Bay Biosciences provides high quality, clinical grade bio-samples, cryogenically preserved biopsy tissue, FFPE blocks, sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Bullous Pemphigoid (BP) disease.
The PBMC, sera (serum) and plasma biofluid samples are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The Bullous Pemphigoid bio-specimens are collected from unique patients diagnosed with Bullous Pemphigoid disease and are provided to a valued pharmaceutical customer for research, development and drug discovery.
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 people over 80 years of age, and mostly affects people over 50.
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
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.
Detailed clinical data, elevated levels of antibodies, serology, biomarkers, M2, genetic information, biopsy tissue, pathology annotations associated with the Bullous Pemphigoid disease patient’s specimens is provided to a valued customer for drug discovery, development and research.
The Bullous Pemphigoid disease sera (serum), plasma and PBMC biofluid samples are processed from patients peripheral whole-blood using customized collection and processing protocols provided by the researcher.
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