The bullous pemphigoid is a rare disease. They appear mainly on the limbs (previously under the sole of the foot sometimes) and are accompanied by intense itching. Bullous pemphigoid is the autoimmune form of bullous dermatoses, so called because of bullous lesions appearing in their clinical signs. It is a disease usually acquired, due to the effect of antibodies on the components of the skin, as desmosomes and hemidesmosomes, proteins of cohesion between cells and extracellular matrix. Rarely, the mucous membranes are affected (inside the mouth in 10 to 20. Co-morbidities and the tendency to use immunosuppressive drugs and / or systemic corticosteroids may also affect morbidity and overall mortality.
healing - in the absence of complications - is effective in an interval of 1 to 5 years.
Pruritus preceding or accompanying the appearance of large patches of red and many bubbles often exceeding 3-4. The lesion are generally symmetrical on both halves of the body. Diabetes and psoriasis are often associated, in two case-control studies. It does not seem to be any additional risk of cancer (same age).
Evolution.
The causes of this disease are still poorly understood. Like all autoimmune diseases, it implies that the immune system, rather than attack only elements ". In some cases, medication (spironolactone, bumetanide, fluoxetine, etc..) Could have been triggers for bullous pemphigoid, as well as photochemotherapy (PUVA combines psoralen and UV-A) and an epidemiological study (with case- control) has suggested the potential role of spironolactone and trigger any degree of neuroleptics.
Autoantibodies target two structural proteins of the skin. This is where the blisters are formed.
There are also other specific forms of autoimmune bullous dermatoses.
In severe cases (generalized forms or extended) the patient is hospitalized and treated in day hospital and outpatient. Two types of treatment are possible. In total, the case of 1 to 5 years of treatment were necessary (and this period is extended in case of complications).
In case (very rare) inefficient processing (cortico-resistance), the doctor offers immunosuppressants (methotrexate, azathioprine), which limit the dose of corticosteroids necessary in case of relapse.
In severe cases, psychological support is helpful and sometimes necessary.
In France, two hospitals are referral centers labeled and there is a group of research to better understand this disease.
Shingles Mastocytosis