LICHEN SCLEROSUS (ET ATROPHICUS)

By Gary M. White, MD

Lichen sclerosus


Lichen sclerosus (LS) is an inflammatory dermatosis producing white areas on the skin and genitalia. It commonly occurs on the vulva in females of all ages.

Etiology

Clinical

White atrophic areas are seen in the vulva in both women and girls. Purpura is not uncommon. In adults the purpura may just be thought of as a bruise and the clinician may not notice the LS. In children, the purpura may be confused with abuse. LS may occur extragenitally and occasionally may be widespread. Any woman with LS of the skin should have the vulva and perianal area examined as well. Sometimes, there is an overlap with morphea.

Lesions on the penis are called balanitis xerotica obliterans. Extragenital lesions in men may rarely occur. LS may occur around urostomies, again suggesting that urine on the skin plays an etiological role [BJD 2013:168;643-6].

Children

Diagnosis

Squamous Cell Carcinoma in Lichen Sclerosus

Treatment

A potent topical steroid ointment (e.g., clobetasol) BID is quite effective at reducing any itch and improving the physical appearance of the skin. It is appropriate for all females including woman and young girls. The patient should be treated for 3-6 weeks until symptomatically and clinically improved. Then, taper. Various maintenance approaches may then be taken. Perhaps the simplest is to have the patient use clobetasol only once or twice a week. Alternatively, one can: 1) Switch to tacrolimus ointment alternating with clobetasol x 1 month and then just tacrolimus maintenance. 2) Stay with clobetasol daily x 1 week/month and tacrolimus the rest of the time. 3) Step down to triamcinolone. Or 4) Go to tacrolimus directly.

The benefit of long-term followup should be emphasized to the patient. In a 15-year, prospective study of 507 women [JAMA Derm 2015;151;1061], treatment compliance resulted in zero malignancies whereas partial-compliance resulted in a rate of 4.7% biopsy-proven SCC or vulvar intraepithelial neoplasias. Scarring and progression of adhesions was also reduced with full compliance.

In some cases, atrophy can be at least partially reversed with daily therapy over 3-6 months.

In resistant cases, the patient can soak the vulva in warm water x 15 minutes before application of the topical steroid to increase absorption.

With regard to clobetasol and potential atrophy, alert patient (or parents) what to watch for. Keep the steroid off the thighs. Stria are more likely in obese patients.

Low-dose UVA-1 phototherapy has been reported effective for patients with extragenital LS. In one series, 10 patients underwent 4 phototherapy sessions per week for 10 weeks. The dose was 20 J/cm2 resulting in a cumulative dose of 800 J/cm2.

Additional Pictures

Classic hour-glass distribution in a young child. Courtesy Michael O. Murphy, MD
Lichen sclerosus with the classic hour-glass distribution

Before and after treatment with topical clobetasol ointment nightly.
Lichen sclerosus Lichen sclerosus

Typical location.
Lichen sclerosus Lichen sclerosus

The upper back is a common extragenital location.
Lichen sclerosus Lichen sclerosus

Perineal area as well as inner wrists.
Lichen sclerosus Lichen sclerosus

Note the "crinkled surface."
Lichen sclerosus Lichen sclerosus

70ish woman with perianal LS.
Perianal lichen sclerosus Perianal lichen sclerosus

Lichen sclerosus and morphea overlaps.
Lichen sclerosus Lichen sclerosus

Lichen sclerosus in a very unusual location causing alopecia in a young man.
Lichen sclerosus in the scalp, an unusual location

Purpura is not uncommon.
Lichen sclerosus with purpura

LS in an elderly woman.
Lichen sclerosus Lichen sclerosus

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