Vulvar Crohn's disease

　　Vulvar Crohn's diseaseMostly occurs in multiple ulcers on the vulva and perineum, it is a chronic inflammatory disease of the gastrointestinal tract that has not been fully clarified.. It may be related to the following factors:

　　1. The disease has a high incidence in the same family, and the incidence rate is also inconsistent among different races. The incidence rate is significantly higher in Jews than in other races, suggesting that its occurrence may be related to genetic factors. However, it has been found that Crohn's disease has specific histocompatibility antigens.

　　2, bacterial, fungal, and viral infections may be related to the onset of Crohn's disease, but there is no direct evidence so far.

　　3, immune factors.

　　The characteristic lesions of vulvar Crohn's disease are non-caseating granuloma infiltration of subcutaneous tissue, and extension to subcutaneous fat. It is manifested as tissue hyperplasia, hypertrophy, and ulcer formation. Granulomas are mainly composed of epithelioid cells, multinucleated giant cells with infiltrating lymphocytes, and have a structure similar to granulomatous perivascular inflammation. The pathological manifestations of granulomas are consistent with the components of intestinal lesions, and the typical histological features include inflammatory cell infiltration, ulceration, and abscess formation. Non-caseating granulomas can be found in 10% to 25% of the lesions. 22% to 75% of Crohn's disease patients have related mucocutaneous symptoms. It is rare for Crohn's disease to involve the vagina. Burgdorf et al. classify extra-intestinal damage of Crohn's disease into four categories: 1, skin granulomas include perianal rectal sinus tract and fistula formation; 2, aphthous ulcers of oral mucosa; 3, nutritional changes such as zinc, iron, and folic acid deficiency; 4, idiopathic lesions include erythema multiforme, acquired bullous epidermolysis, necrotizing vasculitis. Among them, perianal skin lesions are the most common, and in 25% of Crohn's disease cases, the initial symptom is manifested as perihepatic lesions.

　　Vulvar Crohn's disease can complicate with vulvar abscess and other infectious lesions. Vulvar abscess is an acute suppurative infection of a single hair follicle and its associated sebaceous glands caused by Staphylococcus aureus or Staphylococcus albus and other pathogens, also known as carbuncle. Multiple carbuncles occur simultaneously or repeatedly, become chronic, and are called carbunculosis. Vulvar abscess and carbunculosis have the same pathogen as vulvar folliculitis.

　　Most patients with vulvar Crohn's disease have associated ileocolitis or a history of intestinal resection. Clinical manifestations of vulvar lesions in Crohn's disease can include vulvar swelling, vulvar tenderness, perineal ulcers, skin fissures, and skin tags. Other rare symptoms include bilateral or unilateral vulvar hypertrophy, erythema multiforme, vulvar abscess, perineal fistula or sinus tract formation, and so on. The manifestations of vulvar lesions in Crohn's disease are diverse and are easily misdiagnosed. Especially when there are no intestinal symptoms as the initial symptoms of Crohn's disease, more than half of the patients may also experience menstrual abnormalities.

　　Common symptoms include fever, abdominal pain, diarrhea, and easy fatigue. Some patients may experience abdominal distension, lower abdominal mass, and 14% to 17% of patients may have perianal fistulas and sinus tracts. Among them, 21% of the fistulas are connected to the peritoneum. Extra-intestinal damage includes joints, skin, liver and gallbladder, which can manifest as arthritis, fatty liver, pericholangitis, sclerosing cholangitis, perihepatic abscess. Other rare symptoms include nodular erythema, gangrenous pyoderma, malabsorption syndrome, glossitis, pharyngeal ulcers, and so on.

　　Patients with vulvar Crohn's disease should have regular physical examinations and early detection and treatment. Pay attention to the protection of the vulva, and it is also very important to keep the affected area dry and clean. The affected area can be cleaned with a senna root wash solution.

　　Vulvar Crohn's disease is a multiple ulcer occurring in the female vulva, which is a chronic inflammatory disease of the gastrointestinal tract with unknown etiology, characterized by segmental specific lesions in all segments of the gastrointestinal tract from the mouth to the anus. The external vulvar lesions of Crohn's disease are diverse and easily misdiagnosed. Particularly, when there are no intestinal symptoms as the initial symptom of Crohn's disease, more than half of the patients may still have menstrual abnormalities. Therefore, the examinations that should be done for vulvar Crohn's disease include occult blood test (OBT), fecal bacterial culture, urine routine, blood routine, detection of essential trace elements, stool routine, immunopathological examination, blood trace element examination, blood routine examination, erythrocyte sedimentation rate, and other examinations. If there are lesions in other parts, corresponding examinations should be performed to assist in the examination. In addition, pathological examination of living tissue should be performed.

　　For patients with vulvar Crohn's disease, easily digestible protein foods such as milk, eggs, fish, and soy products can improve the body's anti-cancer ability. Eating more foods rich in zinc and folic acid is also very important.Avoid stimulants such as coffee, alcohol, and other beverages. Avoid spicy and irritating foods such as scallions, garlic, ginger, and cinnamon..

　　The treatment of vulvar Crohn's disease mainly adopts internal medicine treatment and local care. If gastrointestinal inflammation is present, gastrointestinal lesions should be treated first.

　　1. The first-line drug is sulfasalazine (SASP) taken orally at 2-4g/d. If symptoms are not relieved after 1-2 months of monotherapy, high-dose glucocorticoids can be used in combination.

　　2. Metronidazole can be used as a second-line drug for SAPS or used during the interval of corticosteroid treatment. It is reported that metronidazole 25mg (kg?d) treatment for 6 months can alleviate vulvar edema symptoms, but is ineffective for vulvar erythema.

　　3. Glucocorticoids are suitable for patients with fulminant or severe types. Local hormone injection can be performed.

　　4. Immunosuppressive therapy can be considered, such as oral azathioprine, generally given at 1.5mg (kg/d), taken in divided doses. There are also reports that local injection of azathioprine 40mg has a good effect on relieving vulvar swelling and pain.

　　5. Consider surgical treatment for those who are ineffective to the above treatments.