Bay Biosciences provides high quality, clinical grade, fresh frozen biopsy tissue samples, FFPE tissue blocks with matched cryogenically preserved sera (serum), plasma, peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with Bullous Pemphigoid (BP).
The sera (serum), plasma and PBMC biofluid specimens are processed from Bullous Pemphigoid patient’s peripheral whole-blood using customized collection and processing protocols. The Bullous Pemphigoid biopsy tissue and matched biofluid samples are collected from unique patients diagnosed with Bullous Pemphigoid and are provided to a valued pharmaceutical customer for research, diagnostics, discovery and drug development.
Detailed clinical data, patient’s history, symptoms, complete blood count (CBC), serology, MRI, biopsy tissue findings, histopathology information, elevated biomarker levels, genetic and metabolic information associated with the Bullous Pemphigoid specimens is provided to a valued customer for research, development and drug discovery.
The Bullous Pemphigoid sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluids are processed from patients peripheral whole-blood using customized collection and processing protocols.
Bullous Pemphigoid Overview
Bullous Pemphigoid is a rare autoimmune skin (sub-epidermal) disease that causes large, fluid-filled blisters. They develop on areas of skin that often flex, such as the lower abdomen, upper thighs or armpits. Bullous pemphigoid is most common in patients older male and female adults. Bullous pemphigoid often occurs in elderly patients between the ages of 60 to 80 years.
Bullous pemphigoid (BP) is a chronic blistering of the skin. It ranges from mildly itchy welts to severe blisters and infection, and may affect a small area of the body or be widespread. Bullous pemphigoid (BP) is the most common autoimmune sub-epidermal blistering disorder, representing 80% of sub-epidermal immuno-bullous cases. The vast majority of those affected are elderly, but it has been seen at all ages.
Bullous Pemphigoid (BP) is an autoimmune disease, which means it is caused when the body’s immune system malfunctions. The immune system is meant to defend the body against bacteria, viruses, and disease, but instead produces antibodies against healthy tissue, cells and organs. Bullous Pemphigoid (BP) occurs when the body’s own immune system attacks the layer of tissue below the top layer of skin. The reasons for this attack are not known, but bullous pemphigoid can sometimes be triggered by certain medications, including penicillamine.
Some patients with bullous pemphigoid (BP) have other autoimmune disorders such as diabetes and rheumatoid arthritis. Various other factors have been reported to play a role in triggering bullous pemphigoid (BP). These include drugs such as furosemide, penicillin’s etc., mechanical trauma, and physical traumas such as burns from sun, heat or radiation.
Bullous pemphigoid occurs when the immune system attacks a thin layer of tissue below the outer layer of skin. The reason for this abnormal immune response is unknown, although it sometimes can be triggered by taking certain medications. Bullous Pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins antibodies and activated T-lymphocytes, white blood cells. The target is the protein BP180, also called Type XVII collagen, or less frequently BP230, a plakin. These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal.
Signs and Symptoms of Bullous Pemphigoid
Bullous pemphigoid can present with itching, hive-like welts, and multiple blisters, called bullae. Blisters are usually located on the arms, legs, or middle of the body. In rare cases, blisters can form in the mouth. The blisters may break open and become an open sore or ulcer. The fluid inside the may be clear, or contain some blood. The skin around the blisters can appear either normal or red. The blisters are usually located along creases in the skin, these are most commonly seen on the:
- Arms
- Legs
- Abdomen
- Groin
- Mouth
The signs and symptoms of Bullous Pemphigoid (BP) may include:
- Itching skin, weeks or months before blisters form
- Large blisters that don’t easily rupture when touched, often along creases or folds in the skin
- Skin around the blisters that is normal, reddish or darker than normal
- Eczema or a hive-like rash
- Small blisters or sores in the mouth or other mucous membranes (benign mucous membrane pemphigoid)
Other variable features include:
- Nonspecific rash for several weeks before blisters appear
- Eczematous areas resembling nummular dermatitis
- Urticaria-like red skin
- Annular, ring-shaped lesions
- Smaller blisters, vesicles
- Prurigo nodules, Pemphigoid Nodularis
- Clear or cloudy, yellowish or bloodstained blister fluid
- Post-Inflammatory Pigmentation
- Milia (small bump like cyst) in healed areas
Bullous Pemphigoid may be localized to one area, or widespread on the trunk and proximal limbs, frequently it affects the skin around skin folds, blisters inside the mouth and in genital sites are uncommon. Some patients have a diagnosis of Bullous Pemphigoid made despite not having any bullae (Non Bullous Pemphigoid), this can affect any site on the body.
Causes of Bullous Pemphigoid
The blisters occur because of a malfunction in the immune system. The body’s immune system normally produces antibodies to fight bacteria, viruses or other potentially harmful foreign substances. For reasons that are not clear, the body may develop an antibody to a particular tissue in the body. In Bullous Pemphigoid (BP) disease, the immune system produces antibodies to the fibers that connect the outer layer of skin (epidermis) and the next layer of skin (dermis). These antibodies trigger inflammation that produces the blisters and itching of Bullous Pemphigoid.
Bullous Pemphigoid usually appears randomly with no clear factors contributing to the onset of disease. Some cases may be triggered by:
- Medications: Prescription drugs may cause Bullous Pemphigoid (BP) disease.
- Light and radiation: Ultraviolet (UV) light therapy to treat certain skin conditions may trigger Bullous Pemphigoid disease, as can radiation therapy to treat cancer.
- Medical conditions: Diseases that may trigger Bullous Pemphigoid include psoriasis, lichen planus, diabetes, rheumatoid arthritis, ulcerative Colitis and multiple sclerosis.
Diagnosis of Bullous Pemphigoid
To confirm the bullous pemphigoid diagnosis, your doctor may order blood tests and take a small sample of the affected skin or skin biopsy for laboratory testing. The diagnosis may not be clear at first, since there can be other causes of skin blisters and mouth erosions. Also, the early symptoms, before blisters appear, may look like eczema or allergy. If your doctor suspects that you have bullous pemphigoid, they will usually refer you to a skin specialist (dermatologist) or eyes (ophthalmologist), depending on the patients signs and symptoms and the results of the lab tests.. Following diagnostics tests are usually performed to confirm the diagnosis of bullous pemphigoid:
- Biopsy– A skin biopsy samples may be taken, which is looked at under the microscope and tested to confirm that the blisters are due to bullous pemphigoid.
- Blood tests: Blood tests can detect the antibody that causes bullous pemphigoid (the bullous pemphigoid auto-antibody). The antibody may also be detected in urine or in blister fluid.
Treatment of Bullous Pemphigoid
Bullous Pemphigoid treatment is mostly focused on healing the skin and relieving itching, while minimizing adverse side effects of medications. Usually the doctors prescribe one or a combination of the following drugs for the treatment of bullous pemphigoid:
- Corticosteroids: The most common treatment is prednisone, which comes in pill form. But long-term use of corticosteroids can increase the risk of weak bones, diabetes, high blood pressure, high cholesterol and infection. Corticosteroids ointment can be rubbed on the affected skin and causes lesser side effects.
- Steroid-sparing drugs: These drugs affect the immune system by inhibiting the production of the body’s disease-fighting white blood cells. Examples of steroid-sparing drugs include azathioprine (Azasan, Imuran) and mycophenolate mofetil (CellCept). If the patient’s signs and symptoms involve the eyes or upper digestive tract, the drug rituximab (Rituxan) may be used, specially in cases where the other treatments are ineffctive.
- Anti-inflammatory drugs: Sometimes anti-inflammatory drugs are given to patients to fight inflammation, for examples methotrexate also known as Trexall.
Outlook (Prognosis) of Bullous Pemphigoid
Bullous pemphigoid usually responds well to treatment. The medicine can often be stopped after several years. The disease can sometimes return after treatment is stopped, but most of the time the outlook (prognosis) is generally good. Bullous pemphigoid often goes away between 1-5 years after treatment. Meanwhile, treatment usually keeps the blisters away or down to a tolerable level. Often, treatment can be stopped after about 1-5 years, as it is no longer required.
Bullous pemphigoid can sometimes cause serious illness, which may cause death. This is because:
- Serious infection of raw skin is dangerous. Bullous pemphigoid most commonly affects older people, who are more prone to develop serious illness if a skin infection occurs.
- Side-effects from steroids may be a problem and can sometimes be serious.
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