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The disease often goes undiagnosed for several years, as it is sometimes not recognized and misdiagnosed as thrush or other problems and not correctly diagnosed until the patient is referred to a specialist when the problem does not clear up.
A biopsy of the affected skin can be done to confirm diagnosis. When a biopsy is done, hyperkeratosis, atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis are histological findings associated with LS. The biopsies are also checked for signs of dysplasia.
It has been noted that clinical diagnosis of LS can be "almost unmistakable" and therefore a biopsy may not be necessary.
The prognosis for vaginal atresia is one that is complicated. There are variations in patients' anatomic findings as well as an absence in consistent surgical techniques which makes it difficult to give a prognosis for this condition. Along with other conditions that give rise to an abnormal perineum (i.e. ambiguous genitalia and other various abnormalities that range from cloaca to urogenital sinus), individuals with vaginal atresia often report reconstruction as an outcome of treatment. Due to this, it is difficult to compare outcomes between individuals with vaginal atresia.
Fertility options for girls and women with Rokitansky-Mayer-Küster-Hauser syndrome has a bit more information. Girls and women who are born without a complete vagina, but still have a regular sized uterus more than likely will be able to become pregnant and have a baby. However, if the female is born with a tiny uterus, or without a uterus, they will not be able to have a baby. As the ovaries may be normal in this case, the egg may be fertilized with a donor's or partner's sperm. In this case, surrogacy, would be an option where there will be a gestational carrier to carry the pregnancy for the couple. Adoption may also be an option for females with Rokitansky-Mayer-Küster-Hauser syndrome. Another possibility could be uterine transplants, however this a new and developing form of treatment. Fertility options are being researched daily, so there can always be a new method available.
Any pain associated with Rokitansky-Mayer-Küster-Hauser syndrome comes from menstruation related cramping and can be treated with several ways. Individuals with this syndrome may be born with a uterine remnant (tiny uterus), which can fill with become filled with blood in the pelvic cavity causing pain. A medical professional can assess the severity of having a uterine remnant within each patient to determine if removal of the uterus is necessary.
The main causes are Müllerian agenesis and complete androgen insensitivity syndrome.
In order to facilitate sexual intercourse, the main treatments are self-dilation methods (using intra-vaginal cylinders of increasing size) and surgical vaginoplasty to lengthen the vagina.
Self-dilation has a high success rate, estimated at 75%, and is usually the first-line treatment due to low surgical invasiveness. Overall, the complication rates are significantly lower with dilation than with vaginoplasty.
Surgery is indicated when there is inability or reluctance to perform self-dilation, or where it is performed but with failed result. One appropriate surgical variant is the "Vecchietti technique". In this procedure, an olive-shaped pressure device is pressed towards the potential vaginal space by a thread that goes through the skin, behind the urinary bladder and pubic bone and exits the skin in the hypogastrium, where it is attached to a plate that provides counter-traction. Vaginoplasty can also be performed using a skin graft or an intestinal graft. Traction vaginoplasty such as the "Vecchietti technique" seems to have the highest success rates both anatomically (99%) and functionally (96%), whereas skin graft procedures and intestinal procedures have the lowest successful outcomes (83–95%).
After vaginoplasty, available evidence suggests that continued self-dilation is needed to maintain patency in periods of coital inactivity.
The diagnosis of cervical agenesis can be made by magnetic resonance imaging, which is used to determine the presence or absence of a cervix. Although MRI can detect the absence of a cervix (agenesis), it is unable to show cervical dysgenesis (where the cervix is present, but malformed). Ultrasound is a less reliable imaging study, but it is often the first choice by gynecologists to establish a diagnosis and can identify a hematometra secondary to cervical agenesis.
The condition is one of exclusion and other vulvovaginal problems should be ruled out. The diagnosis is based on the typical complaints of the patient, essentially normal physical findings, and the absence of identifiable causes per the differential diagnosis. Cotton swab testing is used to differentiate between generalized and localized pain and delineate the areas of pain and categorize their severity. Patients often will describe the touch of a cotton ball as extremely painful, like the scraping of a knife. A diagram of pain locations may be helpful in assessing the pain over time. The vagina should be examined, and tests, including wet mount, vaginal pH, fungal culture, and Gram stain, should be performed as indicated. Fungal culture may identify resistant strains.
Many sufferers will see several doctors before a correct diagnosis is made. Many gynecologists are not familiar with this family of conditions, but awareness has spread with time. Sufferers are also often hesitant to seek treatment for chronic vulvar pain, especially since many women begin experiencing symptoms around the same time they become sexually active. Moreover, the absence of any visible symptoms means that before being successfully diagnosed many patients are told that the pain is "in their head".
Lichen planus has a unique microscopic appearance that is similar between cutaneous, mucosal and oral. A Periodic acid-Schiff stain of the biopsy may be used to visualise the specimen. Histological features seen include:
- thickening of the stratum corneum both with nuclei present (parakeratosis) and without (orthokeratosis). Parakeratosis is more common in oral variants of lichen planus.
- thickening of the stratum granulosum
- thickening of the stratum spinosum (acanthosis) with formation of colloid bodies (also known as Civatte bodies, Sabouraud bodies) that may stretch down to the lamina propria.
- liquefactive degeneration of the stratum basale, with separation from the underlying lamina propria, as a result of desmosome loss, creating small spaces (Max Joseph spaces).
- Infiltration of T cells in a band-like pattern into the dermis "hugging" the basal layer.
- Development of a "saw-tooth" appearance of the rete pegs, which is much more common in non-oral forms of lichen planus.
About 5-8% of the reproductive age female population will have four or more episodes of symptomatic Candida infection per year; this condition is called recurrent vulvovaginal candidiasis (RVVC). Because vaginal and gut colonization with Candida is commonly seen in people with no recurrent symptoms, recurrent symptomatic infections are not simply due to the presence of Candida organisms. There is some support for the theory that RVVC results from an especially intense inflammatory reaction to colonization. Candida antigens can be presented to antigen presenting cells, which may trigger cytokine production and activate lymphocytes and neutrophils that then cause inflammation and edema.
The disease can last for a considerably long time. Occasionally, "spontaneous cure" may ensue, particularly in young girls.
Lichen sclerosus is associated with a higher risk of cancer. Skin that has been scarred as a result of lichen sclerosus is more likely to develop skin cancer. Women with lichen sclerosus may develop vulvar carcinoma. Lichen sclerosus is associated with 3–7% of all cases of vulvar squamous cell carcinoma. In women, it has been reported that 33.6 times higher vulvar cancer risk is associated with LS. A study in men reported that "The reported incidence of penile carcinoma in patients with BXO is 2.6–5.8%".
Sexually transmitted disease that affect the vagina include:
- Herpes genitalis. The herpes simplex virus (HSV) can infect the vulva, vagina, and cervix, and this may result in small, painful, recurring blisters and ulcers. It is also common for there to be an absence of any noticeable symptoms.
- Gonorrhea
- Chlamydia
- Trichomoniasis
- Human papillomavirus (HPV), which may cause genital warts.
HIV/AIDS can be contracted through the vagina during vaginal intercourse, but it is not associated with any local vaginal or vulval disease.
Because of STIs, health authorities and other health outlets recommend safe sex practices when engaging in sexual activity.
Lichen planus lesions are diagnosed clinically by their "lichen-like" appearance. A biopsy can be used to rule out conditions that may resemble lichen planus, and can pick up any secondary malignancies.
Vaginitis an inflammation of the vagina, such as caused by infection, hormone disturbance and irritation/allergy.
For infrequent recurrences, the simplest and most cost-effective management is self-diagnosis and early initiation of topical therapy. However, women whose condition has previously been diagnosed with candidal vulvovaginitis are not necessarily more likely to be able to diagnose themselves; therefore, any woman whose symptoms persist after using an over the counter preparation, or who has a recurrence of symptoms within 2 months, should be evaluated with office-based testing. Unnecessary or inappropriate use of topical preparations is common and can lead to a delay in the treatment of other causes of vulvovaginitis, which can result in worse outcomes.
When there are more than four recurrent episodes of candidal vulvovaginitis per year, a longer initial treatment course is recommended, such as orally administered fluconazole followed by a second and third dose 3 and 6 days later, respectively.
Other treatments after more than four episodes per year, may include ten days of either oral or topical treatment followed by fluconazole orally once per week for 6 months. About 10-15% of recurrent candidal vulvovaginitis cases are due to non-"Candida albicans" species. Non-"albicans" species tend to have higher levels of resistance to fluconazole. Therefore, recurrence or persistence of symptoms while on treatment indicates speciation and antifungal resistance tests to tailor antifungal treatment.
Diagnosis is typically suspected based on a women's symptoms. Diagnosis is made with microscopy (mostly by vaginal wet mount) and culture of the discharge after a careful history and physical examination have been completed. The color, consistency, acidity, and other characteristics of the discharge may be predictive of the causative agent. Determining the agent is especially important because women may have more than one infection, or have symptoms that overlap those of another infection, which dictates different treatment processes to cure the infection. For example, women often self-diagnose for yeast infections but due to the 89% misdiagnosis rate, self-diagnoses of vaginal infections are highly discouraged.
Another type of vaginitis, called desquamative inflammatory vaginitis (DIV) also exists. The cause behind this type is still poorly understood. DIV corresponds to the severe forms of aerobic vaginitis. About 5 to 10% of women are affected by aerobic vaginitis.
The International Statistical Classification of Diseases and Related Health Problems codes for the several causes of vaginitis are:
The following measures are advisable for keeping the vulva and vagina healthy:
- Avoiding washing with soap, since soap disturbs the natural pH balance of the vagina. Some pH-balanced soaps exist, but their effects remain unclear. Other measures are seldom necessary or advisable. Two notable examples: so-called "feminine hygiene sprays" are unnecessary, may be generally harmful, and have been known to cause severe allergic reactions. Vaginal douching is generally not necessary and has been implicated in helping to cause bacterial vaginosis (BV) and candidiasis ("yeast infections").
- After using the toilet, wipe from the front toward the back to avoid introducing bacteria from the anal area into the vulva. Use non-perfumed, undyed toilet paper.
- Drink plenty of water and urinate frequently and as soon as possible when you feel the need, to help flush bacteria out of the urinary tract and avoid urinary tract infections. For the same reason, try to urinate before and after sex.
- Change out of a wet swimsuit or other wet clothes as soon as possible.
- Avoid fragrances, colours, and "deodorants" in products that contact the vulva/vagina: sanitary napkins, tampons, and toilet paper. Some women who are sensitive to these substances should also avoid bubble baths and some fabric detergents and softeners.
- Use a menstrual cup instead of sanitary pads or tampons. Menstrual cups are a new form of menstrual product that has recently been rapidly gaining in popularity as a greener, more cost-effective and healthier alternative to tampons and sanitary pads.
- Avoid wearing leather trousers, tight jeans, panties made of nylon or other synthetic fabrics, or pantyhose without an all-cotton crotch (not cotton covered by nylon - cut out the nylon panel if necessary).
- Anything which has been in contact with the anal area (see anal sex) should be thoroughly washed with soap and water or a disinfectant before coming in contact with the vulva or vagina.
- Use condoms during heterosexual intercourse, practise safer sex, know your sex partners, ask sex partners to practise basic hygiene of their genitals. Use artificial lubrication during the intercourse if the amount naturally produced is not enough.
- Be careful with objects inserted inside the vagina. Improper insertion of objects into any body opening can cause damage: infection, cutting, piercing, trauma, blood loss, etc.
- Avoid letting any contaminants inside the vagina, including dirt but especially sand.
- See your gynecologist regularly.
- Be careful while removing hair in this area.
Women who are unable to walk are more likely to have infections. The problem can be prevented according to above-mentioned and following measures:
- Wash crotch and rectal areas (with a soap-substitute if desired) and large amount of warm running water, every morning and evening. The disabled person can sit on a shower chair with an open seat or on a toilet. Use a shower head or water container to wash more directly.
- Use towel to dry.
- DO NOT use talcum/body powder, if desired use corn starch powder (corn-flour) on the skin of the genital area to absorb perspiration.
- Avoid sitting on plastic or synthetic materials for extended lengths of time.
- Wear loose underpants and change if soilage or wetness occurs.
Prevention of candidiasis, the most common type of vaginitis, includes using loose cotton underwear. The vaginal area should be washed with water. Perfumed soaps, shower gels, and vaginal deodorants should be avoided. Douching is not recommended. The practice upsets the normal balance of yeast in the vagina and does more harm than good.
Prevention of bacterial vaginosis includes healthy diets and behaviors as well as minimizing stress as all these factors can affect the pH balance of the vagina.
Prevention of trichomoniasis revolves around avoiding other people's wet towels and hot tubs, and safe-sex procedures, such as condom use.
Some women consume good bacteria in food with live culture, such as yogurt, sauerkraut and kimchi, or in probiotic supplements either to try to prevent candidiasis, or to reduce the likelihood of developing bacterial vaginitis following antibiotic treatment. There is no firm evidence to suggest that eating live yogurt or taking probiotic supplements will prevent candidiasis.
Studies have suggested a possible clinical role for the use of standardized oral or vaginal probiotics in the treatment of bacterial vaginosis, either in addition to or in place of the typical antibiotic regimens. However, recent articles question their efficacy in preventing recurrence compared with other means, or conclude that there is insufficient evidence for or against recommending probiotics for the treatment of bacterial vaginosis.
The first line of therapy after diagnosis typically involves the administration of the combined oral contraceptive pill, medroxyprogesterone acetate or a gonadotropin-releasing hormone agonist to suppress menstruation and thereby relieve pain. Surgically, cervical agenesis has historically been treated through hysterectomy (removal of the uterus) to relieve symptoms caused by hematocolpos (the accumulation of menstrual fluid in the vagina). Other surgical methods of management involve the creation of an anastomotic connection between the uterus and vagina by neovaginoplasty or recanalization of the cervix. Outcomes in these cases are generally poor, since the natural functions of the cervix—such as mucus production and providing a barrier against ascending infection—cannot be replicated. Furthermore, the success rate of uterovaginal anastomosis is less than 50% and most patients require multiple surgeries while many develop cervical stenotis. Despite this, several pregnancies have been reported in women with cervical agenesis who underwent surgical treatment.
Diagnosis is readily made by the cotton-swab test, in which pressure is applied in a circular fashion around the vulvar vestibule to assess complaints of pain. Laboratory tests are used to exclude bacterial or viral infection, and a careful examination of the vulvo/vaginal area is conducted to assess whether any atrophy is present.
Vestibulectomy, during which the nerve fibers to the area are cut out, may be recommended if other treatments have not been found to be effective. There have been no high quality studies looking at surgery as a treatment. While improvement has been noted in 60% to 90%, those who were treated without surgery improved in 40 to 80% of cases.
With only a small number of case reports, treatment can be difficult and focuses primarily on axillary disease and specific symptoms. First line treatment includes topical and intalesional glucocorticoids. Specific therapies include estrogen hormones and steroid creams. Use of tretinoin reduced pruritic and axillary papules in one study. However, irritation limited drug use to short term only. Clindamycin mixed with pimecrolimus cream and benzoyl peroxide was also effective. Clindamycin is used to inhibit bacterial growth and is used when treating staphylococcal and streptococcal infections.
Proper treatment will usually relieve the symptoms, at least to some extent.
Clinical evaluation along with identification of typical symptoms such as the eruption of papules on the apocrine glands along with a patient history report is used to diagnose the disease. Surgical removal along with a microscopic evaluation by a dermatopatholgist is the most reliable form of diagnosis.
Among individuals being treated in intensive care units, the mortality rate is about 30-50% when systemic candidiasis develops.
Use of vaginally administered estrogens (including vaginal tablets or cream) is appropriate before the condition becomes severe. Regular sexual activity may be helpful. A water-soluble vaginal lubricant can be helpful in mild cases.
Increasingly, vaginally administered estrogens based on low dose of estriol are used to stimulate the vaginal epithelium proliferation. There is growing evidence to support the use of both Fractional Erbium and Fractional CO2 laser therapy, both have proven to be an effective treatment strategy, especially for patients such as cancer survivors for whom vaginal estrogen is not always an option. The characteristic of both Erbium and CO2 laser wavelengths is that they are highly absorbed within water. It is the water within the sub mucosa that is targeted by the laser. The hypothesised mode of action for Erbium laser is that through selectively heating the submucosa a process of neocollagenesis and neo vascularisation occurs. This can lead to an improvement of the blood flow and overall health of the treated area. Treatments take approximately 20 minutes and can be performed within an outpatient setting.