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VULVA: BENIGN AND INFLAMMATORY CONDITIONS OF THE VULVA
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Childhood LS |
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Early LS |
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Advanced LS |
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"Classic" LS |
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INTRODUCTION/HISTOPATHOLOGY
In 1987, the international society for the study of vulvovaginal diseases proposed a classification for the non-neoplastic epithelial disorders of the vulvar skin and mucosa. These entities had previously been incorporated under the designation of vulvar dystrophies. This new classification in contrast to the prior classification, which has been based purely on histopathologic features of the lesions, was based on gross and histopathologic features. It is as follows:
- Squamous cell hyperplasia (formerly hyperplastic dystrophy).
- Lichen sclerosus.
- Other dermatoses.
Lichen sclerosus has formerly been broken down into two subgroups including pure lichen sclerosus and lichen sclerosus associated with squamous cell hyperplasia classified as a mixed dystrophy. The current classification recommends that lichen sclerosus with associated squamous cell hyperplasia should be reported as such rather than as a mixed dystrophy. Epithelial lesions associated with cellular atypia are currently classified under vulvar intraepithelial neoplasia. In most instances a clinical impression can be established on the basis of gross inspection alone, however, this is not always possible and classification of the lesion may be dependent upon its microscopic features. This is important, since the management of the patient will vary significantly depending upon the disease being treated.
Lichen sclerosus was first described by Henri Hallopeau in 1897 who referred to it as lichen planus atrophicus. Lichen sclerosus represents a specific dermatological entity. It is most commonly seen in the genital area in women. It may also be found on other sites of the body. The gross and microscopic appearance and the clinical course of lichen sclerosus are characteristic.
Lichen sclerosus is primarily a disease of the postmenopausal woman, however, it can be seen at any age and not uncommonly is even seen in children (Fig. 1).
CLINICAL
Pruritus is the most common symptom seen in association with lichen sclerosis. This was observed in 99% of the patients we evaluated. The next most common symptom was that of vulvar irritation seen on 60.5% of women. Burning and dyspareunia were each seen in almost 30% of women studied (Table 3). In the presence of introital stenosis, dyspareunia is a frequently complaint.
RELATIONSHIP TO CARCINOMA
In the early part of this century, the common wisdom suggested that there was a strong relationship between the presence of lichen sclerosis and invasive squamous cell carcinoma of the vulva. It was felt that if left untreated, almost all patients with this problem would ultimately develop an invasive squamous cell carcinoma. More recent studies have proven this premise to be completely false. In fact, the risk of a woman with lichen sclerosis developing invasive squamous cell carcinoma of the vulva is extremely small, varying between 2 and 5%. In our own experience, we have seen only three patients in over 200 women with lichen sclerosis develop invasive squamous cell carcinoma over a period of 5 to more than 20 years. Invariably, the patient who did develop squamous cell carcinoma was the one who was negligent regarding therapy and continued to have pruritus with associated scratching.
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TREATMENT
Currently, the appropriate treatment for lichen sclerosis is one of the high potency corticosteroids, clobetasol propionate. This may be utilized either in the form of the cream or ointment, and many clinicians prefer the ointment because it appears to be somewhat less irritating than the cream. The routine we currently utilize is to have the patient rub the clobetasol into the vulva twice daily for one month; then at bedtime for two months; and then twice weekly for three months. Following this routine, we observed complete remission of symptoms in 77% of the women studied. In 18% of the women, there was partial remission of symptoms and in 5% of our patients; there was no change in the symptoms of pruritus. In almost 1/3 of our patients, there was complete regression of the changes of lichen sclerosis; in 46% there was partial remission of the changes; and in 22% of women, the clinical appearance of the lichen sclerosis was unchanged. Bracco compared the effectiveness of clobetasol propionate cream with treatment using 2% testosterone, 2% progesterone and a cream-base alone. The findings noted that topical clobetasol was the most effective drug in relieving symptoms and improving objective and histopathologic findings. Whereas the long term use of high potency are purported to result in adverse changes on the vulva such as atrophy and striai, the long-term use of clobetasol in patients with lichen sclerosis does not appear to have any adverse effects.
After the completion of the above routine, the patient is re-examined and advised to use the clobetasol on an as needed basis once or twice a week. Not uncommonly, when women complain of a sudden recurrence of vulvar pruritus, this is associated with another cause for pruritus such as a candidal infection, and it is our routine to obtain a culture for candida when this occurs . as well as before the original initiation of therapy so that any such infection can be eradicated.
In patients who have persistent debilitating pruritus despite the above therapy, there are two other approaches that we occasionally find to be effective. One of these, as suggested by Woodruff and Thompson, utilizes 5 mg of Triamcinolone suspension diluted in 2 ml of normal saline injected subcutaneously beneath the skin of both labia majora. We utilize a 3-inch 21-gauge spinal needle that is inserted into the upper portion of the labium majus and passed down subcutaneously toward the perineum. The suspension is then slowly injected as the needle is withdrawn. Following this, the tissues are massaged to aid distribution of the suspension. This will often result in relief of the pruritus following which the patient stops scratching the vulvar tissue. By the time the effects of the injections have abated, the symptom of pruritus remains quite mild and is even absent and can usually be controlled by topical medication. When this fails, we will rarely resort to a subcutaneous injection of alcohol. This approach requires hospitalization and anesthesia of the patient. A grid-like map is painted on the vulva so that the lines meet at 1 cm intervals. Aliquots of 0.1 to 0.2 ml of absolute alcohol are injected at 1 cm intervals. The tissues are then gently massaged to facilitate distribution of the alcohol. This will usually result in relief of pruritus and the relief may persist for a period of six months to a year following which sensation gradually returns to the vulvar tissues, often with no recurrence of the pruritus. Of prime importance, however, is the fact that alcohol injection is of absolutely no value in the patient complaining of vulvar burning. If the alcohol is injected intradermally, there may be focal sloughing of the skin. This is usually associated with ulceration and severe vulvar pain with ultimate healing and disappearance of the discomfort.
Lichen sclerosis in the child is usually aimed primarily at relief of pruritus. This can usually be accomplished with the use of a low to mid potency steroids, but at times higher dose topical steroids may be required.
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