Results. Eighteen of 24 questionnaires were completed and returned. The average age of onset of LS was 4.2 years, but average age at diagnosis was 5.2 years; only 1 patient was diagnosed correctly by her primary care physician. Itching was the most common symptom (78%). Severe constipation was reported in 67% of patients, and 89% had at least 1 gastrointestinal complaint (bleeding with bowel movements, fissuring, soiling, fecal impaction, or constipation).Conclusions. L ichen sclerosus (LS) is an uncommon inflammatory disorder that can occur at any age, but has a predilection for the vulvar skin of prepubertal girls and postmenopausal women (Fig 1). It is an eruption, the typical appearance of which is an ivory-white, sharply demarcated figure-8 pattern encircling the vagina and anus. It begins as papules containing a central depression and often coalesces into plaques. The skin surface may appear finely wrinkled or have purpuric spots, fissures, and erosions. Although less common, lesions may also present extragenitally. An often-unappreciated sign of LS, extremely distressing to these children, is severe constipation, which is unrelieved by standard treatment measures.During the past 2.5 years we have seen 24 girls with LS in the pediatric dermatology clinic at the Medical College of Wisconsin (Milwaukee, WI). A significant number of their parents have expressed considerable anger and frustration due to the failure of their physicians to recognize the association between severe constipation and LS, resulting in a long delay in diagnosis. The English-language literature, including major textbooks of medicine, pediatrics, obstetrics and gynecology, and dermatology, fail to mention constipation as a feature of LS. By reporting our experience with these patients, we hope to bring this association to the attention of primary care physicians. We asked about timing of complaints in relation to actual diagnosis of LS. The questionnaire also focused on specific signs and symptoms, diagnosis, and specialists consulted for the problem.
RESULTSOf the 24 surveys mailed, 18 (75%) were completed and returned.The patients ranged in age from 4 to 17 years (mean: 8 years). The average age at onset of symptoms was 4.2 years, but the average age at diagnosis was 5.2 years. There was a time lag of ϳ1 year from onset of signs and symptoms until diagnosis of LS, the longest interval being 3 years. Only 1 patient was diagnosed by a primary care physician (a family practitioner). Two children were diagnosed in a hospital pediatric gastrointestinal (GI) clinic, and a pediatric dermatologist diagnosed all others. Efforts to obtain a diagnosis also prompted families to make multiple visits to pediatricians, gynecologists, and pediatric general and vascular surgeons. Table 1 lists the signs and symptoms of LS in our patients and indicates those that were most bothersome. Itching was the most common complaint, but GI-related complaints were also quite prevalent. Twelve of 18 patients had constipation, and 9 had perianal fissuring. Eleven o...