What is Pemphigus?

Pemphigus is a chronic, recurrent, severe intraepithelical spinous lytic bullous skin disease. Patients have antibodies against cadherin, a Ca [sup] 2 + [/ sup] -dependent intercellular adhesion molecule. Therefore, loose blisters appear on normal skin or mucous membranes, and they have positive Nissl sign.

Basic Information

English name

pemphigus

Visiting department

dermatology

Multiple groups

50 60 years old

Common locations

Skin, mucous membrane

Common symptoms

Pemphigus vulgaris, Pemphigus proliferative, Pemphigus decidum, Pemphigus erythematosus

Causes of Pemphigus

The etiology of this disease has not been fully clarified and is now considered to be an autoimmune disease. Pemphigus antigens are mainly desmosomes, and antibodies are mainly IgG. Four IgG subtypes can be seen, and a few are IgA. Antibodies bind to epidermal cells, causing spines to unravel. The cDNA sequence of pemphigus antigen has obvious homology with cadherin. Therefore, pemphigus antibody also damages the adhesion function between epidermal cells, resulting in spinous release.

Pemphigus clinical manifestations

The age of onset varies widely. The average age of onset is 50 to 60 years. Pemphigus is traditionally divided into four types in China: common type, proliferative type, deciduous type, and erythema type.

1. Pemphigus vulgaris

It is the most common type of pemphigoid. More than half of the patients first develop blisters and erosions in the oral mucosa, and then skin damage occurs. In the future, the skin with normal appearance appeared large blisters of soybeans to walnuts, the blister fluid was clear or slightly muddy, the blister walls were thin and loose and easily broken, and the Nissl sign was positive. The blister rupture reveals the flushed and erosive surface with a little exudation or scabbing, the wound heals slowly, consciously burns, and pigmentation and miliaria are left after healing. Blisters can occur anywhere in the body, and are common on the head, face, neck, chest and back, underarms, and groin. May have nail dystrophy, acute paronychia, and hypothyroidism. Pregnancy and stillbirth can occur in severe pemphigoid during pregnancy.

2. Proliferative Pemphigus

Younger onset. Skin lesions are common in seborrheic areas, such as the head and face, underarms, umbilical fossa, chest and back, and femoral area. It is a loose blister at the beginning, and it is easy to rupture to form erosive surface and mushroom-like, papillary hyperplasia, especially in the friction part. Serum or pus exudate on the damaged surface, covered with thick palate, and there are inflammatory flushes around it. Damage to agglomerate groups or expansion and fusion into tablets, there is a stench. Skin damage can occur before or after mucosal damage. Conscious symptoms are not obvious. During the course of the disease, there are sometimes symptoms such as high fever due to secondary bacterial infection. The lesions are severe and mild, and the course is longer than usual.

The disease is divided into two types:

(1) Heavy (Neumann type) skin lesions are blisters and bullae. After rupture, the hypertrophic granular erosive surface can easily bleed. The proliferative plaque formed has exudation of serum and pus, and small pus around Blister. Erosion at the border forms new proliferative plaques, and finally these proliferative lesions become dry, hyperkeratotic, and cleft. This type of disease has a long course and it is difficult to relieve itself before the application of glucocorticoids.

(2) Light (Hallopeau type) early lesions are characterized by pustules rather than blisters. Proliferative plaques are formed after blister rupture. There are small pustules around the plaque. A variety of bacteria can be cultured within the lesion. The disease is chronic, mild, and relieves spontaneously with a good prognosis.

3. Deciduous Pemphigus

Mostly on the head, face, and trunk, normal skin appears with loose bullae, positive Nissl sign, herpes wall is thin, easily ruptured, and quickly dries. It has a yellowish brown palate, the center of the crust is attached, the edge is free, and the crust is wet, gradually Development to the whole body. The skin is dull red, covered with a large number of leaf-shaped crusts, and has a foul odor. Sometimes it looks like exfoliative dermatitis without obvious blisters. Oral damage is rare, and hair is thin and often light. Nail changes can be seen in the nails. Consciously itching or burning, systemic symptoms vary, may have fever, chills, mental disorders and so on. The course of disease can last for more than 10 years, the prognosis is good, it is easy to be controlled by glucocorticoids, and some patients can be completely relieved.

4. erythematous pemphigus

Also known as Senear-Usher syndrome. Skin lesions occur in the head, forehead, nose, cheeks, ear shells, and sometimes the back of the chest, armpits, and groin can be invaded, but the limbs are rarely affected. Head and face skin lesions are similar to discoid or systemic lupus erythematosus and seborrheic dermatitis. There are fatty scales and scutellaria on localized erythema. One to several months after the above-mentioned skin lesions appeared, flaccid bullae suddenly appeared on the chest, back, and limbs. The walls of the blister were thin and easily broken. The erosive surface gradually expanded. The exudate was more frequent. Alas, it is not easy to fall off, leaving tan pigmentation in the prognosis. The blisters came one after another, with a positive Nissl sign. There is generally no mucosal damage. Consciously itching, the systemic symptoms are not obvious.

Pemphigus Diagnosis

The main points of diagnosis are loose bullae on the skin, positive Nissl sign, and often accompanied by mucosal damage. Pemphigus cells can be seen on the basal smear of blisters, histopathological changes are characteristic, and spines are loosened in the epidermis. Indirect immunofluorescence examination of pemphigoid antibodies in serum, direct immunofluorescence examination of normal skin or new skin lesions around the blister, and IgG and C3 deposition between epidermal cells.

Pemphigus Treatment

1. General supportive care

It is extremely important that those with extensive damage should be given a high-protein diet and supplemented with multivitamins. Pay attention to water and electrolyte balance, fasting should be supplemented by intravenous. Systemic failure can involve multiple small blood transfusions or plasma. Strengthen care and pay attention to skin cleanliness to reduce secondary infections on the wound surface and prevent pressure ulcers.

2. Systemic treatment

(1) Glucocorticoid The drug of choice for the treatment of this disease, try to achieve timely treatment, adequate control, correct reduction, followed by the minimum maintenance amount. In severe cases, shock therapy can be used. Nearly half of the patients who use glucocorticoids die from complications of glucocorticoids, such as respiratory infections, pulmonary embolism, diabetes, and peptic ulcers. Therefore, they must always be alert to the occurrence of adverse reactions and take appropriate measures in a timely manner.

(2) Immunosuppressive agents can inhibit the formation of autoantibodies. It is the main adjuvant therapy for this disease. Combined with glucocorticoids, it can improve the efficacy and reduce the amount of hormones. Commonly used are azathioprine and cyclophosphamide.

(3) Gold preparation After using glucocorticoids to control the disease, gold preparation can be added for treatment.

(4) Plasma exchange therapy is suitable for patients with severe disease, ineffective combination therapy of glucocorticoids and immunosuppressants, and high blood pemphigus antibody titers. Large-dose glucocorticoid therapy can be used when there are side effects or the effect is not obvious. 1 or 2 times a week, each time exchange 1-2L, depending on the condition can be carried out 4-10 times continuously. For example, it works best with small doses of glucocorticoids or immunosuppressants.

(5) Others A few cases are effective in the treatment of the following drugs, such as ampicram, sulfamethoxamine, nicotinamide, and tetracycline, either alone or in combination with glucocorticoids. High-dose immunoglobulins relieve pemphigoid quickly, but for a short duration. Avi A combined with prednisone for the treatment of proliferative pemphigoid.

3. Local treatment

Pay attention to oral hygiene and treat periodontal diseases. Oral erosion can be rinsed with 2% boric acid solution or 1% hydrogen peroxide every 3 to 4 hours. When the pain is obvious, apply 3% benzocaine boric acid glycerol solution or 1% procaine solution to gargle before eating. When skin lesions are small, topical use of zinc oxide oil, 2% methyl violet zinc oxide oil on the eroded surface. Erythema damage can be applied topically with glucocorticoid cream. When the damage is extensive, pay attention to avoid bacterial infections caused by conditions, such as exudate crusting, patients are generally in good condition, you can take a medicated bath, such as 1: 10000 potassium permanganate solution. Topical antibiotics, antifungals.