Girls With Lichen Sclerosus May Present With Constipation  CME News Author: Laurie Barclay, MD CME Author: Désirée Lie, MD, MSEdDisclosures To earn CME credit, read the news brief along with the CME information that follows and answer the post test questions. Release Date: February 7, 2005; Valid for credit through February 7, 2006 Feb. 7, 2005 ? Lichen sclerosus (LS) may present with constipation and/or other gastrointestinal tract complaints in girls, according to the results of a study published in the February issue of Pediatrics. The investigators urge primary care physicians to consider the diagnosis in girls presenting with these symptoms. 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, write Mandi L. Maronn, MD, and Nancy B. Esterly, MD, from the Medical College of Wisconsin in Milwaukee. An often-unappreciated sign of LS, extremely distressing to these children, is severe constipation, which is unrelieved by standard treatment measures. The investigators sent a focused questionnaire to parents of 24 girls with anogenital LS seen in the pediatric dermatology clinic at the Children's Hospital of Wisconsin between January 2001 and May 2004, and 18 (75%) of the 24 questionnaires were completed and returned. Although the average age of onset of LS was 4.2 years, average age at diagnosis was 5.2 years, and only one girl was diagnosed correctly by her primary care physician. Itching was the most common symptom (78%), but 67% had severe constipation, and 89% had at least one gastrointestinal tract complaint, such as bleeding with bowel movements, fissuring, soiling, fecal impaction, or constipation. Childhood anogenital LS often presents with recalcitrant constipation or some other gastrointestinal complaint, the authors write. Primary care physicians need to consider the diagnosis of LS and perform a thorough examination by looking for anogenital lesions when a female pediatric patient presents with unexplained constipation or other severe gastrointestinal complaints. The authors cite several reports claiming that tacrolimus, an immunomodulator recently developed for patients with atopic eczema, is effective in LS. Should tacrolimus prove to be as effective as a class 1 superpotent topical steroid, it will offer a tremendous advantage because of the absence of side effects, the authors conclude. However, additional trials of tacrolimus in LS patients and careful surveillance of their course are needed to determine if this drug will cure, maintain remission, or only be effective for short-term use. The authors report no conflict of interest. Pediatrics. 2005;115:e230-e232 Learning _object_ives for This Educational Activity Upon completion of this activity, participants will be able to: Identify presenting symptoms of LS in children. Describe the most bothersome symptoms reported by girls presenting to a clinic with LS. Clinical Context Childhood LS represents approximately 15% of total cases and is seen in a 10:1 female to male ratio. Prevalence was estimated in one study to be 1 in 900 with a mean age at symptom onset of 5.0 years but a mean age at diagnosis of 6.7 years. Anogenital LS may present with a variety of symptoms, ranging from itching, purpura, and soreness locally to dysuria, constipation, pain on defecation, soiling, perianal fissures, and bleeding to no symptoms. Vulvar itching and soreness are the most commonly reported symptoms. Frequency of constipation has been variably reported at a low of 12% to a high of 82% according to the authors. The typical lesion is ivory white, demarcated with a figure-eight pattern circling the vagina and anus, beginning as a papule and coalescing into plaques. The skin may be wrinkled or show fissures and erosions. LS is sometimes mistaken for child abuse because of its appearance and associated symptoms. The prognosis of LS is unknown, but some improvement is expected by puberty. Current standard of care consists of high potency topical steroids, including betamethasone, clobetasol, and halobetasol. Tacrolimus, an immunomodulator recently developed for use in atopic eczema, is being considered as an alternative agent. This is a retrospective survey study of 24 young girls diagnosed as having LS who presented to a university dermatology clinic. The current study was conducted to document the most common presenting symptoms of the girls. Study Highlights A survey was developed for parents of girls who presented to a university dermatology clinic with an eventual diagnosis of LS. The questionnaire focused on timing of complaints related to the LS diagnosis, specific signs and symptoms, and specialists consulted on the problem. Of 24 surveys mailed, 18 (75%) were returned. Patients ranged in age from 4 to 17 years (mean, 8 years). Average age of symptom onset was 4.2 years, and average age at diagnosis was 5.2 years, with an average time lag of 1 year from symptom onset to diagnosis. The longest time lag was 3 years. Only one patient was diagnosed by a primary physician (a family physician). Two were diagnosed in a pediatric gastrointestinal tract clinic, and the remainder was diagnosed by a pediatric dermatologist. Multiple visits were made by parents to pediatricians, gynecologists, and general and vascular surgeons before the diagnosis was made. Symptoms were analyzed as being present and being most bothersome. Most bothersome symptoms were identified 22 times (4 more than the actual number of patients, suggesting that some patients identified more than one symptom as being most bothersome ). Itching was the most common complaint (14 patients, with 6 identifying it as the most bothersome symptom). 10 patients had soreness, and two of these identified it as the most bothersome symptom. 12 (67%) of 18 patients had constipation, and 6 identified it as the most bothersome symptom. 11 had pain on defecation and bleeding with bowel movements, and 9 had perianal fissuring. 3 in each of these groups identified the symptoms as being the most bothersome. A total of 8 patients identified itching and soreness as being most bothersome symptoms. A total of 13 patients cited gastrointestinal tract symptoms as being the most bothersome. 9 (50%) of 18 patients were misdiagnosed as having been sexually abused by a caregiver, 7 were misdiagnosed as having a yeast infection, and 3 were thought to have a primary process causing constipation. Patients were treated by physicians with numerous topical agents including antifungal creams, antibiotics, estrogen, moisturizers, and corticosteroids of various potencies. Most patients had relief from potent topical corticosteroids after the correct diagnosis was made. Pearls for Practice Anogenital LS may present with a variety of symptoms, ranging from vulvar itching, purpura, and soreness to dysuria, constipation, pain on defecation, soiling, perianal fissures, and bleeding to no symptoms. Gastrointestinal tract symptoms are most often cited as bothersome, and itching and soreness are the next most bothersome symptom for girls with anogenital LS. Carol in IL Mom to  seven kids,  twin grandson's and Christine, 4 DS Unless the Lord builds the house, they labor in vain. Psalm 127   My problem is not how I look, it's how you see me