About a year ago, an otherwise healthy 8-year-old girl developed itching and discomfort in the perivaginal and perianal areas that persists despite use of anti-yeast creams (nystatin, clotrimazole) and OTC hydrocortisone 1%. It was diagnosed as a yeast infection, but the patient was referred to dermatology when all treatment attempts failed.

A well-defined, figure-8 pattern of white, atrophic skin surrounds the vaginal and perianal areas. Erosions can be seen on the more atrophic areas of the labia, along with faint patches of telangiectasias. The urethra is uninvolved, as is the immediate perianal skin.

A punch biopsy is obtained; it shows hyperkeratosis, epidermal atrophy, and scleroderma with a lymphocytic infiltrate.