Lichen Sclerosus

Introduction/Histopathology

Childhood LS

Early LS

Advanced LS

"Classic" LS

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:

1. Lichen sclerosus.
2. Squamous cell hyperplasia (formerly hyperplastic dystrophy).
3. 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 appearances 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 commonly is seen in children (Fig. 1).

Clinical

Pruritus is the most common symptom seen in association with lichen sclerosus. 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 frequent 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 sclerosus 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 sclerosus 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 sclerosus 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.

Treatment

Currently, the appropriate treatment for lichen sclerosus is one of the high potency corticosteroids, clobetasol propionate 0.05%. 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 long term treatment. 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 pruritus. In almost 1/3 of our patients, there was complete regression of the changes of lichen sclerosus; in 46% there was partial remission of the changes; and in 22% of women, the clinical appearance of the lichen sclerosus 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 steroids are reported to result in adverse changes on the vulva, such as atrophy and striai, the long-term use of clobetasol in patients with lichen sclerosus rarely has 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. Commonly, 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.

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. 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. Another treatment is intramuscular triamcinolone. Triamcinolone (Kenalog) is injected into the thigh or gluteus muscle (1 mg/kg). No more than 80 mg should be given at a time.  This can be repeated in 6-8 weeks.

Lichen sclerosus 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.

Commonly Used Topical Corticosteroids Potency Ranking