Aplasia cutis congenita in a setting of fetus papyraceus associated with small fetal abdominal circumference and high alpha-fetoprotein and amniotic acetylcholinesterase.

Fetus papyraceus is the fetal death of one or more fetuses in a multiparous pregnancy. The surviving infants can experience extensive aplasia cutis in an H-shaped distribution over the flanks and abdomen as a consequence of the loss of their fetal sibling. We report the case of a monochorionic, diamniotic pregnancy complicated by a single fetal death at 13 weeks of gestational age. Aplasia cutis of the surviving twin was suggested in utero by three criteria: high amniotic and maternal alpha-fetoprotein, detectable acetylcholinesterase, and small abdominal circumference on prenatal ultrasound. This constellation of findings in the setting of fetus papyraceus can be an indicator of aplasia cutis in the surviving fetus.

Surgical treatment of vulvar vestibulitis syndrome: outcome assessment derived from a postoperative questionnaire.

INTRODUCTION: Vulvar vestibulitis syndrome (VVS) is the most common pathology in women with sexual pain. Surgery for VVS was first described in 1981. Despite apparently high surgical success rates, most review articles suggest that surgery should be used only "as a last resort." Risks of complications such as bleeding, scarring, and recurrence of symptoms are often used to justify these cautionary statements. However, there are little data in the peer-reviewed literature to justify this cautionary statement. AIMS: To determine patient satisfaction with vulvar vestibulectomy for VVS and the rate of complications with this procedure. METHODS: Women who underwent a complete vulvar vestibulectomy with vaginal advancement by one of three different surgeons were contacted via telephone by an independent researcher between 12 and 72 months after surgery. MAIN OUTCOME MEASURES: The primary outcome measurement of surgical success was overall patient satisfaction with surgery. Additional secondary outcome measurements included improvement in dyspareunia, changes in coital frequency, and occurrence of surgical complications. RESULTS: In total, 134 women underwent surgery in a 5-year period. An independent research assistant was able to contact 106 women, and 104 agreed to participate in the study. Mean duration since surgery was 26 months. A total of 97 women (93%) were satisfied, or very satisfied, with the outcome of their surgery. Only three patients (3%) reported persistently worse symptoms after surgery and only seven (7%) reported permanent recurrence of any symptoms after surgery. Prior to surgery, 72% of the women were completely apareunic; however, after surgery, only 11% were unable to have intercourse. DISCUSSION: In this cohort of patients, there was a high degree of satisfaction with surgery for VVS. In addition, the risks of complications with this procedure were low, and most complications were transient and the risk of recurrence after surgery was also found to be low.

Prevalence of vulvar lichen sclerosus in a general gynecology practice.

OBJECTIVE: To describe the rate of vulvar lichen sclerosus in 1 general gynecology practice. STUDY DESIGN: A database of 1,675 consecutive patients presenting in a 3-year period to a general gynecology practice was utilized to identify women with lichen sclerosus. Data included age, menopausal status, symptoms and physical examination findings. Pathology specimens were reexamined by a gynecologic pathologist to confirm the diagnosis of lichen sclerosus. RESULTS: Of the 1,675 patients, 28 (1.7%) had biopsy-proven vulvar lichen sclerosus. Nine patients been diagnosed previously, and 19 were new cases. The mean age at diagnosis was 52.6 years (SD +/- 15.9) versus 37.1 years (SD +/- 16.4) for those without lichen sclerosus (p < 0.001). Fifteen of the 28 patients (54%) were post-menopausal at the time of diagnosis. Of the 19 women with newly diagnosed lichen sclerosus, 8 (42%) were symptomatic. Of the 11 asymptomatic women, 7 (64%) had scarring of the clitoral prepuce or resorption of the labia minora. CONCLUSION: The rate of vulvar lichen sclerosus in 1 general gynecology private practice is approximately 1.7%. Clinicians must maintain a high index of suspicion to make the diagnosis, as at least one third of patients may be asymptomatic.

Pimecrolimus for the treatment of vulvar lichen sclerosus: a report of 4 cases.

BACKGROUND: Lichen sclerosus is a chronic cutaneous disorder with a predilection for the vulva. The etiology is unknown. Superpotent topical corticosteroids are the most effective treatment. Dermal atrophy, however, is a well-known complication associated with changes of lichen sclerosus superpotent topical corticosteroids. In addition, some women do not respond adequately to topical steroids. Therefore, a treatment regimen that does not rely on corticosteroids may be beneficial. As lichen sclerosus is a T-lymphocyte-mediated disorder, it has been suggested that a topical macrolide immunosuppressant, such as pimecrolimus, may be a safe and effective alternative treatment for lichen sclerosus. Since pimecrolimus does not affect collagen synthesis, it does not cause thinning of the dermis. CASES: Four patients with biopsy-proven lichen sclerosus were treated with 1% pimecrolimus cream twice daily. At the end of 3 months of treatment, 3 of the 4 patients reported complete resolution of their symptoms of vulvar itching and burning. Two patients had repeat vulvar biopsies at the end of treatment that showed reversal of the histologic changes of lichen sclerosus. CONCLUSION: In this small series, pimecrolimus appeared to be a safe and effective treatment of vulvar lichen sclerosus. A randomized, controlled trial comparing pimecrolimus and clobetasol propionate should be performed to determine which is the safer and more effective medication for the long-term treatment of lichen sclerosus.

Pimecrolimus for the treatment of vulvar lichen sclerosus in a premenarchal girl.

BACKGROUND: Lichen sclerosus is a chronic cutaneous disorder with a predilection for the vulva. Lichen sclerosus affects more than one in 900 girls. Superpotent corticosteroids like clobetasol propionate are the most effective treatment for vulvar lichen sclerosus. However, recurrence after stopping steroids is very high. As repeated courses of corticosteroids are frequently needed, there are concerns about potential side effects. Therefore, a treatment regimen that does not rely on corticosteroids may be beneficial. As lichen sclerosus is a T-lymphocyte mediated disorder, it has been suggested that pimecrolimus, a topical T-lymphocyte inhibitor, may be safe and effective for the treatment of lichen sclerosus in children. CASE REPORT: A 10-year-old girl with lichen sclerosus was initially treated with clobetasol. Remission was achieved, but 3 months later she had a recurrence. Subsequent treatment with clobetasol led to a breakdown of her peri-anal skin with a superimposed infection. She was then treated with pimecrolimus and remission was achieved. She has had no recurrence of active lichen sclerosus and has less burning with pimecrolimus than with clobetasol. CONCLUSION: Pimecrolimus may be an effective treatment of vulvar lichen sclerosus. Pimecrolimus has been shown to be very safe in the pediatric population for the treatment of mild to moderate eczema, without causing dermal atrophy, tachyphylaxis, striae, rebound flares, or hypothalamic-pituitary axis suppression. As the recurrence rate of active lichen sclerosus in prepubertal girls treated with topical corticosteroids is high, and the majority of prepubertal girls with lichen sclerosus continue to have disease after menarche, a treatment regimen that does not rely on corticosteroids may be beneficial.