Accessibility Information

The Pemphigus and Pemphigoid Society is working to make our web site accessible to people with disabilities. If you find a page that is not yet accessible by a screen reader or other assisting device; if you have questions not addressed here; please contact us. We will provide you with any information we have in another format.

By telephone at 510-755-4266.

By mail at PPS, 15934 Hesperian Boulevard, #122, San Lorenzo, California, 94580.

Welcome to the Pemphigus & Pemphigoid Society website.

The Pemphigus & Pemphigoid Society is a 501(c)(3) nonprofit foundation dedicated to pemphigus and pemphigoid patients, and their caregivers. The PPS provides emotional support and ongoing education on treatments, medications and recovery strategies, so patients may regain control of their lives as soon as possible. The PPS also works to promote understanding of these two rare autoimmune diseases in the public and medical community. As the Pemphigus & Pemphigoid Society is an all volunteer foundation, 100% of your donations go entirely toward these goals.

Pemphigus and pemphigoid are a group of rare autoimmune skin diseases. In very simple terms, an autoimmune disease is when the body attacks itself. In the case of pemphigus, the body attacks the "glue" (desmogleins) that holds one layer of skin to another. This causes burn-like blisters (lesions) which will not heal, and may cover a significant area of the skin and/or mucous membrane. If untreated pemphigus is usually fatal; but most often it can be controlled with medications. Like all other autoimmune diseases, pemphigus and pemphigoid are not contagious. Except in drug-induced pemphigus and pemphigoid, the cause or causes are unknown.

There are three main types of pemphigus: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP).

There are two main types of pemphigoid: bullous pemphigoid (also know as mucous membrane pemphigoid), and ocular pemphigoid.

Who gets pemphigus or pemphigoid?

Definitive statistics on the incidence of these diseases are not available. Estimates are that pemphigus strikes about 30 people per million in the United States, slightly higher in nations around the Mediterranean Sea and the subcontinent (around India) and about 60 per million among Jews and Arabs. Pemphigoid is much more rare, there are about 1 case of pemphigoid for every 5 or 6 cases of pemphigus.

Although the onset for these diseases usually occurs in middle-aged and older adults, cases have also been documented in young adults and children as young as 6 months. Pemphigus and pemphigoid affect people across all racial, cultural and economic lines. Research suggests a genetic predisposition to these diseases, although many more people have the genetic marker than ever get them. Incidences among family members is extremely rare. Unlike other autoimmune diseases, where women are 80% of the affected population, pemphigus and pemphigoid afflict both sexes equally. Because they are so rare, a correct diagnosis usually takes months, even years. In a survey of patients, the average time before a correct diagnosis is found, was four months.

If you have any persistent skin or mouth lesions, consult your dermatologist. There are many diseases which cause blisters. The only way to confirm a diagnosis of pemphigus or pemphigoid is by specific laboratory tests. Early diagnosis may permit treatment with lower levels of medication.

How is pemphigus and pemphigoid treated?

Mild cases of pemphigoid may respond to topical steroid creams, but usually the first line of defense for is systemic corticosteroids (steroids, for short); usually prednisone or prednisolone. Immunosupressive drugs (which reduce the body's natural immune response), such as Imuran and CellCept, are used with steroids; and the combination is very successful in controlling outbreaks (flares).

Treatment should always be addressed according to the disease activity. It should be treated "crisply" with the smallest dose of steroids required to control blistering, most patients start with 60 milligrams to 80 milligrams. Higher doses may cause more trouble than adjunctive or alternative treatments. Once the outbreak is under control (no new blisters) tapering may begin. Tapering must be done slowly over many months and under a doctor's supervision. Tapering too quickly may result in a relapse. Raising and lowering the dosage of prednisone is not recommended as it will cause the disease to "harden" and become more difficult to treat.

All of the drugs used to treat pemphigus and pemphigoid may have serious side effects. Lab tests are required on a regular basis to monitor blood and other factors. A direct immunofluorescent test (DIF, an antibody titer count) will generally show a high count when the disease is more active and low or undetectable when the disease is in remission. The DIF may be most useful with patients on maintenance doses of medications. If a titer count is low, then it could be reassuring that the flare is controllable and short. A high titer might raise concern and call for early intervention.

Alternative treatments for pemphigus or pemphigoid.

There is no proof that alternative, homeopathic or any other nontraditional method of treatment has been successful in treating pemphigus. It is imperative that traditional treatment be administered for the best possible results. Once the disease is under control, other methods may be applied along with traditional methods to possibly help reduce the side effects of the drugs. Discuss with your doctor, any herbs or other nontraditional treatments before you take them. Some of these products will stop or reduce the effectiveness of prescribed medicines. In fact, some products have been shown to aggravate the disease and put patients in the hospital.

How the PPS can help you.

Pemphigus in any form can be painful and terrifying; and leave one with feelings of hopelessness and isolation. Pemphigoid is not much better. We can recommend a doctor for treatment or consultation. The Pemphigus & Pemphigoid Society offers a support network, along with the latest information about treating these diseases. We can provide many tools and suggestions to prepare you for the road ahead. We urge you to contact us to get answers for any questions you may have.

Thank you.

Sal Capo.

President and Executive Director.