vulvar pemphigus
Introduction
Introduction to vulva pemphigus Vulvar pemphigus is a chronic, relapsing, autoimmune bullous skin disease mediated by anti-epidermal intercellular substance antibodies, characterized by loose blisters on normal skin or mucous membranes, positive for Nissl. basic knowledge The proportion of illness: 0.003% Susceptible people: no special people Mode of infection: non-infectious Complications: malignant tumor
Cause
Causes of vulvar pemphigus
(1) Causes of the disease
The occurrence of vulva pemphigus needs incentives. Some drugs and foods can induce pemphigus. Recent studies suggest that infection factors may be another cause of pemphigus. Drugs that can induce pemphigus often include: thiol-like drugs such as blue. Moldy amine, captopril, tazobactam, brain rejuvenation, etc.; antibiotics: penicillin, rifampicin, isoniazid, fungi, etc.; pyrazolone: aminopyrine, Baotaisong, etc.; Others such as digoxin, IL-2, -interferon, nifedipine, etc., of which the amount of pemphigus caused by thiol drugs accounts for about 80% of the total amount of drug pemphigus, drug-induced vulgaris days Patients with acne can detect circulating pemphigus antibodies in the blood. Ultraviolet irradiation can also aggravate the condition. It has been reported that PUVA is used to treat psoriasis-induced pemphigus.
Many studies have shown that there may be genetic susceptibility to pemphigus vulgaris. Serological methods suggest that Nordic Asknezim Jewish pemphigus vulgar susceptibility is associated with HLA-DR4, among non-Jews, HLA-DR4, DR6, Japan A study found that pemphis susceptibility is associated with HLA-DRBl*0406, *0403 and HLA-DRBl*1403, *1405, although current research data suggest that pemphigus susceptibility is associated with HIA, but not susceptible genes The population is ill.
Pemphigus vulgaris is currently considered to be an autoimmune disease based on:
1. Direct immunofluorescence confirmed that IgG-type pemphigus antibody was observed in the epidermis, oral mucosal epithelial involvement and interstitial cells in the unaffected area. C3 deposition was also observed in the acantholytic site.
2. Using indirect immunofluorescence technique, 80% to 90% of patients with pemphigus vulgaris can detect circulating pemphigus antibody, and the antibody titer is proportional to the severity of the disease, and the plasma is used to replace the day in the patient's serum. The acne antibody can relieve the condition.
3. The serum of pemphigus patients was added to normal skin culture, and the epithelial layer was released.
Pemphigus lesions occurred within 18 to 72 hours after injection of human pemphigus antibody in neonatal mice. Further studies showed that the anti-epidermal intercellular substance antibody IgG4 subclass is a pemphigus pathogenic antibody.
(two) pathogenesis
Pemphigus vulgaris antigen is desmoglein 3 (Dsg3), molecular weight 130kd, is a cell surface glycoprotein synthesized by keratinocytes and intercellular adhesion materials, belonging to the family of calcium-dependent adhesion molecules (cadherin). Dsg3 binds to 85kD of desmoplain in desmosome and plays an important role in adhesion between epidermal cells. Therefore, anti-epidermal intercellular substance antibody binds to Dsg3 and inhibits the function of desmosome-adhering epithelial cells, resulting in Acanthosis is one of its pathogenesis. In addition, after binding of pemphigus antibody to Dsg3, phospholipase C and inositol 1,4,5 triphosphate mediate intracellular signaling, causing epithelial synthesis and release. A plasminogen activator that converts plasminogen to plasmin, resulting in loss of spine cell adhesion and release.
Prevention
Vulvar pityriasis prevention
Early detection, early treatment, and good follow-up.
1. Eat more vegetables and fruits.
2 , drink more tea or light drinks.
3 , should cool blood and detoxify food. Mung beans, glutinous rice, cucumber, bitter gourd, purslane, green tea, etc.
4, eat spicy spicy food: because irritating food can affect the body's secretion, which causes skin itching, affecting treatment. Spicy and irritating foods include pepper, pepper noodles, mustard, raw onions, raw garlic, and white wine.
5, eat greasy food: greasy food mainly refers to oils and fats. Excessive intake of such foods promotes the secretion of sebaceous glands and exacerbates the condition. At the same time, also pay attention to eat less sweets and salty food, in order to facilitate the recovery of the skin.
6. Avoid spicy seafood and irritating and sensitizing foods when itching is severe.
Complication
Vulvar pemphigus complications Complications malignant tumor
Some pemphigus is associated with malignant tumors.
Symptom
Vulvar pemeptic symptoms Common symptoms Vulvar burning irritating blister or bullae damage mucosal damage Candida albicans infection Allergic tissue fluid exudation anorexia
Skin damage
The typical skin lesions are on the normal skin of the appearance. A few blister blisters of varying sizes of peas to eggs appear on the erythema base. The blister can be scattered or scattered, and the blister fluid can be cleared early, and then turbid or bloody. The blister wall is thin, slack, and easily broken. The Nikolsky sign is positive, that is, the top of the complete blister is pressed, and the blister is enlarged to the side; rubbing or squeezing the blister, smashing the nearby epidermis, causing exfoliation of the epidermis, and forming a red erosion surface after the blister wall is broken. There is a oozing out of the slurry, which gradually condenses into a dirty shell, which has an odor. The smashed surface continuously expands to the periphery and merges into an irregular shape. The edge of the collar is separated by a collar-shaped epidermis, and there is little self-healing tendency. Calm, consciously burning or pain, may be accompanied by fever, anorexia and other systemic symptoms.
Bullae can occur throughout the body, but it is more common in the head, neck, chest and back, underarms, and groin.
2. Female genital and mucosal damage
About 60% of patients with blisters can be found in the oral cavity. Almost all cases have oral mucosal involvement. In addition, they can also invade the nose, throat, conjunctiva, esophageal mucosa, external genitalia, urethra, and anal skin mucosa. One of the female genitals often involves the size of the labia, vaginal, cervical mucosa, mucosal damage, early allergic, burning, blistering in the easily rubbed parts, blister rupture quickly, complete blister is rare, forming a painful erosion surface, touch It is extremely easy to bleed, and the erosion is slow to heal. It lasts for several weeks or several months. It is easy to be infected with bacteria or Candida albicans. Sometimes it can develop into ulcers. Cervical lesions can occur in the early stage of the disease, resulting in abnormal smear of Papanicolaou, which is easily misdiagnosed as cervix. Epithelial hyperplasia.
Examine
Examination of vulvar pemphigus
Histopathological examination showed that there was a fissure above the basal layer. The blisters and blister tops were composed of most of the epidermis. The basal cells attached to the dermal papilla of the basal cells protruded into the internal cavity of the epidermis, called villi, blister fluid. There are acantholytic cells in the middle. The cells are large and round, the core is concentrated, the cytoplasm is evenly basophilic, there is a light-stained area around the nucleus, the dermis is mildly edema, and there are a small amount of eosinophils, neutrophils. Cell infiltration.
Immunofluorescence check:
1. Direct immunofluorescence:
Take normal skin or new skin lesions around the skin lesions to check, showing that IgG and C3 are fishnet-like deposits between epidermal cells, and other components have C1q, C4 deposition, the positive rate is 80% to 95%, active lesions can be Up to 100%, a small number of antibodies can be IgM, IgA.
2. Indirect immunofluorescence:
80% to 90% of patients can detect anti-epidermal intercellular substance antibodies, such as pebbles, mainly IgG, a small number of IgM, IgA, antibody titer is highly correlated with disease activity, but early disease It can be false negative, observed by electron microscopy: early changes are local or total dissolution of interstitial cell matrix or sugar film, cell gap widening, desmosomes are separated from each other, and the late tension wire falls off from the bridge of the bridge particles, and the bridge particles disappear.
Diagnosis
Diagnostic identification of vulvar pemphigus
According to the clinical skin-mucosa with loose bullae, Nissl's sign is positive, histopathology shows the loose layer of blisters above the basal layer, direct immunofluorescence shows the involvement of IgG, C3 deposition in the epidermal cells, indirect immunofluorescence in the serum Anti-epidermal interstitial substance antibodies (pemphis antibodies) can be diagnosed.
Bullous pemphigoid, severe polymorphous erythema, familial chronic benign pemphigus, linear IgA bullous skin disease can have groin, perineum, vesicles in the perianal area, erosion, occasionally vaginal mucosal damage .
