Vulvar Paget's disease

　　1. Etiology

　　The etiology is unknown, it may originate from large sweat glands or Bartholin's glands, and then along the duct to the epidermis. Additionally, the disease may also originate from in situ epithelial stem cells. It may be related to factors such as arsenic agents, viral infection, local injury stimulation, and genetics.

　　2. Pathogenesis

　　This disease is an intraductal adenocarcinoma originating from apocrine and eccrine sweat glands, as these lesions manifest as apocrine secretion, it is believed that the malignant cells originate from undifferentiated basal cells. 'Transformed cells' spread intradermally and may reach additional types of cells after penetrating the squamous epithelium. It is transformed into additional types of cells during the process of tumor formation.

　　In most patients with potential invasive tumors in organs with secretory functions such as sweat glands, Bartholin's glands, or anal-rectal areas, malignant cells migrate to the epidermal layer through the duct-like structures of the skin. About 70% of the lesions are in the epidermal layer. The epidermis thickens and shows acanthosis, with Paget cells infiltrating the deep layer of the epidermis. As the Paget cells further proliferate, they can spread to the entire epidermis, epidermal appendages (hair follicles, sebaceous glands, sweat glands). The vulvar surface has exudative crusts or keratotic desquamation, which often spreads to the anal area, occasionally infiltrating the medial thigh, the urinary meatus, the vagina, the cervix, the anal canal, and is rarely involved. Local lymph node metastasis and metastasis to other parts may also occur. The mucin carmine reaction is also positive; the mucin shows a positive mucicarmine stain.

　　Under the microscope, single or small groups of Paget cells are visible in the deep layer of the epidermis, especially at the tip of the rete ridges. Paget cells are large round cells with abundant cytoplasm,呈透明空泡状, stained lightly, with large nuclei that are round or oval, some with folds, and small but distinct nucleoli, often located on one side, with few nuclear divisions. The nuclear-cytoplasmic ratio is 1:3, and the chromatin is fine and scattered. Paget cells contain acidic and neutral mucopolysaccharides, and show a positive Alcian blue reaction; they show a positive PAS reaction before and after amylase treatment. There are no multinucleated keratinocytes in the epidermis, and Paget cells do not enter the dermis from the epidermis, but can extend into the epidermal appendages (hair follicles, sebaceous glands, sweat glands), and may be accompanied by sweat gland cancer. Lymphocytes and a few plasma cells infiltrate the dermal layer.

　　Vulvar Paget's disease often occurs simultaneously or sequentially with other cancers. Commonly associated cancers include breast cancer, Bartholin's gland cancer, basal cell carcinoma of the skin, bladder cancer, gallbladder cancer, and invasive adenocarcinoma of the vulva. Malignant tumors involving the cervix, colon, bladder, and breast account for about 30%. When the anal mucosa is involved, it usually indicates the presence of potential rectal adenocarcinoma.

　　Generally, there are no自觉symptoms, but a few have varying degrees of itching and burning pain, the course of the disease is slow, and it can last for many years. The lesion begins at the hairy part of the vulva, the genital creases, and the perianal area, and then spreads to the labia minora. It often appears during long-term standing. The lesion observed with the naked eye is a clear red eczema-like patch with exudative crusts or keratotic desquamation on the surface. It often spreads to the anal area, occasionally infiltrating the medial thigh, the urinary meatus, the vagina, the cervix, and the anal canal, which are rarely involved. After removing the crust, a bright red, fine-grained erosive surface is exposed. The dermatological lesions have clear boundaries but irregular edges. The red and white areas in the lesion are respectively the ulcerated area and the highly keratinized area. Some lesions are soft and homogeneous erythema. The surgical specimens with infiltration in the vestibular lesions are more common, and if ulcers appear, they are often signs of invasive growth. About 20% of patients with this disease have invasive adenocarcinoma of the vulva, and clinical invasive cancer is more common than microscopic invasive cancer. Patients with vulvar Paget's disease have an increased risk of adenocarcinoma in other parts, especially the anal and breast areas, and invasive adenocarcinoma occurring in recurrent Paget's disease is rare.

　　Wider lesions are usually raised and often start at the area where the vulva has hair.

　　1. There is a higher chance of accompanying or developing malignant tumors in this disease, and the patient should have a comprehensive examination and long-term follow-up to observe the possibility of other tumors.

　　2. Try to use arsenic agents as little as possible and avoid using strong long-wave ultraviolet radiation. If there are multiple skin lesions, attention should be paid to the presence of other malignant tumors.

　　3. Prognosis: 20% to 30% may develop into invasive cancer or even metastasis.

　　The diagnosis of vulvar Paget's disease, in addition to clinical manifestations, also requires essential related examination methods. The following are the examinations:

     1. Secretion examination, tumor marker examination, polymerase chain reaction.

　　2. Histopathological examination.

　　One, vulvar Paget's disease food therapy formula

　　1. He Shou Wu egg soup

　　Composition: 120 grams of He Shou Wu, 4 eggs.

　　Usage: Boil He Shou Wu to make a strong decoction, and boil 4 eggs. This is a daily dose, taken twice a day.

　　2. Sesame brown sugar porridge

　　Composition: 200 grams of black sesame, 30 grams of brown sugar.

　　Usage: Clean black sesame, slightly fry, and store in a bottle for later use or crush and store in a bottle. Use 2 tablespoons with an appropriate amount of brown sugar, dip in steamed buns or dissolve in boiling water for consumption.

　　3. Others

　　Composition: 200 grams of walnut kernel, 100 grams of sesame, 100 grams of glutinous rice.

　　Usage: Grind walnuts and sesame separately. Boil glutinous rice with an appropriate amount of water, then add walnuts and sesame seeds and it is ready to eat.

　　4. He Shou Wu and Chinese yam lamb soup

　　Composition: 30 grams of He Shou Wu, 100 grams of Chinese yam, 500 grams of lamb meat, 9 grams of ginger.

　　Two, what foods are good for vulvar Paget's disease

　　1. Provide easily digestible protein foods such as milk, eggs, fish, and soy products, which can enhance the body's anti-cancer ability. Among them, milk and eggs can improve protein disorder after radiotherapy.

　　2. Consume an appropriate amount of carbohydrates to supplement calories. For patients undergoing high-dose radiotherapy, this can destroy the sugar metabolism within the body, causing a sharp drop in glycogen and an increase in lactic acid in the blood, making it unusable; and insufficient insulin function can be exacerbated. Therefore, supplementing with glucose is more effective, and it is also recommended to eat more foods rich in sugar such as honey, rice, flour, and potatoes to supplement calories.

　　3. Eat more foods with anti-cancer properties, such as turtle, mushrooms, black fungus, garlic, seaweed, mustard vegetables, and royal jelly, etc.

　　4. Drinking oolong tea regularly has certain anti-cancer effects.

　　5. Regular consumption of bee milk can enhance the body's immunity and has anti-cancer effects.

　　6. The chlorophyll in sprouts can prevent colorectal cancer and some other cancers.

　　Three, it is best not to eat certain foods for vulvar Paget's disease.

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

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

　　3. Avoid broilers, geese, and other triggering substances.

　　4. Avoid seafood and刺激性, allergenic foods when itching is severe.

　　5. Avoid warm foods such as mutton, chives, ginger, pepper, cinnamon, etc., when ulcers and bleeding occur.

　　1. Precautions Before Treatment of Vulvar Paget's Disease

　　1. Before treatment: It is necessary to have a detailed understanding of the symptoms of the disease and related contraindications.

　　2. Traditional Chinese Medicine Treatment Methods for Vulvar Paget's Disease: Huachansu 2ml is injected intramuscularly once a day for 1 month, and also used externally, 3 times a day. Some patients can be cured clinically.

　　3. Western Treatment Methods for Vulvar Paget's Disease: The treatment of vulvar Paget's disease is mainly surgical resection. For solitary lesions, extensive resection of the lesion can be performed, and for multicentric or extensive lesions, simple vulvectomy can be performed. The extent of spread of Paget's disease lesions often exceeds what can be seen with the naked eye, so the margin often shows positive pathological changes. If the surgical margin is positive, the recurrence rate is very high, therefore, frozen section examination of the margin should be performed during surgery to ensure that the lesion is completely resected.

　　4. Regardless of the surgical method adopted, the normal skin margin should be more than 2.5 cm away from the lesion, and the depth should be 0.5 to 1 cm to the subcutaneous fat tissue below the skin. Any mass below the lesion should be removed and sent for pathological examination to exclude malignancy. For those with invasive cancer below the lesion, radical vulvectomy and inguinal lymph node dissection may be performed if necessary. If the range of resection is large and the wound is large, skin grafting may be required.

　　2. Local Treatment

　　There are reports that applying fluorouracil (5-Fu) ointment to the lesion is effective. 1% fluorouracil (5-Fu), propylene glycol, or 0.7% cantharidin acetone gelatin mixture can be used for packing. External application of podophyllotoxin also has certain efficacy.

　　3. Physical Therapy

　　There are electric cautery, electrocoagulation, cryotherapy, superficial X-ray, 32P or 60Co radiotherapy, and other treatments.

　　4. Laser Therapy

　　Regarding laser therapy for recurrent vulvar Paget's disease, it is safe in the absence of adenocarcinoma. Due to the high rate of malignancy of the disease, laser ablation should not be used, at least not for the treatment of the primary lesion. If there is invasive cancer beneath it, laser therapy should not be used.