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Autoimmune bullous dermatoses include pemphigus, bullous pemphigoid, pemphigoid gestationis, linear IgA dermatosis, mucous membrane, lichen pemphigoid, anti-p200 pemphigoid, epidermolysis bullosa acquisita and dermatitis herpetiformis. Autoimmune bullous dermatoses are rare and have an incidence of 20-60 new cases per 1 million person- year in Europe. The incidence of the individual entities is significantly different within Europe, but also in comparison to other countries such as Kuwait, Singapore, USA and South America. The most common of these disorders is the bullous pemphigoid.
A considerable progress has been made in the last years to elucidate the pathogenic role of autoantibodies in these diseases. To this end, various in vitro and animal experiments have been used to understand some basic pathophysiological mechanisms in these diseases. Further studies are currently being carried out to explain a precise elucidation of the disease process and to be able to treat the patients targeted later.
At present, however, no data are available to explain why certain individuals develop the autoimmune disease and others do not. Epidemiological studies showed some triggers to the development of autoimmune dysregulation, e.g. drugs.
Furthermore, it has been shown that genetic factors play a role in the pathogenesis of the disease. A clear association with certain HLA regions have been shown in patients with pemphigus, e.g. about 95% of pemphigus patients from the group of Ashkenazi Jews have the HLA-DRB1*0402 haplotype. Recently, the first non-HLA gene associated with pemphigus was described. For other conditions such as bullous pemphigoid, pemphigoid gestationis or linear IgA dermatosis the association with HLA antigens is less pronounced. Another indication of the importance of the genetic background in these diseases can be elucidated from the observation of autoantibodies at a low concentration in healthy relatives of pemphigus patients.
Study genetic and microbiologic predisposition of patients developing a bullous pemphigoid from peripheral blood and skin flora.
Allocation: Non-Randomized, Intervention Model: Parallel Assignment, Masking: Open Label, Primary Purpose: Basic Science
Blood sample and cotton skin swabs
Chu de Reims
CHU de Reims
Published on BioPortfolio: 2016-08-23T12:23:22-0400
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease of the skin in European countries, including France. Immunologically, BP is characterized by the productio...
Patients diagnosed with bullous pemphigoid were confirmed based on the investigators national diagnostic criteria. Patients who meet all inclusion criteria and conflict the exclusion crite...
Bullous pemphigoid affects mainly elderly patients. It is often associated with neurologic disorders, which represent a major risk factor of the disease
The aim of the study is to assess that it will be possible to control with a single cycle of rituximab patient with bullous pemphigoid.
Bullous pemphigoid (BP) is the most common blistering auto-immune disease of skin with an incidence estimated to 400 new cases per year. Topical corticosteroid therapy is considered the st...
Bullous pemphigoid is a potentially life-threatening autoantibody-mediated dermatosis characterized by blister formation. Experimental mouse models of bullous pemphigoid feature complement-induced inf...
The clinical features of bullous pemphigoid are extremely polymorphous. Several atypical forms of bullous pemphigoid have been described, and the diagnosis critically relies on immunopathological find...
Bullous pemphigoid is a type of acute or chronic autoimmune disease that involves subepidermal skin lesions with bulla formation. Although viral infections, such as, human herpes virus (HHV), human im...
The widespread use of immune checkpoint inhibitors has shed light to several unusual immune-related adverse effects of the drugs. Severe cutaneous adverse reactions are generally rare with anti-PD1 ag...
We identified Th1/Th17 cell responses against desmoglein 3 and bullous pemphigoid antigen 180 in lichen planus. In contrast, patients with pemphigus vulgaris and bullous pemphigoid showed significantl...
An itching, autoimmune, bullous SKIN disease that occurs during the last two trimesters of PREGNANCY and PUERPERIUM. It also affects non-pregnant females with tissue of PLACENTA origin, such as CHORIOCARCINOMA; or HYDATIDIFORM MOLE. It exhibits antigenic and clinical similarity to bullous pemphigoid (PEMPHIGOID, BULLOUS). This disease does not involve herpes viruses (old name, herpes gestationis).
A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.
A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement.
A TEXTILE fiber obtained from the pappus (outside the SEEDS) of cotton plant (GOSSYPIUM). Inhalation of cotton fiber dust over a prolonged period can result in BYSSINOSIS.
The fraction of a blood sample, following CENTRIFUGATION, that is distinguished as a thin light-colored layer between the RED BLOOD CELLS, underneath it, and the PLASMA, above it. It is composed mostly of WHITE BLOOD CELLS and PLATELETS.
Autoimmune disorders are conditions that occurs when the immune system mistakenly attacks and destroys healthy body tissue. There are more than 80 different types of autoimmune disorders. Normally the immune system's white blood cells help protect ...
The term allergy is used to describe a response, within the body, to a substance, which is not necessarily harmful in itself, but results in an immune response and a reaction that causes symptoms and disease in a predisposed person, which in turn can cau...