Vulvar albinism

　　1. Etiology

　　Vulvar albinism is a heterogeneous autosomal recessive genetic disease. Marriage between close relatives plays a significant role. The genetic basis of albinism is determined by a single recessive gene. Melanocytes cannot form melanin, which may be due to a defect in the mechanism of supplying free tyrosine or the inability of tyrosinase to transfer to the pre-melanosome.

　　2. Pathogenesis

　　The histopathology shows clear cells in the basal layer, and negative silver staining cannot prove the presence of melanin. Dopamine staining can be positive (tyrosinase-positive type) or negative (tyrosinase-negative type). According to the molecular pathogenesis, OCA can be divided into tyrosinase-related OCA (i.e., tyrosinase-negative OCA, type I-A; yellow mutation OCA, type I-B; temperature-sensitive OCA, type I-TS; trace pigment OCA, type I-MP), and tyrosinase-independent OCA (type II OCA). Patients with type I OCA have tyrosinase gene mutations leading to the lack of tyrosinase activity (type I-A), reduced activity (types I-B, I-MP), or reduced enzyme activity at higher temperatures (type I-TS). Tyrosinase is a key enzyme in the melanin biosynthesis pathway, and its lack or reduction of activity can lead to reduced or absent skin pigmentation. Patients with type II OCA have P gene deletions or mutations, leading to the loss of function of P protein. P protein is related to the transport of free tyrosine into the melanosome membrane and is an essential protein for melanin synthesis. The loss of P protein function can lead to melanin synthesis disorders.

　　There are no自觉 symptoms in local vulvar leukoderma. The skin lesions in the vulvar area are part of systemic leukoderma, manifested as light yellow pubic hair, vulva, and the skin of the labia majora呈 milky white or pink, the skin of the labia minora, and the vaginal mucosa is bright red. Other manifestations include thin, yellow or silvery white hair, light blue iris, red pupil, frequent aversion to light, tears, nystagmus, and astigmatism. The skin of the whole body is milky white or pink, soft and dry. Due to the lack of melanin protection, the skin is prone to acute and chronic sun damage, and it is easy to develop squamous cell carcinoma and basal cell carcinoma.

　　There are no自觉 symptoms in local vulvar leukoderma. The skin lesions in the vulvar area are part of systemic leukoderma, manifested as light yellow pubic hair, vulva, and the skin of the labia majora呈 milky white or pink, the skin of the labia minora and vaginal mucosa is bright red. Other manifestations include thin, yellow or silvery white hair, light blue iris, red pupil, frequent aversion to light, tears, nystagmus, and astigmatism. The skin of the whole body is milky white or pink, soft and dry. Due to the lack of melanin protection, the skin is prone to acute and chronic sun damage, and it is easy to develop squamous cell carcinoma and basal cell carcinoma.

　　It can be diagnosed according to the congenital onset and clinical manifestations. At birth, there are pure white or pink spots, which are prone to dermatitis after exposure to the sun. The local boundary is distinct, and the histopathology shows transparent cells at the basal layer, with normal quantity and appearance. Silver staining proves the lack of melanin in the epidermis; hair turns white or pale yellow; the iris is pink, the pupil becomes red, and there is aversion to light.

　　1. In daily life, it is advisable to wear loose, breathable underwear and pants, mainly made of cotton, and avoid wearing cotton underwear made of synthetic fibers such as acrylic.

　　2. It should be kept dry, ventilated, and cool at the affected area.

　　3. Some women are too clean, with the habit of washing the vulva once or several times a day. In this case, we suggest that due to the self-cleaning function of the vulva in women, it is generally sufficient to wash 2-3 times a week. Do not use any detergent (as it is generally alkaline) during washing, and only use warm water. Be careful not to use water that is too hot.

　　4. In daily life, attention should be paid to the adjustment of life stress and emotions, maintaining an optimistic mood, and being cheerful. In this regard, the family members of patients should actively cooperate to help them build confidence in overcoming the disease.

　　5. Vaginal leukoderma is a congenital, recessive genetic disease. It belongs to autosomal recessive inheritance. It is a genetic disease of congenital pigmentation deficiency and hair follicle melanin synthesis disorder. Therefore, the prevention of vaginal leukoderma should avoid close marriage. Pay attention to the adjustment of life stress and emotions, maintain an optimistic mood, and be cheerful.

　　The diagnosis of vulvar leukoplakia relies not only on clinical manifestations but also on related examinations, which are indispensable. The methods of examination are as follows:

     1. Genetic testing, tumor marker testing.

　　2. Histopathological examination.

　　First, what foods are good for vulvar leukoplakia patients:

　　1. Eat more foods with anti-vulvar tumor and leukoplakia effects, such as sesame, almonds, wheat, barley, loofah, black-bone chicken, cuttlefish, green mamba, pork pancreas, chrysanthemum, black plum, peach, lychee, spurge, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, tortoise shell.

　　2. Eat horseshoe crabs, red crabs, lobsters, clams, sea cucumber, tiger fish, beets, mung beans, radish, chicken blood when there is pain.

　　3. Eat amaranth, cabbage, rapeseed, taro, kelp, purple kelp, chicken blood, snake meat, pangolin, when there is itching.

　　4. To enhance physique and prevent metastasis, eat silver ear, black fungus, mushrooms, monkey head mushrooms, gizzards, sea cucumber, Job's tears, walnuts, crabs, lizard, needlefish.

　　Second, what foods should not be eaten with vulvar leukoplakia:

　　1. Avoid smoking, alcohol, and spicy刺激性 foods.

　　2. Avoid greasy, fried, moldy, and salted foods.

　　3. Avoid stimulants such as rooster and goose.

　　4. Avoid seafood and irritant, sensitizing foods when itching is severe.

　　5. Avoid warm and spicy foods such as mutton, chives, ginger, pepper, and cassia when there are ulcers and bleeding.

　　First, prevention:

　　1. Avoid marriage between close relatives. OCA protection is very important, and protective glasses should be worn in daily life.

　　2. Traditional Chinese medicine treatment: Vulvar leukoplakia, also known as chronic vulvar dystrophy, refers to a group of diseases in which the skin and mucosa of the female genitalia are nutritionally impaired, leading to tissue degeneration and pigment change. The formation of this disease is often related to nutritional disorders of nerve and blood vessels in the deep connective tissue of the vulva, causing changes in the skin covering it.

　　Traditional Chinese medicine treats the different characteristics of the disease and clinical symptoms according to 'vulvar itching' and 'vulvar boils'. Vulvar itching is accompanied by wind, and vulvar boils are accompanied by dampness and heat.

　　Second, the disease often occurs in women of childbearing age and the elderly, and is rare in young girls. Generally, it is divided into two types:

　　1. Hyperplastic malnutrition: Symptoms include severe vulvar itching, accompanied by local pain after scratching, mainly affecting the labia majora and the interlabial sulcus, often presenting as symmetry, the skin in the disease area thickens like leather, with raised wrinkles, generally without atrophy and adhesion.

　　2. Sclerotic lichen nutrition deficiency: Symptoms include itching in the area of skin lesions, white, thin, dry, and prone to cracking skin or mucosa, loss of elasticity, and clitoral atrophy. Sexual intercourse difficulties may occur in the late stage.

　　Diagnosis should exclude vulvar itching caused by diabetes, mold, and other factors. Consider pathological examination when there are ulcers or obvious hyperplasia to prevent the occurrence of malignancy. In general, observe the body shape, whether there are phenomena such as obesity, thinness, weakness, strength, and whether there are symptoms such as aversion to cold, hot flushes, alternating chills and fever, and cold limbs. Examine the tongue quality, distinguish the tongue fur, and wait for the pulse. Ask in detail: about menstruation, leukorrhea, pregnancy, childbirth, and whether there are any sisters with this disease.

　　Locally, carefully inspect the lesion for cracks, atrophy, ulcers, nodules, adhesions, swelling, exudation, desquamation, and reduced elasticity. The edges of the skin lesions should be even, and the boundaries should be clear. Observe the morphology of these rashes to differentiate them according to cold dampness, heat and dampness stagnation, yang deficiency, phlegm and blood stasis, deficiency of Qi and blood, and the severity and urgency of the disease development. 'The Internal Classic' says 'the kidney opens at the two yin' and 'the liver meridian connects to the genitalia'. The vulvar white lesion is most closely related to the liver and kidney meridians. 'The kidney stores essence' and 'the liver stores blood'. Deficiency of kidney essence and liver blood, lack of nourishment of the vulva, and invasion by wind evil or downward flow of dampness and turbidity are the main causes and pathogenesis of vulvar white lesions. Although vulvar white lesions occur on the vulva, they are actually caused by liver depression, spleen deficiency, kidney deficiency, and disharmony of the冲 and 任 meridians. Cold dampness, heat and dampness stagnation, yang deficiency, phlegm and blood stasis trigger this disease.

　　Vulvar skin plaques with grayish white thickening, severe itching, often resulting in bleeding due to scratching, with excessive leukorrhea, yellowish color, dry mouth and throat, hot and stuffy, yellow urine and hard stools, yellow greasy coating, red edges of the tongue, and wiry and rapid pulse.

　　According to the main clinical characteristics of this disease, it is considered to belong to the category of 'pruritus', 'erosion', 'ulceration' and other categories in traditional Chinese medicine. The pathogenesis is often due to insufficient Yin and blood of the liver and kidney, with blood deficiency in the vulva causing wind and dryness, leading to intolerable itching, or due to deficiency of the spleen qi, where qi deficiency and blood deficiency cannot nourish the vulva, and spleen deficiency can also produce dampness, flowing to the vulva, forming a mixed condition of deficiency and dampness stagnation, or due to internal damp-heat, heat accumulating in the skin and muscles of the vulva leading to vulvar itching and rough skin; the disease enters the collaterals, and the smooth flow of Qi and blood is blocked, leading to stagnation, and interacting with dampness and turbidity, resulting in changes such as lichenification, severe itching, and eczema-like changes, and often not healing for a long time. Clinical treatment is based on the etiology and pathogenesis, either nourishing the liver and kidney, nourishing blood and stopping itching with wind-relieving herbs; or clearing heat and detoxifying to benefit dampness; or activating blood circulation to remove blood stasis and dispel wind; fortifying the spleen to remove dampness and kill worms, treating internally and externally, attacking and reinforcing, which is the key to clinical efficacy.

　　Third, Western medicine treatment methods for leukoplakia of the vulva:

　　Medication: Currently, there is no effective treatment, only symptomatic treatment can be given, avoid sunlight exposure, apply sunscreen, regularly check the body, and pay attention to early detection and timely treatment of skin malignant tumors.