What the Phemphigus Vulgaris and how to protec?

Answer 1

Do you mean "Pemphigus vulgaris"?
Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disease characterized by intraepidermal bullae and extensive erosions on apparently healthy skin and mucous membranes. Diagnosis is by skin biopsy and serum antibody titers. Treatment is with corticosteroids and sometimes immunosuppressants. Maintenance therapy is usually required. (Merck)
If not treated, pemphigus is fatal, due to overwhelming systemic infection. The most common treatment is the administration of oral steroids, especially prednisone. Recently, there has been great promise of surviving some forms of pemphigus (especially PNP) by using a pooled blood product known as gamma globulin or IVIG. Mild cases sometimes respond to the application of topical steroids. All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.
If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune supressant drugs is sometimes used in an attempt to halt the rapid progression of bronchiolitis obliterans. Some drugs used include solumedrol, cyclosporin, azathioprine, and in rare instances, extremely controlled use of thalidomide in eligible patients. Immune phoresis procedures are also a possible treatment.
If skin lesions do become infected, antibiotics may be used for treatment. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.(Wikipedia)
Consult a dermatologist.
Please see the web pages for more details and images on Pemphigus vulgaris.

Answer 2

Pemphigus, or commonly referred to as "PV", is an auto-immune blistering disorder of the skin and mucous membranes where those afflicted suffer from burn-like lesions all over their bodies. The sores may never heal in some cases or may not heal for extended periods of time. Some patients can coexist with the disease for years, while others will die a slow and complicated death.

Janet Lehne has it.

"Sometimes I feel like I'm a walking open sore, and believe it or not, I actually have a mild case." When she first realized something was wrong in 1983, she thought she was dying from AIDS. The common lesions now known to be associated with AIDS and HIV are very similar to a PV outbreak. "My doctors, in the early eighties, were convinced that I was symptomatic and suffering from an aggressive case of AIDS. It scared the hell out of me. I thought I was a dead person." But then she was told she had one of those unusually rare skin conditions that no one really knew much about. "What is PV?" would be one of the obscure, extra-credit questions for medical students of dermatology.

She says her scalp literally tears. Her gums bleed and blister before disintegrating. Janet says PV is usually recognized by its slow-progression of infections in the mouth.

Although PV is a very rare disorder, but left untreated, doctors say you'll die.

At the present time, there is no cure -- only treatments to lessen the effects of the disease and allow some patients to lead more productive lives. Those treatments, many quite successful, center on the use of steroids such as prednisone and other corticosteroid medications. Chemotherapeutic immuno-suppressive drugs such as Imuran and Cytoxan are also administered to offset the disabling and toxic side effects of long-term prednisone use.

Janet's physician, Dr. Greta Clarke, a dermatologist in Berkeley, California says their have been remarkable advances in the prognosis of patients since the discovery of the disease, "PV was lethal in the pre-steroid era, now it's manageable." The disease can usually be diagnosed through a biopsy that can essentially detect a "split" in the epidermal layer of the skin.

Like other auto-immune disorders, PV is very mysterious. The body literally attacks itself whereby suspect antibodies target a component of keratinocytes called "PVA". This month's issue of The PV Foundation News, reports that knowledge of this "target" has been greatly expanded. Researchers at the National Cancer Institute have successfully identified the DNA sequence of PVA and shown that it belongs to a family of cells called cadherins. These cadherins are commonly involved in maintaining the structure of organs, including the body's largest organ -- the skin. The discovery that PVA is in fact a cadherin, offers new insight into how PV works to destroy and disrupt normal bodily functions.

Very often, PV lesions are commonly misdiagnosed as simple mouth sores. The tissue on the gums goes through cycles of tearing-healing-tearing-bleeding. An ulcerated sore can multiply rapidly and take over the inside of the mouth.

But the mouth isn't the only target for the disease, just the starting point. Red, blistered patches can spring up anywhere on the body, especially in the area of the genitals. If the patient has a severe case, the patches can cover a large portion of the body "suffocating" the skin and eventually causing death.

Answer 3

Will he survive