Vulvar lichen sclerosus refers to a chronic, inflammatory, progressive, dermatosis of unknown etiology. The typical lesions are atrophic, porcelain-white, coalescent, papules and plaques often associated with areas of hyperkeratosis, fissuring and ecchymosis. Intense pruritus is associated. If the treatment is not initiated early in the course of the disease, irreversible changes in the vulvar architecture may occur, impairing the patient’s quality of life. Beside the functional and aesthetic damage, vulvar lichen sclerosus also associates the risk of developing vulvar intraepithelial neoplasia, a precursor of invasive squamous cell cancer.

We present the case of a 54 years old patient with a pruritic, anogenital rash, consisting of coalescent, porcelain-white, atrophic plaques associated with changes in the vulvar architecture, that had been evolving for about 2 years. The clinical suspicion of vulvar lichen sclerosus was confirmed by histologic examination.
The patient received initial treatment with clobetasol propionate 0,05% ointment, followed by tacrolimus 0,1% ointment, with relatively rapid resolution of the pruritus.

At 6 months follow-up, she had developed an erythematous, purpuric plaque with small ulcerations on the vaginal introitus, raising suspicion of vulvar intraepithelial neoplasia, which was not confirmed by the histologic examination.
Currently, the patient continues the topical treatment with tacrolimus 0,1% ointment and she is being followedup on a monthly basis.
We intend to review the existing data on the management of vulvar lichen sclerosus and also the risk of associated malignancy.