What Is Pemphigus Vulgaris?

Pemphigus vulgaris

This disease belongs to its own immune disease. It can detect antibodies in the lesions, and can also detect antibodies against epidermal spines in the blood.

Pemphigus Vulgaris Disease Name

Pemphigus vulgaris

Classification of Pemphigus Vulgaris Diseases

Stomatology

Overview of Pemphigus Vulgaris Diseases

Clinical symptoms:

The disease is more common in middle-aged men. A bullae occurs from the mouth first, and then becomes a painful erosion surface. Later, there are flaccid bullae in the head, face, trunk, and extremities. It is positive for Nissl's disease. After the ulceration, an erosion surface, exudation, bleeding, and conscious pain are formed. The disease can be divided into 4 types: common type, proliferative type, erythema type, deciduous type, and the course of the disease is chronic. Due to the large amount of nutritional consumption in the body, patients can die. Histopathology included epidermal bullae and spinous layer lysis, and spinous layer lysis cells in blister fluid. Direct immunofluorescence showed positive immunofluorescence between epidermal cells.

Prevention:

Patients should pay attention to the protection of the mouth and skin after the onset to prevent collision and friction. Systemic corticosteroid preparations are effective. If simultaneous use of immunosuppressive preparations will have a synergistic effect, cyclosporin A or triptolide can also be used to enhance skin and oral care.

Pemphigus vulgaris symptoms and signs

(1) Oral cavity: lesions appear earlier. Usually dry mouth, dry throat or tingling when swallowing, 1-2 or widely occurring blisters of varying sizes, the walls of the blisters are thin and transparent, the blisters are easy to break, and have an irregular erosion surface; there are residues The wall of the blister and shrinks around; the bitter wall of the blister often tears away the adjacent normal-looking mucosa and leaves a bright red wound; this phenomenon is called a positive peel test. The bitterness is gently inserted into the probe at the edge of the erosion surface, and it can be seen that the probe enters the lower part of the mucosa painlessly. This is a phenomenon of spinous layer loosening, which has diagnostic significance. Lesions can occur in the soft palate, hard palate, the pharynx, and any other areas that are easily rubbed. The blister can occur before or concurrently with the skin. The secondary infection worsens the condition, and the pain also worsens. The patient has difficulty chewing, swallowing, and even speaking, having nonspecific bad breath, enlarged lymph nodes, increased saliva, and blood stains.

(2) Skin: The lesions mostly occur on the front chest, trunk, and scalp, neck, axilla, groin and other vulnerable areas. In the early days, there are only 1-2 blisters in the chest or torso, which are often not noticed. On normal skin, blisters of various sizes often appear suddenly, the blister is not fused, the wall of the blister is thin and loose, and easy to break. After the break, the red and wet erosion surface is exposed. After infection, the pus can form pus, blood, and odor. It will heal later. And leave deeper pigmentation. With your fingers, gently push the normal skin or mucosa on the outside to quickly form blister, or move the original blister on the skin. In the mouth, the tongue and mucous membrane can be used to make the surface of the normal-looking mucosa fall off or torn off. These phenomena are called Nikolsky's sign, that is, Nissl sign, which has diagnostic value. The conscious symptoms of skin damage are mild itching and pain during erosion. Systemic symptoms such as fever, weakness, and loss of appetite may appear during the course of the disease; as the disease progresses, body temperature rises, and new blisters may appear continuously. Due to a large amount of dehydration and electrolyte disturbances, patients develop cachexia and can die from infection.

(3) The mucosa of the nasal cavity, eyes, external genitals, anus, etc. can all have the same lesions as the oral mucosa, and it is often difficult to return to normal.

Causes of Pemphigus Vulgaris Disease

The etiology of pemphigus is unknown. At present, there are many studies on the cause of autoimmune diseases. It is believed that the infection with virus infection, ultraviolet radiation, and certain drugs (such as penicillamine) makes the adhesive substance between the layers of spinous cells an autoantigen. And evoked autoimmune response.

Diagnosis of Pemphigus Vulgaris

1. Characteristics of clinical damage Clinically, only a red wound or erosive surface is often seen. If the probe can be used to penetrate under the epithelium or the adjacent mucosal surface without resistance, a positive Nissl sign or a positive peel test will help. Make a diagnosis, but do not use skin peel tests on a large scale to avoid increasing patient suffering. The general condition of the patient is a decline in physique, even sub-morbidity, which also helps the diagnosis. In addition, the following methods are helpful for correct diagnosis.

2. Cytological examination After the local sterilization, the early fresh bullae were cut off the blister top, the tissue at the bottom of the blister was gently scraped, and then coated on a glass slide. After drying, stained with Giemsa or erythrolimin-eosin. Disintegrating disintegrating cells. The nucleus is large and round, with deep staining and less cytoplasm. It is also known as pemphigoid cells or spinous layer release cells. The amount of these cells is related to the severity of the disease.

3. The biopsy is near the lesion, and the blister is rubbed with a mouthpiece handle, and then the epithelium and the tissue below it are excised.

4.Immunological examination

(1) Immunohistochemistry: Immunofluorescence direct method showed antibodies against cell adhesion substance between spinal cell layers.

(2) Detection of serum antibody substances: The indirect immunofluorescence method is used to detect circulating antibodies against basal cells in the cytoplasm, interstitial cells of the spinous cell layer, and circulating antibodies in the spinal cells. The general antibody titer is 1: At 50 o'clock it makes sense.

Pemphigus vulgaris treatment options

1. Supportive therapy should be given a high-protein, high-vitamin diet, intravenous supplementation, and a small number of multiple blood transfusions for those with systemic failure.

2. The initial dose of the adrenal cortex hormone prednisone is 120-180mg / d; or 60-100mg / d, the initial dose can be reduced until no new damage occurs for 1-2 weeks, 5mg each time, 1- Once every 2 weeks, the dose should be carefully reduced below 30mg / d until 10-15mg per day is the maintenance amount. For severe pemphigoid patients, shock therapy and intermittent dosing can be used. That is, the adrenocortical hormone is given in large doses until the condition is stable (approximately 10 weeks), and then gradually reduced to 30 mg / d of prednisone, and then administered every other day or 3 days of medicine and 4 days of rest.

3, immunosuppressive agents such as cyclophosphamide, azathioprine or methotrexate and prednisone and other adrenocortical hormones combined treatment, in order to reduce the amount of the latter, thereby reducing the side effects.

4. Antibiotics Add antibiotics to prevent concurrent infections.

5. For topical application, oral erosion and pain, before eating, can be coated with 1% -2% tetracaine solution, and gargle with 0.25% tetracycline or aureomycin can help maintain oral hygiene. Topical application of corticosteroid ointment can promote healing of oral wounds.

6. Enzyme inhibitors The corresponding inhibitors of various proteolytic enzymes have been proven to resist the production of spinous layer loosening, but no successful clinical application has been reported.

7, Chinese medicine, traditional Chinese medicine, spleen deficiency and damp heat type can be selected with Buzhong Yiqi Decoction, Qingpi Dehumidifying Drink, Wulingsan, etc .; hot and hot type can be Huanglian Jiedu Decoction, Qingwendu soft, Qingying decoction, manna disinfectant , Yu Nu Jian and other parties add and subtract.