WHISTLER, B.C. -- Approximately 10%-15% of all cases of lichen sclerosus occur in children, with girls outnumbering boys 10:1, Dr. Sheryll Vanderhooft said at a clinical dermatology seminar sponsored by Medicis.
The clinical features of lichen sclerosus include hypopigmentation, atrophy, telangiectasias, erosions and fissures, purpura, and scarring. Symptoms include pruritus, vaginal discharge, and pain on urination or defecation severe enough to lead to chronic constipation or avoidance of micturition. However, up to 9% of young female patients are asymptomatic, said Dr. Vanderhooft, director of the pediatric dermatology clinic at the University of Utah, Salt Lake City.
The etiology of pediatric lichen sclerosus is unknown, but there may be an association with autoimmunity and with the class II human leukocyte antigen DQ7, just as there is in adults.
She cited a study of children with vulvar lichen sclerosus in which DQ7 was present in 66% of the patients, compared with 31% of controls. Of the patients with DQ7, 16% were homozygous for it, compared with 5% of the controls. Only 4% of the children with lichen sclerosus had another autoimmune disease, but 56% of their parents or grandparents had other autoimmune conditions, leading the investigators to conclude that patients with early-onset lichen sclerosus are likely to have a family history of autoimmune disease, as well as a stronger than average association with DQ7 (Br. J. Dermatol. 142[3]:481-84, 2000).
Lichen sclerosus responds well to treatment with ultrapotent topical steroids, such as clobetasol ointment, which produces few adverse effects in this patient population. Dr. Vanderhooft recommended the ointment over the cream because it doesn't sting.
She described an 11-year-old patient with significant purpura and hypopigmentation due to lichen sclerosus, which resolved within 3 weeks after the patient started applying clobetasol ointment twice a day.
Good results have also been reported with topical tacrolimus, which could be an alternative to steroid therapy. Combination therapy with topical tacrolimus and pimecrolimus is another option. Treatment with testosterone or other topical hormones is not recommended.
In at least one published report, 50% of girls studied who developed lichen sclerosus as children went into remission at puberty, at an average age of 15 years. The likelihood of resolution is greater if the condition occurs before menarche, although many patients experience no change in disease activity when they start menstruating. Dr. Vanderhooft said.
Squamous cell carcinoma of the vulva has been reported in 5% of older adult women with chronic lichen sclerosus. This form of cancer is extremely rare in younger women, however, and the lifetime risk for women who develop lichen sclerosus as children is unknown. The effect of treatment on squamous cell carcinoma risk in this patient population is also unknown.
BY NORRA MACREADY
Los Angeles Bureau
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