Bay Biosciences provides high quality, clinical grade bio-samples, skin biopsy tissue samples, FFPE tissue blocks, sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with Pemphigus Vulgaris disease. The PBMC, sera (serum) and plasma samples are processed from patient’s peripheral whole-blood using customized processing protocols.
The human Pemphigus Vulgaris bio-specimens are collected from unique patients suffering from Pemphigus Vulgaris disease and provided to a valued pharmaceutical customer for research, development and drug discovery.
Pemphigus Vulgaris
Pemphigus Vulgaris is a rare severe autoimmune disease in which blisters and sores of varying sizes break out on the skin, genitals, the lining of the mouth and other mucous membranes. Pemphigus Vulgaris disease can occur at any stage, but most often develops in middle age or older people affecting equal number of men and women, it rarely develops in children. Pemphigus vulgaris tends to be a long-lasting, chronic condition, and some types can be life-threatening without treatment. Pemphigus vulgaris is a lifelong condition, and it cannot be cured. However, most people go into remission after receiving corticosteroids, improvement is usually noticed within days of starting the corticosteroids.
Pemphigus Vulgaris disease occurs when the immune system mistakenly attacks proteins in the upper layers of the skin. Patients develop severe blistering in the mouth and on other areas of the body, and sometimes sheets of skin peel off. Pemphigus Vulgaris disease can be diagnosed by examining skin biopsy samples under a microscope. Treatment usually involves corticosteroids or drugs that suppress the immune system.
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 disease, the body’s immune system mistakenly attacks the body’s own tissues, in this case, the patients own skin. The 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 called Bullous Pemphigoid.
Symptoms
The major symptom of Pemphigus Vulgaris disease is the development of clear, soft, and painful (sometimes tender) blisters of various sizes. In addition, the top layer of skin may detach from the lower layers in response to slight pinching or rubbing, causing it to peel off in sheets and to leave painful areas of open skin (erosions). Pemphigus Vulgaris disorder causes blisters and sores on the patients skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected. The blisters often first appear in the mouth and soon rupture, forming painful sores (ulcers). More blisters and ulcers may follow until the entire lining of the mouth is affected, causing difficulty swallowing, eating, and drinking. Blisters form in the throat as well.
Types of Pemphigus
- Pemphigus Vulgaris- This type of vulgaris disease usually begins with blisters in the mouth and then on the skin or genital mucous membranes. The blisters typically are painful but don’t itch. Blisters in the mouth or throat may make it hard to swallow and eat and creates discomfort.
- Pemphigus Foliaceus- This type of vulgaris disease causes blisters on the chest, back and shoulders. The blisters tend to be more itchy than painful. Pemphigus Foliaceus doesn’t cause blisters in the mouth.
- Pemphigus Vegetans- Pemphigus Vegetans causes blisters that appear on the groin, under the arms, and on the feet.
- Paraneoplastic Pemphigus is a very rare type of Pemphigus that occurs in people with some cancers. The blisters and sores may appear in the mouth, on the lips, and on the skin. This type of Vulgaris disease may also cause scars on the eyelids and eyes, it can also cause lung problems.
Pemphigus Vulgaris disease is distinct from Bullous Pemphigoid, which is a blistering skin condition that affects older adults and may cause death.
Complications of Pemphigus Vulgaris
The complications of Pemphigus Vulgaris can be severe and life threatening. The most common cause of death is a severe secondary infection. Possible complications of pemphigus include:
- Skin Infection
- Sepsis or the spread of Infection through the bloodstream
- Dehydration
- Malnutrition, because painful mouth sores make it difficult to eat
- Medication side effects, such as high blood pressure and infection
- Death, if certain types of pemphigus are left untreated
Treatment of Pemphigus Vulgaris
Treatment is aimed at reducing pain and symptoms and preventing complications like infection. It involves one or more drugs and other methods. It may include any of the following:
A high dose of corticosteroids is the core treatment for the condition, a high dose is usually needed to control the condition at first, with other medications that suppress the immune system. These drugs have many side effects, including:
- Increased likelihood of becoming infected
- Osteoporosis
- Cataracts
- Glaucoma
- High levels of blood sugar
- Diabetes
- Loss of muscle mass
- Stomach Ulcers
- Water retention
Plasmapheresis
In very severe cases, a person 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.
Detailed clinical data, elevated levels of antibodies, serology, biomarkers, Desmoglein-1 (DSG-1), Desmoglein-3 (DSG-3) genetic, pathology annotations, associated with the Pemphigus Vulgaris disease patient’s tissue specimens is provided to a valued customer for drug discovery, development and research. The Pemphigus Vulgaris disease sera (serum), plasma and PBMC samples were processed from patients peripheral blood using customized processing protocols provided by the researcher.
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