Vulvar lichen sclerosis

　　First, the cause of the disease

　　The etiology is unclear. At present, there are several views:

　　1. Genetic disease: There are reports that mother-daughter and sister pairs in the family have the disease at the same time, and it is found that the positive rate of HLA-B40 antigen in patients is significantly higher than that in women without the disease, so this disease is closely related to HLA-B40.

　　2. Autoimmune diseases: There are reports that about 21% of patients have autoimmune diseases such as diabetes, hyperthyroidism or hypothyroidism, vitiligo, pernicious anemia, alopecia areata, etc., and there is lymphocytic infiltration in the epidermis, indicating that there is an immune reaction in the local tissue, suggesting that this disease may be related to subepidermal damage caused by autoimmune antibodies against collagen fibers.

　　3. Deficiency of sex hormones: Since the lesions in prepubertal patients can be relieved after menarche, it is believed that it may be related to the deficiency of estrogen, but the clinical application of estrogen therapy is ineffective; some scholars reported that the levels of dihydrotestosterone and androstenodione in the serum of patients are reduced, while free testosterone is increased, and local testosterone therapy is effective. After treatment, the total testosterone and dihydrotestosterone in the blood increase, and the author speculated that this may be related to the decreased activity of 5α-reductase, leading to the obstruction of testosterone conversion to dihydrotestosterone. However, in patients with 5α-reductase deficiency, the risk of lichen sclerosis is not increased. Recently, there have been reports that corticosteroid therapy is effective in patients lacking androgen receptor.

　　4. In recent years, some have found that some patients with vulvar lichen sclerosis have spirochetal infection, suggesting that spirochetes may be the pathogenic cause of this disease.

　　5. Neurovascular nutrition disorder There is a disorder of neurovascular nutrition in the deep connective tissue of the vulva, leading to lesions in the skin covering it. Some people have exchanged the diseased skin of the vulva with the normal skin of the patient's thigh, and found that the diseased skin transplanted to the thigh gradually returns to normal, while the normal skin transplanted to the vulva developed into lichen sclerosus. Therefore, it is proposed that local neurovascular nutrition disorder is the cause of the disease.

　　Although the various phenomena observed in clinical practice seem to be related to the pathogenesis, so far, they have not been confirmed and generally recognized.

　　Second, pathogenesis

　　Epidermal atrophy, superficial hyperkeratosis, often visible hair follicle keratin plugs, stratum spinosum becomes thin, basal layer cells liquefy and vacuolate, the rete pegs become blunt or disappear; in the early stage of the superficial dermis, there is edema, in the late stage, collagen fibers become glassy, forming a homogenization zone, below the homogenization zone there are lymphocytes and plasma cell infiltration. In addition, the epidermal melanocytes decrease. Due to the hyperkeratosis of the epidermis and the decrease of melanocytes, the skin appears white.

　　1. Vulvar atrophy, the skin color becomes pale, with poor elasticity, often accompanied by fissures and desquamation; in the late stage, the skin becomes thin and皱缩 like cigarette paper or sheepskin paper, with frequent subcutaneous hemorrhage.

　　2. The vaginal orifice becomes narrow and contracted, and severe cases may have difficulty urinating. Urine can soak the thin vulvar skin, causing erosion and pain.

　　3. Hardened lichen sclerosus rarely progresses to invasive cancer, but there may be硬化性苔藓 around invasive cancer.

　　The main symptoms are vulvar itching, dyspareunia, and burning or pain during the occurrence of fissures. The perineal skin may crack during cycling, defecation, or sexual intercourse. The typical clinical feature is vulvar atrophy, manifested as shrinkage or even disappearance of the labia minora, which may be adherent to the clitoris. The labia majora become thin, the clitoris atrophies, and the prepuce is elongated. The skin color becomes pale and shiny,皱缩, with poor elasticity. It is often accompanied by fissures and desquamation. The lesions are usually symmetrical and can affect the perineum and perianal area, forming a butterfly shape. Early lesions are relatively mild, with redness and swelling of the skin, pink or ivory white papules, which may merge into purpura; if the lesions further develop, they can form typical clinical manifestations. In the late stage, the skin becomes thin and皱缩 like cigarette paper or sheepskin paper, with frequent subcutaneous hemorrhage,呈对称性分布. The vaginal orifice becomes narrow and contracted, and severe cases may have difficulty urinating. Urine can soak the thin vulvar skin, causing erosion and pain. Hardened lichen sclerosus rarely progresses to invasive cancer, but there may be硬化性苔藓 around invasive cancer.

　　The itching symptoms of young female patients are often not obvious, and they may feel discomfort in the vulva or perianal area after urination or defecation. The hardening and atrophy in young girls and some adult women are mainly manifested as swelling of the clitoris and adhesion with the prepuce, absence of the labia minora, white spots on the posterior labial junction, the skin of the labia majora can retain a certain degree of elasticity. Due to the excessive keratinization in young girls, the lesions are not as obvious as in adults, and the examination shows that the local skin is呈珠黄色 or intermingled with hyperpigmented spots to form a speckled pattern. If there are lesions in the vulva and perianal area, they may present as keyhole-like or white ulcerative lesions. Most patients' lesions may disappear spontaneously during puberty.

　　Preventive measures for vulvar lichen sclerosis: pay attention to vaginal hygiene, prevent infection, actively treat systemic related diseases. This disease is more common in adult women, and the level of testosterone in the blood of patients is significantly higher than that of normal age-matched women. When testosterone is used locally for the affected skin, it is often effective, thus suggesting that low testosterone levels in the blood of patients may be one of the causes of the disease.

　　1, microscopic examination of secretions.

　　2, pathological examination of living tissue.

　　3, initial diagnosis can be made according to clinical manifestations, and pathological examination of living tissue is the only final diagnostic method. According to the patient's vulvar itching and typical predisposed sites, clinical manifestations, local pigment loss and white patch formation, pathological examination shows hyperkeratosis, atrophy of the stratum spinosum, dermal edema and collagen fiber transformation, the diagnosis is not difficult.

　　One, dietetic recipe for vulvar lichen sclerosis

　　1, Kelp and green bean porridge

　　Ingredients: 30g kelp, 30g green beans, appropriate amount of sugar, 100g glutinous rice.

　　Preparation method: first wash the kelp and chop it, soak the green beans for half a day, wash the glutinous rice clean, and cook them into porridge. Add sugar for seasoning when it is almost done.

　　Usage: take it twice a day in the morning and evening, and it is recommended to continue eating for 7 to 10 days.

　　Effect: clearing heat and detoxifying, promoting diuresis and heat elimination. It is suitable for vaginal itching.

　　2, Coix seed and red date porridge: Coix seed 30g, 10 red dates, 50g rice, wash and cook into porridge for consumption. It has the effect of clearing heat, invigorating the spleen and stopping itching.

　　3, Polygonum multiflorum and mulberry seed sesame porridge: Polygonum multiflorum 30g, mulberry fruit 10g, black sesame 10g, 50g rice. Wash and cook into porridge for consumption. It has the effect of nourishing blood, moistening the Yin and stopping itching.

　　4, steamed pork liver: 60g pork liver, 30g hemostem. Cut the pork liver and hemostem into small pieces, mix well, place them in a covered bowl, and steam in a steaming pot for 30 minutes. Take it all at once. It has the effect of clearing heat and removing dampness.

　　5, crucian carp and adzuki bean soup: 1 crucian carp, 60g adzuki beans. Remove the head, tail, and bones of the crucian carp, take the meat and cook with adzuki beans until the beans are soft. Take it in two doses. It is used for women with excessive leukorrhea and damp heat toxins.

　　6, Poria and glutinous rice porridge: Poria 30g (ground powder), glutinous rice 30-60g. First, cook the glutinous rice into porridge, add the Poria powder when half-cooked, mix well, and cook until the rice is done, take it on an empty stomach. It belongs to the syndrome of spleen deficiency and dampness in traditional Chinese medicine.

　　Two, what is good for the body to eat with vulvar lichen sclerosis

　　1, eat more foods rich in protein and sugars. For example: milk, soy milk, eggs, meats, etc.

　　2, drink plenty of water, and eat fresh fruits and vegetables. Such as apples, pears, bananas, strawberries, kiwi, cabbage, green vegetables, rapeseed, mushrooms, seaweed, kelp, etc.

　　3, prefer cool blood and detoxifying foods. Green beans, glutinous rice, cucumber, bitter melon, Portulaca oleracea, green tea, etc.

　　Three, it is best not to eat certain foods for vulvar lichen sclerosis.

　　1. Avoid eating irritants. For example: fish, shrimp, crab, chicken head, pork head meat, goose meat, chicken wings, chicken feet, etc., eating them will worsen the itching and inflammation of the vulva.

　　2. Eat less spicy and irritating foods. For example: onions, black pepper, chili, Sichuan pepper, Brassica juncea, fennel.

　　3. Avoid eating fried and greasy foods. For example: fried dough sticks, butter, butter, chocolate, etc., which have the effect of promoting dampness and increasing heat, which can increase the secretion of leukorrhea and is not conducive to the treatment of the disease.

　　4. Quit smoking and drinking. Smoking and drinking are very irritating and can worsen inflammation.

　　Firstly, the TCM treatment for vulvar lichen sclerosus: home remedies:

　　1. The composition includes 10g of Panax ginseng, 10g of Cimicifuga foetida, 10g of Cornu cervi degelatinized, 15g of Dioscorea opposita, 15g of Rehmannia glutinosa, 15g of Eucommia ulmoides, 15g of Angelica sinensis, 15g of Cornus officinalis, 15g of Lycium barbarum.

　　The dosage is decocted in water, 1 dose per day, taken twice a day, in the morning and evening.

　　2. The composition includes 9g of Scrophularia ningpoensis, 9g of Ophiopogon japonicus, 15g of Leonurus heterophyllus, 15g ofPolygonum multiflorum, 12g of Lycium barbarum, 12g of Cuscuta chinensis, 9g of Schisandra chinensis, 90g of Moutan bark, 9g of Rubus coreanus, 15g of Morus alba.

　　The dosage is decocted in water, 1 dose per day, taken twice a day.

　　The Western medical treatment for vulvar lichen sclerosus includes: 1. General treatment: Keep the vulva clean and dry, avoid using strong irritant drugs or soap to clean the vulva, avoid wearing non-breathable synthetic underwear, do not eat spicy and allergic foods. For those with severe itching symptoms leading to insomnia, sedative, hypnotic, and antiallergic drugs can be added.

　　Secondly, local drug treatment

　　The disease is mainly characterized by severe itching, and it is generally recommended to use corticosteroids for treatment, with definite effects. Commonly used 1% to 2% hydrocortisone cream or 0.025% fluocinolone acetonide cream, 3 to 4 times a day. Generally, after 6 weeks of medication, the skin lesions can return to normal.

　　It is generally considered that the local application of testosterone propionate is the standard method for treating lichen sclerosus, but its efficacy varies from person to person. Some atrophic skin can basically return to normal, some lesions have improved, but some may not show significant efficacy. After applying 2% testosterone propionate ointment (200mg testosterone propionate added to 10g vaseline ointment) and slightly massaging, the initial 3 to 4 times per day, apply for 3 weeks, then change to once per day, apply for 3 weeks, and then apply the maintenance dose, once per day or every other day. Generally, the treatment should be continued for 3 to 6 months, and the frequency of medication can be gradually reduced to once or twice a week to maintain the dose. If the itching symptoms are severe, the above testosterone propionate preparation can also be mixed with 1% or 2.5% hydrocortisone cream for application. After the itching is relieved, the hydrocortisone cream can be gradually reduced until it is finally discontinued. During the treatment, closely observe the side effects of testosterone propionate. If there are side effects such as increased hair growth or clitoral enlargement, or if the efficacy is poor, the medication should be stopped and 0.3% progesterone ointment (100mg progesterone in oil added to 30g vaseline ointment) can be used, applied 3 times a day. It can also be selected 0.05% betamethasone cream, in the first month, applied twice a day, then once a day, for 2 months, finally twice a week, for 3 months, a total of 6 months of treatment time.

　　III. Subcutaneous Injection

　　For those with persistent itching and ineffective surface treatment, triamcinolone acetonide suspension can be used for subcutaneous injection. After diluting 5mg of triamcinolone acetonide suspension with 2ml of normal saline, inject it subcutaneously below the pubic symphysis using a spinal anesthesia needle. The injection is made through the subcutaneous tissue of the labia majora until the perineum, slowly withdraw the needle tip, and inject the suspension into the subcutaneous tissue. Treat the opposite side in the same way. Gently massage after injection to disperse the suspension. If this method does not achieve the therapeutic goal and the patient still has itching, hospital treatment can be arranged: subcutaneous injection of pure alcohol under regional anesthesia block. This method involves marking longitudinal and transverse lines 1cm apart on the vulva, and injecting 0.1 to 0.2ml of pure alcohol subcutaneously at each intersection of the longitudinal and transverse lines. After injection, gently massage to disperse the liquid, but be careful not to inject the alcohol into the skin, otherwise it may cause skin necrosis. Subcutaneous injection of alcohol can completely relieve itching, but it cannot relieve the burning sensation of the vulvar skin.

　　IV. Vaginal Plaque in Young Girls

　　It may heal spontaneously by puberty, and its treatment is different from that of adult women. Progesterone is generally not used to avoid masculinization. The main purpose of treatment is mainly to temporarily relieve itching symptoms, which can be treated locally with 1% hydrocortisone ointment or 0.3% progesterone ointment. The symptoms of most young girls can be relieved, but they should still be followed up regularly for a long time.

　　V. Surgical Treatment

　　For those with severe illness or ineffective medication, superficial vulvectomy or laser resection can be performed. However, laser resection can only remove epidermal lesions and is ineffective on subcutaneous dermal lesions; surgical resection has a high recurrence rate, not only at the edge of resection, but also the transplanted skin can recur. In addition, the chance of malignancy in this disease is extremely rare, so surgical treatment is rarely used at present.