① There is Ig deposition between cells in the spinous layer of skin lesions, that is, the presence of pemphigus antibodies;

② There are pemphigus antibodies in the serum, and the titer is parallel to the clinical activity;

③Hemodialysis to remove pemphigus antibodies can lead to short-term clinical remission;

④ Babies born to mothers with pemphigus vulgaris have pemphigus skin lesions that resolve after a few weeks, indicating that pemphigus antibodies can be transferred through the placenta;

⑤ Repeated injection of high-titer pemphigus antibodies into the skin of rabbits can produce acantholysis;

⑥Pemphigus antibodies added to tissue cultured epidermal skin slices can cause acantholysis, and the degree is parallel to the antibody titer;

⑦The use of immunosuppressants is effective

More severe (worst prognosis); generally without prodromal symptoms; almost all have oral mucosal ulcers, most of which are the first or even the only manifestation. They are difficult to heal and easily expand to the surroundings, and can still involve the throat, esophagus, vulva, and anus.

Typical manifestations: The whole body can be affected; serous blisters/bullae of different sizes appear on normal-appearing skin, with thin walls, loose and easy to break; due to the presence of acantholysis, characteristic Nikolsky's syndrome may appear Symptoms: The eroded surface is left after the bullae rupture, with dirty/greasy sticky scabs on the surface, more exudate, slow healing, and often pigmentation after subsidence; varying degrees of itching and pain at the exfoliation site; generally no fever and WBC↑

The damage often develops continuously; epidermal exfoliation causes loss of tissue fluid. Oral mucosal lesions hinder eating → malnutrition, decreased resistance, and susceptibility to infection.

May be accompanied by other autoimmune diseases (such as thyroiditis, myasthenia gravis, lupus erythematosus)

Characteristics: It is a "subtype" of the usual type (but the antigenic composition is inconsistent); mainly limited to the armpit, thigh, gluteal groove, under the breast, umbilicus, genitals and perianal area; oral mucosal ulcers appear later and are milder; resistance is relatively low Strong→The course of the disease is slower and the prognosis is better

Types

Commonly occurs on the head, face and upper chest and back; usually no oral mucosal damage (if any, it is very slight)

Typical manifestations: Blisters often occur on the basis of erythema. The blister wall is extremely thin and fragile. Sometimes it is even difficult to form a blister. Even if it is formed, it is small and loose and easy to break. There is not much exudation on the peeling surface, and the desquamation layer adheres to form a loose, Thick, yellowish-brown, greasy scabs (like fallen leaves); bacterial decomposition under the scabs can produce odor; sun exposure can aggravate the lesions

Brazilian pemphigus: a special type; prevalent in some areas of South America, more common in children and adolescents; similar to the deciduous type, but with higher serum antibody titers and antimalarial drug treatment with a certain effect

It is a "subtype" of the deciduous type (the antigenic composition is the same); it is mainly found on the scalp, cheeks, upper part of the trunk and upper limbs (generally not involving the lower limbs and oral mucosa); sometimes it can develop into the deciduous type

Typical manifestations: scaly erythema distributed in a butterfly shape on the face (similar to lupus erythematosus but without skin atrophy after it subsides); scattered erythema on the scalp and upper part of the trunk with blisters with loose walls; pigmentation or mild epidermal warts remain after recovery. Hyperplasia; subjective symptoms are mild

Paraneoplastic pemphigus: mostly tumors originating from the lymphatic system, with severe disease and prominent mucosal damage; the skin lesions are polymorphic and have poor response to glucocorticoid treatment.

Drug-induced pemphigus: found in drugs containing sulfhydryl groups (such as D-penicillamine, captopril, piroxicam, rifampicin); mostly manifests as erythematous pemphigus, which can heal spontaneously after stopping the drug

IgA pemphigus: more common in skin folds of middle-aged and elderly women

Pemphigus herpetiformis: The skin lesions are often symmetrically distributed on the trunk/proximal limbs, are polymorphic (but mainly small blisters of about 0.5cm), Nissl's sign is negative; itching is obvious

(1) Direct immunofluorescence (DIF): There are Ig deposition (especially IgG) between the spinous cells of the skin around the blister and at the blister, and complement C3 is often positive; in the erythematous type, IgG and C3 deposition can still be seen at the basement membrane at the same time.

(2) Indirect immunofluorescence (IIF) (serological examination): 80~90% have Dsg antibodies (mainly IgG type), and their titers are parallel to the degree of disease and disease activity

Supportive care: very important

Local care: reduce abrasions; pay attention to oral and vulvar hygiene to prevent and treat infection

Be aware of complications and certain aggravating factors

Glucocorticoids: the drug of choice; once diagnosed, they should be used in a timely and sufficient amount (standard: no obvious new blisters appear within 1 week); after relief, the dose needs to be reduced to a maintenance dose, and then maintained for a longer period of time

Immunosuppressive drugs: adjuvant treatment drugs; usually azathioprine and cyclophosphamide are used

IVIg: effective in cases where high-dose glucocorticoids and immunosuppressants are ineffective

Plasma exchange: only suitable for severe cases where glucocorticoids are ineffective

Dapsone (DDS): suitable for mild and vesicular types

Chinese herbal medicine: suitable for patients with mild and less acute exudation; such as Tripterygium wilfordii

Treatment of skin lesions: For ordinary and deciduous types, a large amount of dusting powder, antibacterial ointment or paste can be used; for erythematous and oral mucosal lesions, hormonal ointment can be used

(1) More common in the elderly; often occurs in the armpits, groin, etc.; initial oral mucosal damage is rare, and even if it does occur, it is mild and heals quickly.

(2) Typical clinical manifestations: Scattered blisters/bullae appear on edematous erythema/normal skin. The blister walls are thick, tense and not easy to break. The blister fluid may be bloody; Nissl sign is negative; often accompanied by varying degrees of itching.

(3) Course and onset of disease: chronic disease course with alternating attack and remission phases; onset has nothing to do with gluten diet and no small intestinal mucosal damage

Supportive treatment: such as protein and vitamin supplements

Avoidance of possible allergenic drugs, detection of complications and visceral malignancies

Glucocorticoids: drug of choice

Immunosuppressive drugs: adjuvant treatment drugs; usually azathioprine and cyclophosphamide are used

IVIg: may be effective in cases where high-dose glucocorticoids and immunosuppressants are ineffective. Plasma exchange: only suitable for severe cases where glucocorticoids are ineffective.

Dapsone (DDS)

Chinese herbal medicine: such as tripterygium wilfordii

Tetracycline and nicotinamide: effective in the elderly and those who are intolerant to glucocorticoids