Bay Biosciences provides fresh frozen (-80°) serum, plasma, peripheral blood mononuclear cells (PBMC) biofluid samples from unique patients diagnosed with Bullous Pemphigoid (BP) disease. The fresh frozen (-80°) serum, plasma, peripheral blood mononuclear cells (PBMC) samples were collected from unique Bullous Pemphigoid (BP) patients before and after Intravenous Immunoglobulin (IVIG) treatment.
Bullous Pemphigoid (BP) Overview
Bullous Pemphigoid is a rare autoimmune chronic skin disorder characterized by blistering, urticarial lesions (hives) and itching. Less commonly these blisters can involve the mucous membranes including the eyes, oral mucosa, esophagus and genital mucosa.
Bullous Pemphigoid is a rare autoimmune disorder that can develop at any age, including in kids, but that most often affects the elderly. Pemphigoid is caused by a malfunction of the immune system and results in skin rashes and blistering on the legs, arms, and abdomen. Bullous Pemphigoid can also cause blistering on the mucous membranes. Mucous membranes produce mucous that helps protect the inside of your body. Pemphigoid can be found on the mucous membranes in your eyes, nose, mouth, and genitals. It can also occur during pregnancy in some women. There’s no cure for Bullous pemphigoid, but there are various treatment options available.
Bullous Pemphigoid causes large blisters filled with fluid. The blisters develop on areas of skin that often flex (flexural areas), such as the lower abdomen, upper thighs or armpits. Bullous Pemphigoid disease is most common in older adults. Bullous Pemphigoid can be life threatening, generalized intensely itchy blistering skin condition specially for older patients who are already in poor health and frail.
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.
Signs and Symptoms of Bullous Pemphigoid (BP)
Bullous Pemphigoid (BP) can present with itching, hive-like welts, and multiple blisters, called bullae. These are most commonly seen on the patients:
- Itching of the skin
- Bullae on the arms
- Armpits (axillae)
- Bullae on the Legs
- Bullae in the skinfolds
- Mouth
- Throat
- Abdomen
- Groin
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.
The blisters have certain characteristics, regardless of where on the body they form:
- A red rash develops before the blisters
- The blisters are large and filled with fluid that’s usually clear, but may contain some blood
- The blisters are thick and don’t rupture easily
- The skin around the blisters may appear normal, or slightly red or dark
- Ruptured blisters are usually sensitive and painful
Causes of Bullous Pemphigoid (BP)
Bullous Pemphigoid (BP) is an autoimmune disease. This means that your immune system mistakenly begins to attack your healthy tissues. In the case of Bullous Pemphigoid, your immune system creates antibodies to attack the tissue just below your outer layer of skin. This causes the layers of skin to separate and results in painful blistering. It’s not fully understood why the immune system reacts this way in patients living with Bullous Pemphigoid.
In many cases, there’s no specific trigger for bullous pemphigoid. In some instances, it may be caused by:
- Certain medications
- Radiation therapy
- Ultraviolet light therapy
Patients with other autoimmune disorders are found to be at a higher risk for developing bullous pemphigoid. It’s also more common in the elderly than in any other age group, and seems to occur equally in women and men.
In Bullous Pemphigoid (BP) disease, an autoantibody binds to a component of the skin that holds the dermis and epidermis together, causing separation of these two layers, thus forming a blister. The autoantibodies recognize components of the basement membrane zone called BP antigen 1 and 2 (and in some cases other basement membrane zone antigens). These proteins are part of complexes that hold the skin together, called hemidesmosomes, and provide structural support to the skin. When the body “attacks” these proteins, the skin becomes more fragile and the clinical manifestations of Bullous Pemphigoid (BP) become apparent.
Certain drug reactions can produce skin lesions that are very similar to BP. It is essential to determine whether the patient’s symptoms are adverse reactions to the pharmaceuticals, or whether the blisters are the result of an autoimmune reaction.
Diagnosis of Bullous Pemphigoid (BP)
Diagnosis of Bullous Pemphigoid (BP) disease is made based on a combination of clinical interpretation and laboratory test findings.
- Physical Examination: During a patients physical exam a doctor will suspect the diagnosis of BP in patients with itchy, red, hive-like patches, with or without blistering in elderly patients.
- Skin Biopsy: A sample of skin is taken from the edge of a blister, stained, and then examined under a microscope.
- Direct immunofluorescence (DIF): DIF is considered the gold standard for diagnosis of the skin and dermatological disorders. A biopsy is taken from normal appearing skin adjacent to a lesion. In Bullous Pemphigoid (BP), DIF will show linear localization of immunoglobulin (IgG) and/or complement protein, C3, along the dermal-epidermal junction, also known as the basement membrane. DIF is a procedure that utilizes labeled antibodies to detect immunoglobulin binding and complement deposits in tissue.
- Enzyme-linked immunosorbent assay (ELISA): ELISA test can detect autoantibodies in the serum that are specific to bullous pemphigoid. Detection of anti-BP180 antibodies occurs in 75-90% of patients with BP. Detection of anti-BP230 antibodies occurs in 50-70% of patients with BP. Anti-BP180 antibody levels correlate to disease activity and can be used not only to help diagnose BP but to also determine response to treatment.
Detailed clinical data and pathology annotations including patient’s corresponding diagnostic test values, MG-ADL score, Creatine Kinase data are provided to a valued pharmaceutical customer for research and drug discovery.
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