating certain foods causes blisters, then try it a second time, and if it happens again, eliminate that food from your diet.
More Nutrition Information:
USDA Everything you want to know about food except how it tastes.
USDA (General nutrition information)
Mayo Clinic's nutrition articles
Sodium
Calcium
Potassium
Smoothies
Having trouble swallowing? See this information provided by the Mayo Clinic.
Though the concerns of those who live with pemphigoid are considerable, the impact on spouses, family members, and caregivers should not be ignored or underestimated. Many caregivers take advantage of the online discussion group not only in behalf of the person for whom they care, but to get information, assistance, and even support from other caregivers in similar circumstances. If you are a caregiver, please feel free to use the online discussion group as a tool to help in your endeavors, as well as to contribute your knowledge and experience to those who might need you help.
Being a caregiver comes with its own set of challenges. The uncertainty of the disease, and the need many caregivers feel to always appear to be strong and positive often creates stresses and difficulties which make the challenge even greater. One of the most helpful tools in dealing with this issue is to become as informed as possible. When your loved one has questions, it is of great comfort to him or her to be able to hear a good answer. In general, there is very much to be positive about regarding treatment and control of pemphigoid. Understanding the basics of the disease, along with general treatment protocol, as well as having a few anecdotal instances gives you and your loved one the assurance that you're not alone and that what you are experiencing has been experienced and overcome by someone else.
Read our article on caregiving.
What is Pemphigoid? 
