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Patients nouvellement diagnostiqués

Pemphigus et Pemphigoïde ne sont pas contagieuses. Pas par le sang. Pas par des fluides. Pas de quoi que ce soit. Pemphigus et Pemphigoïde ne sont pas génétiques.

Pemphigus et Pemphigoïde sont rares, maladies auto-immunes qui touchent un très faible pourcentage de la population. Il y a plusieurs différentes maladies auto-immunes, et ils peuvent chacun affecte le corps de différentes façons. Pemphigus vulgaire, la plus courante des maladies pemphigus, affecte la peau et des muqueuses.

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Cloquage peut être accompagnée par une douleur intense, démangeaisons, Burning, et picotements. If extensive, cloquage peut entraîner une perte de fluide menaçant le pronostic vital, infection, et défiguration. PV peut également causer des dommages importants à la peau, y compris la perte des ongles et des altérations pigmentaires, faire la rapidité d'intervention et de traitement essentiels à la prévention de l'invalidité.

Pemphigus et Pemphigoïde sont des maladies chroniques qui, à l'exception de rare, ne s'améliorent pas sans traitement actif. Approches de traitement incluent une phase de contrôle et ensuite une phase d'entretien, avec la possibilité de rémission complète ou rechute de la maladie (torche).

Pemphigus et Pemphigoïde patients sont normaux, tous les jours des gens et peut vivre normal, vie quotidienne. Il faut du temps, traitement, et de la persévérance, mais il est possible.

Most Common Types

This information is used courtesy of Dr. Kristine Krafts at the University of Minnesota School of Medicine. http://www.pathologystudent.com/?p=3566.

Pemphigus vulgaire

“Pemphigus” is used in a very specific way to describe blistering disorders caused by autoantibodies against some part of the epidermis, which lead to disruption of the intercellular junctions (and hence bullae). Pemphigus vulgaire, not surprisingly, is the most common type of pemphigus (“vulgar-” comes from the Latin vulgaris, meaning the general public). It occurs primarily in adults between the ages of 30 and 60, and is characterized by big, flaccid bullae that burst easily (in most patients, you’ll see more ruptured, scab-covered bullae than intact ones). Patients often present first with oral bullae and ulcerations, and later develop bullae on the skin.

In this disease, patients have autoantibodies against desmogleins, which disrupt the connections between the squamous cells of the epidermis and cause very superficial, intraepidermal, fragile bullae. If you do immunofluorescence on the skin, you’ll see a kind of outlining of each individual epidermal cell (because there are autoantibodies bound to the junctions between the cells). Treatment consists of immunosuppressive agents; prognosis is variable, but many patients have a higher than normal mortality rate.

Bullous pemphigoid

This disease is called pemphigoid rather than pemphigus. It looks like pemphigus but really isn’t! Pemphigus is characterized by autoantibodies against the connections between epidermal cells. In bullous pemphigoid, patients have autoantibodies – but they are against the basement membrane of the epidermis, not against epidermal cell junctions. This means that the bullae are actually subepidermal, so they are less fragile than those of pemphigus vulgaris (if you see a patient with bullous pemphigoid, you’ll see lots of intact, tense bullae, rather than a bunch of ruptured bullae covered with scabs). The immunofluorescence pattern is correspondingly different – you’ll see just a line at the base of the epidermis (rather than the lace-like outlining of epidermal cells you see in pemphigus vulgaris).

Patients with bullous pemphigoid are generally elderly, and the clinical presentation varies a lot (but usually it doesn’t start in the mouth, like pemphigus vulgaris). It’s a less serious disease, usually, since the bullae often don’t rupture (so there’s less chance of infection and scarring).