Understanding P/P

Pemphigus is not contagious – not even blood to blood. Pemphigus is treatable and manageable. Pemphigus patients are not alone.

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What are pemphigus and pemphigoid?

Pemphigus

Pemphigus is a group of rare autoimmune blistering diseases of the skin and/or mucous membranes. Pemphigus is chronic and progressive and characterized by blisters and ulcers on the skin and mucous membranes. The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. PV affects the oral mucosa in nearly all cases (>80%) and affects the oral mucosa first in the majority of cases, and may in fact be the only site in patients.

A minority will present only with cutaneous erosions. Skin involved is typically the upper chest, back, scalp, and face, but lesions can occur on any part of the body. Note that the skin dominant form manifests the Dsg1 autoantibody profile (as in pemphigus foliaceus), whereas the mucosally dominant form manifests the Dsg3 autoantibody profile. A mixed pattern of skin and mucosa manifests a mixed Dsg1 and Dsg3 autoantibody profile. The condition progresses over weeks to months.

Sites often overlooked include around the nails (manifest as painful, red, and swollen), the pharynx and larynx (pain on swallowing and hoarseness), and the nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing the nose in the morning). As many as 49% of patients were shown to have laryngeal and nasal involvement.

Antibodies that attack one’s own cells are called autoantibodies. The part of the cells that are attacked in pemphigus are proteins (desmogleins) which act as the glue that attaches adjacent skin cells, keeping the skin intact. When autoantibodies attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal naturally. In some cases, these blisters can cover a significant area of the skin.

The disease appears to affect people across racial and cultural lines, so it’s not possible to predict who may get pemphigus. Although fairly common in adults, pemphigus is rare in children. In children, it is most often caused by placental transmission of IgG autoantibodies in utero from a woman who has pemphigus.

If left untreated, the erosions and bullae of pemphigus vulgaris gradually spread, involving an increasing surface area, and can become complicated by severe infections and metabolic disturbances.

Before the advent of corticosteroids, pemphigus had a high fatality rate, with approximately 70+% of patients dying within a year.

With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus often resolves after several months. Most patients treated for pemphigus will enter a partial remission within 2 to 5 years. In a longitudinal study of outcome in 40 patients with pemphigus vulgaris, 45% entered a complete and long-term remission after 5 years and 71% after 10 years.

Pemphigoid

Pemphigoid is a group of sub-epidermal, blistering autoimmune diseases that primarily affect the skin, especially the lower abdomen, groin, and flexor surfaces of the extremities. Here, autoantibodies (anti-BPA-2 and anti-BPA-1) are directed against the basal layer of the epidermis and mucosa. The condition tends to persist for months or years with periods of exacerbation and remission. Localized variants of the condition have been reported, most often limited to the lower extremities and usually affecting women. There are two predominant types of pemphigoid: mucous membrane pemphigoid (MMP) also called cicatricial pemphigoid, and bullous pemphigoid (BP). Pathogenesis and management are quite different for these conditions. Scar formation in mucous membrane pemphigoid can lead to major disability.

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. Caregivers, patients, and doctors should discuss the patient’s anxiety level and coping abilities with BP, encourage open discussion about BP, treatment regimen, potential complications, and the need for ongoing treatment.

Who does it affect?

Definitive statistics on the incidence and prevalence of pemphigus are not available, but estimates of the number of new cases diagnosed each year ranges from as high as 5 per one hundred thousand to as low as one per million, depending upon the type of pemphigus and the ethnicity of the affected population.

It is known to affect people across racial and cultural lines. However, there are certain groups of people (Eastern European Jews, people of Mediterranean, Northern India and Persian decent) who have a higher incidence of the disease. Research studies suggest a genetic predisposition to the disease.

Men and women are equally affected.

Although the onset usually occurs in middle-aged and older adults, PV and PF also occur in young adults and children.

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.

Cats, dogs, horses, and goats can also have PV or PF, although PF is more common.

How do they affect my quality of life?

A diagnosis of pemphigus can be very upsetting. One day you feel perfectly healthy, the next day you learn you have a chronic, life-threatening illness. In general, once pemphigus and pemphigoid are under control, you should be able to return to your normal lifestyle. You may notice there are times when you feel wonderful, and other times when you feel discouraged, in pain, or depressed. There are cases where pemphigus or 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. Fortunately, this usually occurs with the onset of the disease, during the search for a correct diagnosis and proper treatment. Once the disease is diagnosed and effective treatment is initiated, you will find your life returning to normal.

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.</div>
[box type="info"] Much of the impact on lifestyle comes as a result of side effects caused by prednisone.

Much of the impact on lifestyle comes as a result of side effects caused by prednisone.

  • 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 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.

Does stress have a negative affect on pemphigus and pemphigoid?

Many people who live with pemphigus report a direct relationship between increased stress and flares in disease activity. Others seem to be unaffected by increased stress.

The possible effect of stress on the onset or subsequent flare of pemphigus or pemphigoid is not clearly understood. Many people who live with P/P 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.

Does nutrition play a part in my “new” life?

Many nutrition concerns arise from the medication prednisone. In order to control an outbreak of pemphigus, a prompt response with a large dose of prednisone is generally prescribed.

Use of this drug 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.

Acidophilus, a beneficial bacteria found in yogurt, as well as in dietary supplements, is recommended for those who frequently use antibiotics. It also may helps prevent yeast infections.

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.

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. Be sure to discuss your present diet, medications, and lifestyle with your physician and/or dietician before making changes to your diet.

  • Nobody should avoid these foods, but consideration can be given on a case by case basis, as some patients report exacerbation of disease by certain food groups containing one or more of four ingredients: thiol, isothiocyanates, phenols or tannins.
  • Thiols: garlic and other members of the Allium group which contain plants such as onion, shallot, chive and leek.
  • Isothiocyanates (mustard oils): are found in 3200 species of plants including mustard, horseradish, winter cress, turnip, broccoli, radish, cabbage, brussel sprouts and cauliflower. Capers. Synthetic oil of mustard.
  • Phenols: Urushiol can cause contact dermatitis and is most notably found in poison ivy, poison oak and poison sumac which are related to mango, pistachio and cashew. The artificial sweetener aspartame is phenolic and common in many food additives. Phenol is in cinnamon and cinnamic acid, and pinene. It is in tomatoes, potatoes, mangos and bananas and milk and milk products produced when cows consume phenol laced feed such as cottonseed.
  • Tannins: Common sources of tannin are kola nuts, tea, coffee, raspberry, cherry, cranberry, blackberry, avocado, banana, apple, mango, pear, eggplant and grape skins, coffee and cocoa seeds, ginger, ginseng, garlic, rosemary, arrowroot,.