寻常型天疱疮（PV）是一种可能致命的上皮内自身免疫性疾病，其特征是皮肤和粘膜出现松弛性大疱和糜烂，每10万人中大约3人患病。组织学上，其特征为棘层松解。

PV可能导致皮肤和口腔，鼻子，喉咙，眼睛和生殖器区域的粘膜严重起疱。大疱位于表皮内，表面薄而脆弱，破溃后形成大面积糜烂面，自觉剧烈疼痛。

那么PV是如何发病的？怎么治疗？让我们继续看下去吧～

Pemphigus vulgaris (PV) is a potentially fatal autoimmune, intraepithelial disease characterized by flaccid blisters and erosions of the skin and mucous membranes, and  affects  every 3 in 100,000 people.  Histologically it is characterized by acantholysis. PV may cause severe blistering of the skin and the mucous membranes lining the mouth, nose, throat, eyes and genital area. Blisters develop in the upper layer of the skin and have a thin and fragile outer surface that breaks away easily, leaving raw areas (erosions) that can be extensive and very painful.

Now let's take a look at this disease in detail.

01

寻常型天疱疮是什么原因引起的？症状有哪些？

Causes and Symptoms

在寻常型天疱疮一类的自身免疫性疾病中，机体会产生自身抗体，攻击自体组织。寻常型天疱疮中形成的自身抗体攻击表皮细胞的连接结构：桥粒中的关键蛋白—桥粒芯蛋白。这会导致皮肤和粘膜中的细胞松解，从而产生寻常型天疱疮中的大疱和糜烂的典型皮损。
皮肤糜烂（溃疡）常伴随剧烈疼痛，并且影响生活质量，包括导致睡眠障碍。口腔和咽喉皮损会干扰进食和饮水，从而导致体重减轻。寻常型天疱疮还会影响其他部位粘膜，如生殖器区域，导致性交痛、排尿和/或排便疼痛。鼻部的PV皮损可能导致麻木感和局部结痂。当影响眼结膜时，它可能导致眼睛疼痛和红眼，需要由眼科医生评估。口腔中的PV迅速破溃形成糜烂。可能有单个或两个或几个融合在一起。

皮损早期为薄壁脆弱的大疱（疱内含透明液体），容易破溃，形成糜烂面（疼痛，烧灼感、可渗出液体或结痂、亦可能感染）。愈合后局部皮肤变色。
In an autoimmune disease, such as pemphigus vulgaris, the immune system makes antibodies (auto-antibodies) that work against tissues in the body. The auto-antibodies formed in pemphigus vulgaris attack the desmoglein proteins, which is the key protein in desmosome (cell juction). As a result, the cells in the skin and mucous membranes no longer hold together and separate. This causes blisters and erosions that are typical of pemphigus vulgaris.
Skin erosions (sores) are painful and can affect quality of life including sleep disturbances. Mouth and throat lesions can interfere with eating and drinking which may result in weight loss. Pemphigus vulgaris can also affect other mucous membranes such as the genital area, leading to painful sexual intercourse, urination and bowel movements. PV affecting the nose may cause stuffiness and crusts. When affecting  the conjunctiva of the eyes, it can  cause  sore and red eyes which require  assessment by an ophthalmologist. PV in the mouth  quickly burst to form erosions. There may be one or two, or several, that join together.
The skin lesions start as thin-walled fragile blisters (collections of clear fluid within the skin) that burst easily, leaving raw areas known as erosion (Sore, burn-like areas that can ooze fluid or become crusty and infected). Healing can result  in discoloration of the skin

02

如何诊断寻常型天疱疮？

How is pemphigus vulgaris diagnosed?

通过检查受影响的区域和口腔，皮肤科医生能够对寻常型天疱疮进行初步诊断。活检可确诊。直接免疫荧光检查可确定活检组织是否存在寻常型天疱疮自身抗体。
另一种方法是通过间接免疫荧光测量血液中寻常型天疱疮自身抗体的量。该方法可以确定疾病是否在活动期并且可以有助于确定治疗的疗效。

The specialists will be able to make a provisional diagnosis of pemphigus vulgaris by examining the affected area of skin and in the mouth. A biopsy is taken to confirm the diagnosis. The  biopsy  will be examined by a technique known as direct immunofluorescence which can demonstrate the presence of pemphigus vulgaris autoantibodies in the skin.
Another method is to measure the amount of Pemphigus vulgaris autoantibodies in the blood by indirect immunofluorescence. This method can determine the disease activity and can contribute to the determination in change of treatment.

03

寻常型天疱疮？

How can pemphigus vulgaris be treated?

PV治疗围绕糖皮质激素展开。

通常初始治疗方案由糖皮质激素及1种非糖皮质激素类药物组成。寻常型天疱疮通常首先口服糖皮质激素治疗。糖皮质激素疗效确切且比大多数其他治疗起效更快。高剂量的糖皮质激素，通常是泼尼松龙，常用于控制寻常型天疱疮病情。
非糖皮质激素类药物

糖皮质激素类药物在长期大剂量服用时会产生许多副作用。出于这个原因，常同时与其他药物连用以使糖皮质激素的剂量最小化。这些被称为非糖皮质激素类药物。它们包括以下几类药物：
1.免疫抑制药物：硫唑嘌呤，环磷酰胺，霉酚酸酯，甲氨蝶呤和利妥昔单抗。
2.可以使用的其他药物或治疗包括：静脉注射免疫球蛋白，四环素（米诺环素或多西环素），联合烟酰胺、氨苯砜和血浆置换术。
其他治疗

如果其他治疗无效，可考虑使用血浆置换和静脉注射免疫球蛋白治疗。它们可以联合口服糖皮质激素。
皮肤和口腔局部治疗

可以在皮肤皮损上使用保湿霜和强效糖皮质激素乳膏，以帮助减少系统糖皮质激素的用量。

Treatment usually starts with a corticosteroid and is often combined with a ‘steroid-sparing’ drug
Corticosteroids. Pemphigus vulgaris is usually treated first with an oral corticosteroid. Corticosteroids are effective and work quicker than most other treatments. High doses of corticosteroids, usually prednisolone, are given to control pemphigus vulgaris.
Steroid-sparing drugs. corticosteroids have many side effects when taken long-term at high doses. For this reason, other drugs are prescribed to allow the dose of steroid to be minimised. These are known as steroid-sparing drugs. They include the following:
1. Immunosuppressive drugs: azathioprine, cyclophosphamide, mycophenolate mofetil, methotrexate and rituximab.
2. Additional drugs or treatments that may be used include: intravenous immunoglobulins, tetracyclines (minocycline or doxycycline) combined with nicotinamide, dapsone and plasmapheresis.
Other treatments. Plasmapheresis and intravenous immunoglobulin therapy may be considered if other treatments are not effective. They may be used in combination with steroid tablets.
Skin and mouth topical treatments. Moisturisers and strong steroid creams may be used on skin blisters to help reduce the dose of steroid tablets.

虽然寻常型天疱疮是罕见病，一旦确诊，规律、长期的治疗对于控制疾病进展是非常重要的。如果有疑似寻常天疱疮的皮损或症状，请马上看皮肤科专科医生哦～

Although pemphigus vulgaris is a rare disease, it is important to remain on a regular and standardized treatment course to keep the symptoms of PV under control once diagnoised. In order to do so, if present with likely lesions or symptoms of PV, consult a dermatological specialist is of top priority.