Lichen sclerosus et atrophicus (LSA) is a chronic skin disease characterized by white, flat papules with an erythematous halo and black, hard follicular plugs. In advanced cases, the papules tend to coalesce (blend) into large, white patches of thin, itchy skin.
Lesions often occur on the torso and, almost invariably, in the anogenital (relating to both the anal and the genital) regions. In the latter case, the disease is called kraurosis vulvae.
LSA has the appearance in the skin of pale patches that become thin, shiny, and parchment-like. On close inspection, the patches contain clusters of minute white lesions that blend together. Grooves, cracks, and ecchymoses (the passage of blood from ruptured blood vessels) may appear. Distribution is on the neck, under the breast, in body folds, and in the perianal and vulvar areas. Patients may complain of pruritis (itching). Atrophy and shrinkage of the skin of the vagina and vulva may cause painful sexual intercourse.
The condition is progressive and the atrophy does not regress. If leukoplakia is present, then squamous cell carcinoma must be suspected also.
Women are affected most often. The disorder affects women between the ages of 40 and 60, though cases involving younger women and men have been reported. In the rare cases of LSA in men, it is generally the foreskin that is affected, making retraction of the foreskin impossible.
The cause of LSA is not known. It may be due to an autoimmune process or an injury. A genetic predisposition does not exist.
The initial signs of vulvar dystrophy are likely to be one or more patches of dry, thickened skin, often accompanied by persistent itching. The patch may be red and swollen at first, but later becomes opaque white. As the condition progresses, the abnormal, white skin area expands to cover all or most of the vulva. Thickened, dry skin may form tiny cracks that burn and itch, or the skin may become glossy, and very thin.
Loss of normal vulvar elasticity may narrow the vaginal opening. The fat layer just under the vulvar skin may be lost, so that the outer labia are flat and shrunken. Biopsy is necessary to distinguish between vulvar dystrophy and more ominous precancerous or cancerous vulvar abnormalities. All of these can be similar in appearance.
Treatment consists of topical applications of antipruritics, corticosteroids, and testosterone to the affected areas. Surgical removal of affected skin layers may be of benefit in severe cases.
Promising results have been reported from treatment with oral retinoids in patients with severe LSA of the vulva. Acitretin was shown to be effective in treating severe LSA of the vulva and in reducing pruritis, burning, atrophy, and secondary features such as erosions, ulcers, edema, and lichenification.
Other trials have been reported on a very potent topical steroid (clobetasol propionate 0.05 percent cream.) Thirteen patients in one study showed a marked clinical improvement.