Bay Biosciences provides high quality, clinical grade bio-samples, biopsy tissue samples with matched sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Pemphigus Vulgaris disease.
The PBMC, sera (serum) and plasma biofluid samples are centrifuged from patient’s peripheral whole-blood using customized processing protocols. The human Pemphigus Vulgaris bio-specimens are collected from unique patients diagnosed from Pemphigus Vulgaris and provided to a valued pharmaceutical customer for research, development and drug discovery.
Pemphigus Vulgaris Overview
Pemphigus Vulgaris is a rare, severe autoimmune disease that causes painful blistering and erosions on the skin and mucous membranes, most commonly inside the mouth. In an autoimmune disease, the immune system mistakenly attacks your own healthy tissues. Pemphigus Vulgaris is the most common type of a group of autoimmune disorders called pemphigus. Pemphigus Vulgaris occurs when the immune system mistakenly attacks proteins in the upper layers of the skin. Patients with Pemphigus Vulgaris disease have severe blistering in the mouth and on other areas of the body, and sometimes sheets of skin peel off. Each type of pemphigus is characterized by where the blisters form, in which part of the body.
Pemphigus can occur at any age, but it’s most often develops in people who are middle aged or older, the disease is more prevalent in people with Jewish and Spaniard ancestry. The association of pemphigus with human leukocyte antigen (HLA) is widely accepted. It was described in many ethnic groups and in most countries of the world. Studies showed that the associated HLA haplotype in Jewish pemphigus vulgaris (PV) patients is HLA-B38, DRB1*0402, and DQB1*0302; or HLA-B35, DRB1*0402, and DQB1*0302.
Pemphigus vulgaris tends to be a long-lasting (chronic) condition, and some types can be life-threatening without treatment. Treatment with medication usually controls it. Pemphigus is not a contagious disease. In most cases, it’s unknown what triggers the disease. Rarely, pemphigus is triggered by the use of angiotensin-converting enzyme (ACE) inhibitors, penicillamine and other drugs.
Pemphigus vulgaris affects the mucous membranes, which are found in areas including the:
- Mouth
- Throat
- Nose
- Eyes
- Genitals
- Lungs
Pemphigus vulgaris 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.
Pemphigus Vulgaris Signs and Symptoms
The symptoms of Pemphigus Vulgaris include:
Pemphigus causes blisters on the skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.
The signs and symptoms of common types of pemphigus are as follows:
- Painful blisters that start in the mouth or skin areas
- Skin blisters near the surface of the skin that come and go
- Oozing, crusting, or peeling at the blister site
- Pemphigus Vulgaris – This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful but don’t itch. Blisters in your mouth or throat may make it hard to swallow and eat.
- Pemphigus Foliaceus -This type causes blisters on the chest, back and shoulders. The blisters tend to be more itchy than painful. Pemphigus foliaceus doesn’t cause mouth blisters.
- Pemphigus Vegetans – Pemphigus vegetans causes blisters that appear on the groin, under the arms, and on the feet.
- Paraneoplastic Pemphigus – A very rare type of pemphigus that occurs in people with some cancers is called paraneoplastic pemphigus. The blisters and sores may appear in the mouth, on the lips, and on the skin. This type may also cause scars on the eyelids and eyes. It can also cause lung problems.
Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects older adults and may cause death.
Pemphigus Vulgaris Causes
The immune system produces proteins called antibodies. Antibodies normally attack harmful foreign substances like bacteria and viruses. Pemphigus Vulgaris occurs when the immune system mistakenly makes antibodies against proteins in healthy skin and mucous membranes. The antibodies break down the bonds between the cells, and fluid collects between the layers of the skin. This leads to blisters and erosions on the skin. The precise cause of the attack by the immune system isn’t known.
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. The 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 skin.
The antibodies produced by the immune system attack specific proteins that connect the epidermal cells or 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.
Pemphigus vulgaris (PV) is an acquired autoimmune disease in which IgG antibodies antibodies target desmosomal proteins to produce intraepithelial, mucocutaneous blistering. Desmoglein (Dsg3) is the major antigen, but 50–60% of patients have additional antibodies to Dsg1, the antigen in pemphigus foliaceus (PF). The underlying antibody profile is a major determinant of the clinical phenotype of PV.
Pemphigus Vulgaris Complications
Possible complications of pemphigus include:
Detailed clinical data, antibody biomarker Desmoglein-1 (DSG1), Desmoglein-3 (DSG3), genetic, biopsy, pathology annotations, associated with the Pemphigus Vulgaris patient’s specimens is provided to a valued customer for drug discovery, development and research. The Pemphigus Vulgaris disease sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples were processed from patients peripheral blood using customized processing protocols provided by the researcher.