pemphigus vulgaris
Introduction
Introduction to pemphigus vulgaris The disease is an autoimmune disease, and antibodies can be detected at the lesion, and antibodies against epidermal cell spines can also be detected in the blood. The disease is more common in the middle and young years. The big blisters start from the mouth and break into a painful erosion surface. Later, loose blisters occurred on the head, face, and limbs. It is positive for Nissl's disease. After ulceration, it forms erosion surface, exudation, bleeding, and conscious pain. The disease can be divided into 4 types: vulgaris, proliferative, erythema, deciduous, and the course of the disease is chronic. Due to a large amount of nutrient consumption in the body, the patient may die. Histopathology is the epidermis bullae, acanthosis, and acantholytic cells in the blister fluid. Direct immunofluorescence showed positive immunofluorescence between epidermal cells. basic knowledge The proportion of illness: 0.03%--0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: pemphigus
Cause
Causes of pemphigus vulgaris
Cause (90%):
The cause is not completely clear and is considered to be an autoimmune disease. Poor prognosis. There are certain anti-epidermal cell membrane autoantibodies (pemphapes antibodies) in the serum of patients, most of which are IgG. Direct immunofluorescence of skin lesions or normal skin shows inter-segmental staining of the epithelial cells, which corresponds to the location where the spine cell release occurs. The titer of pemphigus antibodies in the patient's serum is roughly parallel to the severity of the disease.
Prevention
Pemphigus vulgaris prevention
After the onset, patients should pay attention to the protection of the mouth and skin to prevent collision and friction. It is better to treat with systemic corticosteroid preparations. For example, the combination of immunosuppressive preparations may have a synergistic effect, and may also be treated with cyclosporin A or tripterygium wilfordii to enhance skin and oral care.
Complication
Pemphigus vulgaris complications Complications pemphigus
Blisters or bullae damage, blisters in the oropharynx, blisters deep in the watch skin, mucosal damage.
Symptom
Symptoms of pemphigus vulgaris Common symptoms Mucosal damage blister or bullous damage to the oropharynx blister-like damage to the blisters deep in the watch skin
(1) Oral: Early lesions. Often there is tingling in the mouth, dry throat or swallowing pharynx, 1-2 or widely occurring blisters of varying sizes, the blister wall is thin and transparent, the blisters are easily broken, and the irregular erosion surface is left; The blister wall, and retreating to the periphery; bitter taste tearing the wall, often without painful tearing off the adjacent appearance of the normal mucosa, and left a bright red wound; this phenomenon is called the skin test positive. The bitter taste is gently inserted into the probe at the edge of the erosion surface, and the probe is painlessly entered below the mucosa, which is a phenomenon in which the acanthus is loosened.
Lesions can occur in soft palate, hard palate, parapharyngeal and other areas that are susceptible to friction. Blisters can occur before or at the same time as the skin.
Secondary infections worsen the condition and the pain is aggravated. Patients have difficulty chewing, swallowing, and even speaking. They have non-specific bad breath, swollen lymph nodes, increased saliva and blood.
(2) Skin: Most of the lesions occur in the front chest, trunk and scalp, neck, armpits, groin and other vulnerable parts. In the early stage, there were only 1-2 blisters on the chest or torso, which were often not noticed. On normal skin, blister of different sizes often appears suddenly, and the blister does not fuse. The blister wall is thin and slack, easy to break. After the break, it reveals a red and wet smashed surface. After infection, it can form a pus and bloody sputum, smelly, and heal later. And leave a deeper pigment.
By gently pushing the normal skin or mucous membrane with your fingers, you can quickly form a blisters or move the original blisters on the skin. In the oral cavity, the tongue and the mucous membrane can be used to peel off or tear off the surface of the mucous membrane with normal appearance. These phenomena are called Nikolsky's sign, that is, the Nissl's sign, which has diagnostic value.
The symptoms of skin damage are mild itching, pain when smashing, and systemic symptoms such as fever, weakness, loss of appetite, etc. during the course of the disease; as the disease progresses, the body temperature rises and new blisters appear continuously. Due to a large number of water loss and electrolyte imbalance, the patient has cachexia and can die due to infection.
(3) Mucosa in the nasal cavity, eyes, external genitalia, anus, etc. can cause the same lesions as the oral mucosa, and it is often difficult to return to normal.
Examine
Examination of pemphigus vulgaris
Pemphigus vulgaris must check items:
(1) blood routine, urine routine, stool routine and occult blood.
(2) Screening of liver and kidney function, electrolytes, blood sugar, blood lipids, immunoglobulins, infectious diseases (hepatitis B, hepatitis C, syphilis, AIDS, etc.).
(3) Skin biopsy and direct immunofluorescence, serum indirect immunofluorescence assay for pemphigus antibody and titer.
(4) Bacterial culture and drug sensitivity test of wounds.
(5) Chest film and electrocardiogram.
(6) According to the choice of patients with pemphigus vulgaris: tumor screening: complete set of tumor antigen, B-ultrasound, endoscopy and other imaging examinations such as CT or MRI (thoracic, abdominal, pelvic, posterior peritoneum, etc.).
Diagnosis
Diagnosis and identification of pemphigus vulgaris
Clinically, only a red wound or erosion surface is often seen. If the probe can be inserted under the epithelium or under the adjacent mucosa without resistance, the Nissl's sign is positive, or the skin test is positive, which is helpful for diagnosis, but not big. The peeling test is used in scope to avoid increasing the suffering of the patient. The general condition of the patient is characterized by decreased constitution and even sub-quality, which is also helpful for diagnosis. In addition, the following methods are helpful for proper diagnosis.
1. Cytological examination After local disinfection, the early fresh bulls are cut off to the blister top, the blister tissue is lightly scraped, applied to the slide, dried and stained with Giemsa or red hematoxylin-eosin, showing typical acanthosis. Loose disintegrated cells. The nucleus is large and round, with deep staining and less cytoplasm. It is also known as pemphigus cells or acantholytic cells. The amount of such cells is related to the severity of the disease.
2. The biopsy is in the vicinity of the lesion, and the blister is lifted with a mouth mirror, and then the epithelium and the tissue below it are cut.
3, immunological examination
(1) Immunohistochemistry: Immunofluorescence direct method shows antibodies against cell adhesion substances between layers of spine cells.
(2) Detection of serum antibody substances: The indirect method of immunofluorescence is to detect circulating anti-basal cells in the cytoplasm of the patient's serum, the interstitial cells of the spinous cell layer and circulating antibodies in the spine cells. The general antibody titer is 1: 50 o'clock means that.
