Bay Biosciences provides high quality, clinical grade, skin biopsy tissue samples, FFPE tissue blocks with matched cryogenically preserved sera (serum), plasma, peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with pemphigus vulgaris.
The sera (serum), plasma and PBMC biofluid specimens are processed from pemphigus vulgaris patient’s peripheral whole-blood using customized collection and processing protocols.
The pemphigus vulgaris sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluids are processed from patients peripheral whole-blood using customized collection and processing protocols.
Pemphigus Vulgaris (PV) Overview
PV is a rare autoimmune disease that causes blistering of the skin, of varying sizes break out on the skin and mucous membranes of the mouth, throat and genital area.
- Pemphigus vulgaris occurs when the immune system mistakenly attacks proteins in the upper layers of the skin.
- Patients with PV have severe blistering in the mouth and on other areas of the body, and sometimes sheets of skin peel off.
- Doctors can diagnose pemphigus vulgaris by examining skin samples under a microscope.
- Treatment usually involves corticosteroids or drugs that suppress the immune system.
Pemphigus vulgaris is the most common type of a group of autoimmune disorders called pemphigus. Each type of pemphigus is characterized by where the blisters form.
PV affects the mucous membranes, which are found in areas including the following:
- Eyes
- Genitals
- Lungs
- Mouth
- Nose
- Throat
This disease usually starts with blisters in the mouth and then on the skin. The blisters sometimes affect the membranes of the genitals.
Pemphigus vulgaris can be dangerous. Treatment is essential, and typically involves the use of corticosteroids to suppress the immune system. The condition can cause serious complications if it isn’t treated. Some of these complications can be fatal.
The death rate from this disease had averaged 75 percent before corticosteroids were introduced in the 1950s. This has improved dramatically with today’s treatments.
Autoimmune Disorder
Pemphigus vulgaris develops most often in middle-aged or older people, affecting equal numbers of men and women patients. PV rarely develops in children.
The body’s immune system makes special cells that protect the body against harmful foreign invaders such as bacteria and viruses. Some of these cells respond to invaders by producing proteins called antibodies.
Antibodies target and attach to the invaders and attract other cells in the immune system to destroy them. In an autoimmune disorder, the body’s immune system mistakenly attacks the body’s own tissues, in this case, the skin.
These antibodies produced by the immune system attack specific proteins that connect the epidermal cells (the cells in the top layer of skin) to each other. When these connections are disrupted, the cells separate from each other and from the lower layers of the skin, and blisters form.
Similar-appearing blisters occur with a less dangerous skin disorder known as bullous pemphigoid.
Signs and Symptoms of Pemphigus Vulgaris (PV)
The symptoms of pemphigus vulgaris include the following:
- Affected skin that peels easily when rubbed
- Blisters that are easy to burst
- Oozing, crusting, or peeling at the blister site
- Painful blisters that start in the mouth or skin areas
- Skin blisters near the surface of the skin that come and go
Causes of Pemphigus Vulgaris (PV)
Causes of pemphigus vulgaris is not fully understood. Experts believe that it’s triggered when a person who has a genetic tendency to get this condition comes into contact with an environmental trigger, such as a chemical or a drug. In some cases, pemphigus vulgaris will go away once the trigger is removed.
Pemphigus vulgaris is an autoimmune condition. This means that something goes wrong with the immune system (the body’s defense against infection) and it starts attacking healthy tissue.
The condition causes the immune system to fight against the body’s own cells in the same way that it fights off invading germs.
In pemphigus vulgaris, the immune system attacks cells found in a deep layer of skin, as well as cells found in the mucous membrane (the protective lining of the mouth, nostrils, throat, genitals and anus). This causes blisters to form in the affected tissue.
With pemphigus vulgaris, the immune system looks for proteins that bind the cells of the skin. This causes a buildup of fluid between the skin cells, resulting in blisters. Experts believe that the condition fights healthy proteins.
Diagnosis of Pemphigus Vulgaris (PV)
You may need to see a dermatologist to diagnose and treat pemphigus vulgaris. Your doctor visit may include the following:
- Blood tests
- Taking your medical history
- Physical exam
- Review of your symptoms
- Biopsy, which involves taking a tissue sample for testing
Treatment of Pemphigus Vulgaris (PV)
Treatment is aimed at reducing pain and symptoms and preventing complications such as infection. It involves one or more drugs and other methods.
Following are the are common treatments for pemphigus vulgaris:
Antibiotics, Antivirals, and Antifungals
Any of the above medications may be prescribed to prevent other infections.
Corticosteroids and Immune-Suppressing Drugs
A high dose of corticosteroids is the core treatment for the condition. Common corticosteroids include prednisone or prednisolone. A high dose is usually needed to control the condition at first.
These drugs have many side effects, including:
- An increased likelihood of becoming infected
- Cataracts
- Diabetes
- Glaucoma
- Increased blood sugar
- Loss of muscle mass
- Osteoporosis
- Stomach ulcers
- Water retention
PV patients may need to take supplements such as calcium and vitamin D, eat a low-sugar diet, or take other medications to treat these side effects.
Once the blisters are under control, the dose may be lowered to the lowest level needed to prevent new blisters and to keep the side effects at a minimum. A corticosteroid cream can be used directly on the blisters as well.
To help keep the dose of corticosteroids low, your doctor may prescribe additional medications that suppress the immune system.
These may include the following:
- Azathioprine
- Cyclophosphamide
- Mycophenolate mofetil
- Rituximab
- Methotrexate
Intravenous (IV) Feeding
If the patients mouth ulcers are severe, they may not be able to eat without pain. They may need to be fed through the veins, which involves using an intravenous (IV) connection.
Plasmapheresis
In very severe pemphigus vulgaris cases, a patient may undergo a procedure known as plasmapheresis. This procedure is intended to remove the antibodies attacking the skin from the blood. During this procedure, the plasma, or fluid part of the blood, is removed by a device and replaced with donated plasma. This treatment can be very expensive.
Wound Management
If the blisters are severe, the pemphigus vulgaris patient may need to stay in the hospital to get wound treatment. This treatment is similar to what’s given for severe burns. They may need to receive IV fluids and electrolytes if you’ve lost too much fluid through oozing of the blisters.
The treatment for the blisters may also include:
- Avoidance of too much sun exposure
- Numbing lozenges for mouth blisters
- Pain medications
- Soothing lotions
- Wet dressings
- Soft-food diets
- Avoidance of spicy or acidic foods that may irritate the blisters
If the blisters in the mouth keep the patient from brushing or flossing their teeth, they may need special oral health treatment to prevent gum disease and tooth decay. See your dentist to ask them about oral care.
Complications of Pemphigus Vulgaris (PV)
The complications of pemphigus vulgaris can be fatal and severe. Blisters may be painful. They may heal and leave dark patches on the skin for months.
Most patients with pemphigus vulgaris feel better with treatment. Without treatment, the condition can lead to severe pain and infection.
They may include the following:
- Skin infections
- Sepsis , or the spread of infection through the bloodstream
- Dehydration
- Side effects of medication
Serious side effects are possible with some medications. These side effects include the following:
- Chest pain
- Dizziness
- Headache
- Joint pain
- Nausea
- Upset stomach
- Weight gain
Prognosis of Pemphigus Vulgaris (PV)
If left untreated, pemphigus vulgaris can be life-threatening. The most common cause of death is a severe secondary infection.
Pemphigus vulgaris is a lifelong condition. It cannot be cured. However, most people go into remission after receiving corticosteroids. Improvement is usually noticed within days of starting the corticosteroids.
The blisters will heal slowly, especially the ones in the mouth. On average, the blisters stop forming in two to three weeks. Healing of the blisters takes six to eight weeks on average. However, full healing can sometimes take years. Some individuals may need to stay on a low dose of the medication for life.
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