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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 ...
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