Etiology: The etiology of this disease is not clear. At present, most scholars think it may be autoimmune disease, because indirect immunofluorescence examination shows that there are specific antibodies (also known as pemphigus antibodies) in patients' serum, mainly IgG, and the titer of pemphigus antibodies in serum is parallel to the severity of the disease. Histologically, the reaction site of pemphigus antibody is the onset site of pemphigus (the site where spinous process separation occurs). Antibodies act on the junctions between epidermal cells.

Clinically, this disease usually occurs at the age of 30-50. There is no obvious gender difference.

At present, most scholars advocate the lever classification, that is, 1. Pemphigus vulgaris accounts for about 2/3. 2. Deciduous pemphigus. Erythematous pemphigus accounts for about 15%. Hypertrophic pemphigus accounts for about 3%.

(1) pemphigus vulgaris; Blisters as big as soybeans and broad beans suddenly appeared on the skin, sometimes as big as walnuts. The blister wall is thin and loose, and the blister fluid begins to clear. Later, it was turbid and bloody. After the blister breaks, it will form blister liquid scabbing, erosion, and often has a specific stench. Under the action of external force, the epidermis is easy to peel off, or the intact bulla is gently pressed, and the blister fluid spreads around from the pressing point in the bulla, which is positive for Nikolski sign. Its mechanism is due to the unwinding of epidermal spinous cells and the lack of cohesion of epidermal cells.

Generally, there is only mild itching, and there is still pigmentation after treatment, which will not leave scars.

About 80% patients involve oral cavity, vulva and conjunctiva, and the stubborn erosion of oral mucosa is easy to cause massive bleeding and obvious burning pain, which is often the early symptom of this disease.

Herpes can occur in all parts, especially in the back, armpits and groin.

Blisters spread all over the body, with a wide range of ulceration, which is easy to be complicated with infection, fever, general weakness and other symptoms.

(2) Hypertrophic pemphigus: It is an atypical form of pemphigus vulgaris, and skin lesions mostly occur in the head, face, armpit, chest and back. At first, it was a loose blister with positive Nikolski sign. After rupture, erosion, nipple hyperplasia, unclean surface, scab and stench are formed.

It often invades oral cavity, nasal cavity, labia and anal mucosa, and blisters are easy to open into rotten surfaces.

Conscious symptoms are not obvious, sometimes accompanied by systemic symptoms such as high fever. The lesions are light and heavy, and often last for many years.

Some people think that proliferative pemphigus can be divided into two types: ① severe pemphigus (neumann type) and pemphigus vulgaris. ② Mild (Hallopeau type) has no obvious blisters, only small pustules and papillary hyperplasia appear at the rubbing site. It is difficult to distinguish it from proliferative dermatitis clinically. Direct immunofluorescence examination showed that IgG was deposited between epidermal cells of the disease.

(3) Deciduous pemphigus: Relaxed blisters or erythema appear on normal skin, which is characterized by easy rupture and greasy scab, local or extensive peeling and foul smell. Sometimes the affected skin is red, swollen and crusted, similar to exfoliative dermatitis. Nicolschi's sign is positive.

Skin lesions mostly occur in the head, face, chest and back. Oral mucosa is rarely involved. Self-conscious itching, systemic symptoms vary.

(4) Erythematous pemphigus: The disease, also known as Seward-Ehrlich syndrome and seborrheic pemphigus, is a special form of deciduous pemphigus, mainly erythema and small blisters. Nicholski's sign can be covered by scales and scabs, and it usually occurs in the head, cheeks and chest and back. The distribution of facial lesions is mostly butterfly erythema, which resembles lupus erythematosus. Head, chest and back are covered with seborrheic scabs, similar to seborrheic dermatitis.

Generally, it rarely spreads to the whole body, with good prognosis, no involvement of mucosa and mild systemic symptoms. The disease will eventually develop into deciduous pemphigus.

Treatment of pemphigus based on syndrome differentiation

The syndrome of damp-heat type of heart and spleen is characterized by bullous rash, large erosive surface and more exudation, often accompanied by mucosal damage (more common in common pemphigus and proliferative pemphigus, especially in acute attack). Often accompanied by body heat, upset, thirst, anorexia, fatigue, tongue coating erosion, short and red urine, dry stool. The tongue is red with yellow or white fur, and the pulse is full or smooth.

Treatment of clearing heat, promoting diuresis and detoxicating.

Herbs on the prescription

Modified Jianpi Qushi Recipe (Janice et al. "Medical Zong Jin Jian")

Prescription: Rhizoma Smilacis Glabrae 30g, Radix Rehmanniae 25g, Fructus Forsythiae, Herba Artemisiae Scopariae15g, Radix Scutellariae, Fructus Gardeniae, Alismatis Rhizoma, Fructus Aurantii12g, Rhizoma Atractylodis Macrocephalae, Rhizoma Atractylodis, Folium Bambusae 9g and Radix Glycyrrhizae 6g. Decoct with water, daily 1 dose.

For patients with constipation, add yellow powder 12g (lower back) and sodium sulfate 15g (red). For those with strong fire toxicity, remove Atractylodis Rhizoma and Atractylodis Rhizoma, add 9 grams of Coptidis Rhizoma, 30 grams of gypsum (first decocted), and 0/2 grams of Anemarrhena rhizome/kloc-.

Spleen deficiency and dampness excess syndrome is characterized by blisters, sparse bullae, occasional new blisters, reddish rotten surface but not fresh, more exudation, and thicker yellowish brown scab or papillary hyperplasia (more common in the chronic stage of common and proliferative pemphigus). Often accompanied by pale or sallow complexion, loss of appetite, fatigue and weakness, soft stool. Red tongue, white and greasy fur, and slow pulse.

Treatment of spleen dampness.

Herbs on the prescription

The main prescription, Baizhu Powder (Chen et al. "Taiping Huimin Mixture Bureau Prescription") is modified.

Prescription: Radix Codonopsis 25g, Poria, Coicis Semen 30g, Atractylodis Rhizoma, Lentils, Yam 15g, Rhizoma Atractylodis 12g, Pericarpium Citri Tangerinae, Radix Glycyrrhizae Preparata 5g and Fructus Amomi 6g. Decoct in water, and then decoct the residue, daily 1 dose.

The syndrome of yin injury and fluid deficiency is characterized by erythema, scales and scabs, with a small amount of exudation (more common in deciduous pemphigus and erythematous pemphigus). Accompanied by dry mouth and throat, irritability, difficulty sleeping at night and dry stool. The tongue is red with no or little coating, and the pulse is thin and astringent.

Treating yin, nourishing body fluid and moistening dryness.

Herbs on the prescription

Angelababy Decoction (Wang Kentang's standard of syndrome differentiation and treatment) and Zengye Decoction (Wu Jutong's Treatise on Febrile Diseases) were modified.

Prescription: 9 grams of Angelica sinensis, 0/5g of Radix Rehmanniae, Radix Rehmanniae Preparata, Radix Paeoniae Alba, Rhizoma Polygonati Odorati, Flos Lonicerae12g of Radix Scutellariae, Radix Scrophulariae, Radix Ophiopogonis12g, and 6g of Radix Glycyrrhizae. Decoct in water, and then decoct the residue, daily 1 dose.

For patients with deficiency of both qi and yin, 30g of Radix Pseudostellariae can be added, or 6-9g of Radix Panacis Quinquefolii can be stewed separately.

Description: This disease is serious. During acute attack, it is best to take comprehensive treatment measures of traditional Chinese and western medicine. TCM treatment based on syndrome differentiation is as above. Corticosteroid hormone is the first choice for western medicine, and the initial dose is large. Calculated by prednisone, the initial dose is generally 60 ~ 80mg/ day (90 ~ 120mg/ day if necessary). It can also be converted into dexamethasone or hydrocortisone injection and added with 500 ~ 10% glucose solution for intravenous drip. After the disease is controlled, it will be gradually reduced and changed to oral administration. If necessary, it can also be used in combination with immunosuppressants (such as azathioprine and cyclophosphamide). If there is a secondary infection, antibiotics should be used. In addition, supportive therapy is also very important, such as giving a high-protein and nutritious diet, supplementing vitamins, infusion, giving energy mixture, paying attention to the balance of water and electrolyte, blood transfusion or plasma transfusion if necessary, or using human albumin and gamma globulin. Taking comprehensive treatment measures of traditional Chinese and western medicine is conducive to improving curative effect and reducing mortality.

Treatment]: purging fire and removing dampness, nourishing yin and benefiting qi.

Chen Yin 15 Xie Jie 15 Cortex Dictamni Radicis 15 Atractylodis Rhizoma 9 Coicis Semen 18 Ophiopogon japonicus 12 Scrophulariaceae 12.

Dendrobium nobile 12 Radix Pseudostellariae 15 Radix Salviae Miltiorrhizae 12 Cortex Phellodendri 12 Lentils 15 Flos Lonicerae 15.