새롭게 진단된 된 환자

Pemphigus 및 pemphigoid 되지 않습니다 전염. 혈액에 의해 하지. 체액에 의해. 아무것도 의해. Pemphigus 및 pemphigoid 유전 되지 않습니다..

Pemphigus 및 pemphigoid 드물다, 인구의 아주 작은 백분율에 영향을 주는 자가 면역 질환. 거기에 많은 다른 자가 면역 질환, 그리고 그들은 수 각 신체에에서 영향을 여러 가지 방법. Pemphigus vulgaris, pemphigus 질병의 가장 흔한, 피부 및 점 막에 영향을.

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젖어 심한 통증에 의해 동반 될 수 있습니다., 가려움, 레코딩, 그리고 쏘는. If extensive, 유체 손실 생명에 위협이 될 수 있습니다 물집, 감염, 그리고 외관. PV도 피부에 큰 피해를 일으킬 수 있습니다., 네일 손실 및 소성 변경 등, 장애의 예방에 필수적인 개입 및 치료의 적시성을 만들기.

Pemphigus와 pemphigoid는 만성 질환을, 드문 예외와 함께, 적극적인 치료를 하지 않고 개선 되지 않아. 치료 접근 제어 위상 등 다음 유지 관리 단계, 완전 한 죄 또는 질병 재발의 가능성 (플레어).

Pemphigus 및 pemphigoid 환자는 정상입니다., 매일 사람과 정상적인 살 수, 일상 생활. 그것은 시간이 걸립니다., 치료, 그리고 인 내, 그것이 가능 하지만.

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 vulgaris

“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 vulgaris, 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).