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Nonsexual acute genital ulcerations (NAGUs), also known as Lipschütz ulcers, are vulvar ulcers occurring predominantly in adolescent females. Although the pathogenesis is unknown, NAGUs are commonly associated with systemic infections. Here, we present a female child with NAGU along with disseminated Lyme disease. We believe this is the first reported pediatric case of this phenomenon. [ABSTRACT FROM AUTHOR]

The World Health Organization defines female genital mutilation/cutting as any medically unnecessary procedure involving partial or total removal of the external female genitalia or other injury to the female genital organs. It is a violation of human rights and associated with serious complications and lifelong impact on health. This review article summarizes for the pediatric and adolescent care provider the incidence worldwide, the impact of cultural practices, appropriate screening and diagnosis, interventions, and treatment, along with legal and ethical issues. [ABSTRACT FROM AUTHOR]

46,XY partial gonadal dysgenesis (PGD) is a rare cause of disorder of sexual development (DSD) and characterized by a range of testicular dysgenesis, ambiguous genitalia, and absent/rudimentary Müllerian structures 1,2.We present the surgical management of a 21-year-old patient with 46,XY PGD,who has predominantly female external genitalia with clitoral enlargement and complete vaginal agenesis. Our patient presented with primary amenorrhea and lack of breast development. Clitoral enlargement was present with a single urethral opening and absence of an external vaginal opening (Fig.1)Laboratory evaluation revealed low testosterone concentrations (0.3 ng/ml) with elevated FSH and LH leves.Total testosterone/dihydrotestosterone and androstenedione ratios were normal. While pelvic imaging showed no uterus and vagina, bilateral streak gonadal tissue were observed within the distal part of inguinal canals.The genetic evaluation showed a 46, XY karyotype with FISH analyses positive for the SRY gene.Detailed genetic analysis performed for a 46,XY DSD panel with targeted next generation sequencing (NGS) failed to reveal any identified mutation.Our patient was raised a female and expressed no gender identity confusion. Bilateral gonadectomy with open inguinal incisions, reduction clitoroplasy and McIndoe vaginoplasty were performed during the same session.The small dysgenetic gonads in the inguinal canals were determined preoperatively.Two full-thickness skin grafts were harvested from the bilateral inguinal incision. Reduction clitoroplasty was performed by reducing the corpus cavernosum while preserving the glans clitoris and neurovascular structures3.In the presence of a wide urethral opening without a vaginal introitus, creating the appropriate vaginal opening with a perineal approach was meticulously performed.The operation was completed by placing skin grafts into the created vaginal cavity.Pathological findings was significant for seminoma in the right testicle.A vaginal mold was left in place all day for the first 3 months and only at night for an additional 3 months. She was started on estrogen replacement for pubertal induction.Significant reduction in size of the glans and no vaginal stenosis were observed 3 months after the procedure (Fig.2). 46,XY DSD patients, especially those with genital ambiguity, are complex cases in terms of both clinical and surgical management. 46,XY PGD should be considered as a rare cause in these cases where other possible causes have been excluded. Achieving a good cosmetic result without functional loss requires a well-planned and meticulous surgical intervention. Creating a neovaginal space requires great care, especially in cases where there is no vaginal introitus. [ABSTRACT FROM AUTHOR]

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, affecting 1 in 5000 females, is a mullerian anomaly characterized by uterovaginal malformation with the diagnosis typically following investigation of primary amenorrhea (1). Vater Syndrome (VACTERL) is a broad term describing a constellation of congenital abnormalities with an estimated incidence of 1 in 10,000 to 40,000 births (2). Here we present a case of MRKH with co-existing VACTERL to highlight commonalities in presentation with the goal to improve knowledge, early identification, and opportunities for research. 15-year-old, Hispanic female referred to endocrine for evaluation of primary amenorrhea. Patient with past medical history of thyroid nodule, mild persistent asthma, vitamin D deficiency, speech delay and Vater Syndrome. Reproductive and thyroid hormones were found to be within normal range. Physical exam consistent with female external genitalia, normal secondary sexual characteristics, Tanner staging III breast development, Tanner V pubic hair and axillary hair present. Undetected uterus and right ovary on pelvic sonogram, result obscured by bowel gas. Abdominal/ Pelvic MRI confirmed absent uterus, visualization of bilateral ovaries with 2.9cm cyst, and short segment of distal vagina. Clinical findings indicative of MRKH warranting prompt referral to adolescent gynecology. The co-occurrence of MRKH and Vater syndrome is extremely rare with limited case studies (3). Specific subtypes of MRKH may mirror or overlap with VACTERL and can lead to partial/misdiagnosis and/or under detection of MRKH. Standardization of genetic testing and pelvic sonogram/MRI should be considered with suspicion of either disorder in utero, infancy and adolescence. Further research on this topic is indicated in addition to provider education, as there is a shortage of pediatric gynecologists who specialize in congenital malformations. Prompt identification can guide treatment options for the developing child and may be instrumental in preserving sexual health and embryonic function. [ABSTRACT FROM AUTHOR]

While traditionally monochorionic twins are understood to be monozygotic, researchers have identified atypical forms of twinning resulting in monochorionic dizygotic (MCDZ) twins. As a result, intrauterine twin-twin transfusion pathways can result in blood chimerism with one neonate subsequently having two blood cell lines. A 3-year-old female presented to the subspecialty combined clinic consisting of endocrinology, genetics, and pediatric and adolescent gynecology (PAG) physicians secondary to abnormal placental genetic testing noting monochorionic diamniotic (MCDA) twinning without placental fusion and subsequent concern for chimerism. The patient was born as a MCDA twin but was found to be non-identical to her twin brother. Her physical exam at the multidisciplinary clinic was grossly unremarkable with a normal thyroid without nodularity, normal appearing external female genitalia, no clitoromegaly, visible urethral and vaginal openings, no labial fusion, no palpable masses within the labia or inguinal canals, and tanner stage 1 for breast and pubic hair development. A transabdominal pelvic ultrasound showed a 3.5cm prepubertal uterus with suspected ovaries visualized bilaterally. Her labs were remarkable for a total testosterone < 2.5, dihydrotestosterone < 1.0, negative inhibin, LH < 0.09, FSH 3.17, and estradiol < 1. Interestingly, her karyotype identified normal male karyotype (46, XY) in 30 of 31 metaphase cells examined; in one cell there was a normal female karyotype (46, XX) resulting in chi, XY[30]/46,XX[1]. Microarray comparative genomic hybridization identified one copy gain involving 45 probes in the distal region of the long (q) arm of chromosome 4 at bands 4q35.1q35.2. Fluorescence in-situ hybridization (FISH) results suggested the patient has two cell lines: the major cell line (99%) was normal male (XY) signal patterns and the minor line (1%) had normal female (XX) signal patterns. The suspected clinical diagnosis is a confined hematopoietic chimerism in a MCDA dizygotic twin secondary to twin-twin transfusion physiology with her non-identical twin brother. This case demonstrates a confined chimerism persisting into childhood with lab work (inhibin and testosterone) ruling out alternative single gene developmental sex disorders. Interestingly, this patient was phenotypically completely female with a presumable normal uterus despite her chimeric chromosome results. If the patient desires to assess whether her mosaicism is confined to the bone marrow, a skin biopsy FISH for X,Y would be indicated. Blood sampling from the brother would provide insight to verify chimerism secondary to twin-twin transfusion. [ABSTRACT FROM AUTHOR]

46XY pure gonadal dysgenesis (Swyer syndrome) is a rare disorder of sexual development. Patients have a 46XY karyotype, though phenotypically they appear female with normal external genitalia and vagina. Although patients exhibit normal Müllerian structures (uterus, fallopian tubes, and vagina), they possess a pair of bilateral undifferentiated gonad streaks. Delayed puberty and primary amenorrhea are the common presentations. There is an increased risk of developing tumors in the gonads and therefore a bilateral gonadectomy is recommended. A 16-year-old girl who presented with primary amenorrhea was diagnosed with Swyer syndrome. She underwent prophylactic bilateral gonadectomy and salpingectomies. She was discovered to have no gonadal malignancy, conversely dysgerminoma solely within the fallopian tube. Both bilateral salpingectomies and bilateral gonadectomies should be recommended as the operation of choice in patients with Swyer Syndrome. [ABSTRACT FROM AUTHOR]

Androgen receptor (AR) mutations, which cause androgen insensitivity syndrome, impair the actions of 5α-dihydrotestosterone and testosterone, resulting in abnormal sexual development. In most cases, genetic aberrations of the AR are caused by substitutions, but also can result from mutations in splicing regions and deletions in the AR gene. Our present report describes a female patient with 46,XY karyotype and normal female external genitalia. A novel de novo c.1669_1670insC insertion in the AR gene caused androgen insensitivity syndrome. This report provides a detailed clinical characterization of the patient and a possible pathogenic mechanism leading to androgen insensitivity syndrome and should be particularly useful in genetic counseling. [ABSTRACT FROM AUTHOR]

A crucial part of patient management is effective delivery of unfavorable information, in a manner that will not affect future engagement with them, their coping strategies and their compliance to treatment. We present a case report detailing how to communicate a life-changing diagnosis to patients. Two siblings, aged 20 and 18, presented with primary amenorrhea to our Pediatric and Adolescent Gynecology Unit. They were female phenotype with 46XY karyotype. The elder sister had bilateral herniorrhaphy at 14 years with Breast Tanner 4, Pubic Hair Tanner 2, and normal female external genitalia. The younger one had left herniorrhaphy at 12 years, with Breast Tanner 3, Pubic Hair Tanner 1 and normal female external genitalia. They were accompanied by their mother unaware of their diagnosis. Our senior most clinician reviewed their laboratory and imaging results and led the diagnosis disclosure of Complete Androgen Insensitivity Syndrome to the siblings and their mother. Her opening statement was, "We are all different." She then explained the five factors that contribute to gender and then described their diagnosis, what it meant and how it came about. Further, she discussed the cultural and religious implication and informed them of support groups available. Despite the comprehensive discussion and the opportunity to ask questions at any point, the patients seemed perturbed by the news and remained silent. The clinic visit was finalized by answering the concerns raised by their mother and scheduling a follow-up appointment to allow for further conversation and debriefing. Bad news is information that has potential to negatively alter a patients view of their future. Both the process of breaking the news and the content have a profound effect on the patient therefore the delivery has to be well thought out. It is a critical advanced communication skill for clinicians. One can opt to use a non-structured approach, like we did, or employ predefined communication strategies on breaking bad news. One example of these frameworks is the SPIKES protocol: an acronym that details consideration of the Setting, Perception, Invitation, Knowledge, Empathy and Summary. Such protocols have been cited to improve clinicians confidence in breaking bad news and improve patient involvement in the decision-making process. However, effective communication must allow for deviations from the sequence to allow patient expression of emotions, rather than a strict focus on the process of breaking bad news. In addition, breaking bad news should not be seen as a singular event. As seen in our case, the revelation of the diagnosis can 'numb' the patients and hence require follow-up sessions to debrief and chart a care plan. [ABSTRACT FROM AUTHOR]

Abstract Background Fibroepithelial stromal polyp (FESP) is a rare benign mass, usually presenting at the vagina. Herein we report, to our knowledge, the first case of contrast-enhanced magnetic resonance imaging (MRI) with diffusion-weighted images of a giant vulvar FESP, and compare the MRI features with the histopathologic results. Case A 14-year-old girl presented with a huge mass as large as 20 cm that originated from the labium majora. Preoperative MRI showed a polypoid mass consisting of a central stalk and surrounding stroma. Different signal intensities on MRI were correlated with various histopathologic features. The mass was cured by complete excision without remnant lesion. Summary and Conclusion Contrast-enhanced MRI with diffusion-weighted images can help us differentiate FESP from other vulvovaginal stromal tumors with a complete evaluation of the external and internal structures and the depth of invasion. [ABSTRACT FROM AUTHOR]

Ciliated cyst of the vulva is a variety of ciliated cutaneous cyst, which itself is a rare presentation and a very few cases has been reported. This case report shows the presentation of this cyst in a 12-year-old girl with early onset of puberty. The histopathology supports estrogen receptor and progesterone receptor positivity of the cyst lining, which supports the theory of Müllerian heterotopy during embryogenesis. This is the most popular theory on the development of this cyst. Its an uncommon presentation in children but a curable benign cyst. [ABSTRACT FROM AUTHOR]

Background Granular cell tumors (GCTs) are rare soft tissue lesions that can involve the female genital tract, including the vulva. Although malignant tumors are aggressive and uncommon, benign and atypical lesions still have associated risks, including recurrence and multisite development. Cases Four cases of pediatric vulvar GCT are presented, including 1 atypical and 3 benign tumors. The atypical lesion occurred in a 3-year-old girl, to our knowledge, the youngest reported patient with GCT of the vulva. Follow-up information is available for 3 patients, ranging 0-12 years. Summary and Conclusion GCTs are an important differential diagnosis in patients with vulvar lesions. In the pediatric population, the tumors' risk of recurrence and multisite development require gynecologists to advocate for complete resection, careful follow-up, and thorough examination for additional tumor sites. [ABSTRACT FROM AUTHOR]

Infants with differences of sex development (DSD) are often diagnosed at birth due to ambiguous genitalia or incongruence between karyotype and external genitalia. In some cases, the degree of virilization varies significantly within the same condition. This can lead to confusion and distress with the gender assignment process, especially in the immediate postnatal period. This is a retrospective cohort study of patients presenting for care from 2007-2021 with a diagnosis of 45X/46XY or 46XX/46XY mixed gonadal dysgenesis (MGD) or 46XY complete gonadal dysgenesis (CGD), identified by ICD-9 and ICD-10 codes. These are two common DSD conditions with confusion around sex assignment at birth. Descriptive statistics were used to summarize the data. This study was IRB approved. Sixty-two patients met inclusion criteria and were included in the study, including 42 patients with MGD, 20 CGD. Of the patients with MGD, 48% were assigned male at birth and 52% female. No patients reported a gender identity incongruent with sex assigned at birth at the time of most recent clinic visit. The average age at most recent visit was 13 years (range 0-32 years). Of the male-assigned patients, 80% underwent hypospadias repair, the remainder had typical male external genitalia. The majority (16/22) of female-assigned patients had typical female external genitalia, 4 underwent feminizing genitoplasty, and 2 had clitoromegaly with no immediate plans for surgery. All surgeries were performed at age ≤5 years, except for one feminizing vaginoplasty which was performed at age 11 years. The two patients with clitoromegaly were age 1 and 12 years at the time of the last visit, respectively. Of the patients with CGD, 15% were assigned male at birth and 85% female. Similarly, no patients reported gender dysphoria. The average age at most recent visit was 15.7 years (range 0-30 years). Of the male patients, 2/3 underwent hypospadias repair and 1/3 had micropenis. Seventy-six percent of female patients had typical female external genitalia, 2 underwent feminizing genitoplasty, and 2 had clitoromegaly with no immediate plans for surgery. All surgeries were performed within two years of age. The two patients with clitoromegaly were age 2 and 30 years at time of last visit, respectively. Gender identity of all patients remained congruent throughout this study. Patients with increased virilization were likely to be assigned male sex and identify with male gender. Those with less virilization were more likely to identify as female. Parents can be counseled that with careful and thoughtful decision making in the newborn period, the potential for inappropriate sex assignment is low in children with MGD and CGD. [ABSTRACT FROM AUTHOR]

The case report describes the case of 16-year-old girl who came to the pediatric-gynecologist's for primary amenorrhea. She was of normal height 155 cm, with female external genitalia, secondary sexual characteristics according to Tanner M2,A3,Ph 4. In ultrasound we detected uterus of infantile type, without endometrium, ovaries not visualized. In functional cytology was only basal and para-basal cells. As part of the differential diagnosis of primary amenorrhea were taken a hormonal profile, genetic examination. In the laboratory was diagnosed hypergonadotropic hypogonadism, in genetic examination mosaic karyotype 45 × 0/46XY. Concluded as mixed gonadal dysgenesis syndrome. Induction of puberty started by the gynecologist with estrogens. Due to the karyotype, bilateral laparoscopic adnexectomy is indicated as prevention of tumor transformation of dysgenetic gonads, especially of gonadoblastoma. Intraoperatively striated gonads were removed. Continued induction of puberty with estrogens, added progestogens after sufficient growth. After hormonal stimulation, the patient menstruates. Plan to continue combined hormonal contraception. In the future, when planning a pregnancy, it is necessary to use methods of assisted reproduction with a donated oocyt. Mixed gonadal dysgenesis is one of the possible causes of primary amenorrhoea with an incidence of 1:15,000 live births. Its diagnosis is possible only with the help of genetic testing. [ABSTRACT FROM AUTHOR]

Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers. We describe a case of vulvar leiomyoma in a 14-year-old girl who presented a 10 × 10 cm solid tumor in the right hemivulva without other complaints. Differential diagnosis includes Bartholin cysts, abscesses, fibromas, and other solid lesions. Although rare, vaginal leiomyoma must be remembered and included as a differential diagnosis for solid lesions in the vagina. [ABSTRACT FROM AUTHOR]

Labiaplasty (defined as the surgical reduction of the labia minora) is the most common procedure under the umbrella of female genital cosmetic surgery with the prevalence increasing over the past 10-15 years. However, the concept of labial hypertrophy holds an arbitrary definition, with no research into labial size undertaken within the pediatric and adolescent populations. Under the tenets of medical ethics there is acceptance of the need to avoid harm and so, for reasons to be outlined, performance of labiaplasty in children and adolescents should be avoided. This Mini-Review does not extend to pathological conditions that affect the labia minora. [ABSTRACT FROM AUTHOR]

Background Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions might be the herald symptom of LCH and might mimic other cutaneous lesions. Prognosis varies widely on the basis of the extent and spread of disease. Case An 8-year-old girl with a 4-month history of vulvar lesions resistant to topical steroids was referred by her pediatrician. Vulvar biopsy was diagnostic for LCH. Imaging studies revealed a left hip lesion consistent with LCH. The patient was subsequently treated for multisystem LCH with vinblastine and prednisone. Summary and Conclusion Although rare, LCH might be diagnosed by gynecologic providers and should be included in the differential diagnosis of genital lesions. We recommend having a low threshold for performing biopsy of vulvar lesions. [ABSTRACT FROM AUTHOR]

Background Fibroepithelial polyps (FEPs) are benign tumors, of possibly hormone-dependent nature, found in the vulvovaginal region of women of reproductive age. Case A 15-year-old adolescent girl, receiving hormonal contraceptive therapy, who presented with multiple vulvar masses with histopathology consistent with FEP. Summary and Conclusion The spectrum of the morphology of FEPs might make their diagnosis challenging. We describe a rare presentation of vulvar FEPs in an adolescent girl receiving hormonal contraceptive therapy. [ABSTRACT FROM AUTHOR]

Abstract: Study Objective: To better understand the precise role of sensory corpuscles within the female external genitalia. Design: After IRB approval, waste tissue samples were obtained from 10 normal girls (aged 2–9 years) who underwent surgery for labial fusion. Immunocytochemistry against protein gene product 9.5 (PGP 9.5), neuron-specific enolase (NSE), vasoactive intestinal peptide (VIP), 5-hydroxytryptamine transporter (5HTT), 5-hydroxytryptamine receptor 1A (5HT1A), Neuronal Peptide Y (NPY), neuronal nitric oxide synthase (nNOS), and estrogen receptors (ER) α and β was performed. Results: Pacinian-like corpuscles were identified in epithelium of labia minora of prepubertal girls. A central structure composed of an axon surrounded by a central core, outer core, external capsule, surrounded by encapsulated stroma, and a subsidiary innervation in the outer aspect of the corpuscle stroma stained for PGP 9.5 in the outer core and layers of the external capsule, NSE positive cells in layers of the outer core, 5HTT in stroma of the corpuscle and cells located in layers of the outer core, 5HT1A in cells of outer core, NPY in stroma of the corpuscle, and nNOS in external core and external capsule of the central structure. ERα was present in stroma, external core, and external capsule, and ERβ in stroma of the corpuscle with subsidiary innervation in the stroma positive to PGP 9.5, VIP, and NPY. Conclusion: PGP 9.5, NSE, ERα, nNOS, and 5HTT immunoreaction detected in the outer core and external capsule could indicate these areas may play an important role in the functional aspects of the Pacinian-like corpuscle. [Copyright &y& Elsevier]

Abstract: Introduction: Surgical and histologic sources of information give little reference to innervation, vascular, and epithelial details of the labia minora. Little is known about areas of nerve density, epithelial qualities, and vascular compartments of the labial minora that contribute to sexual arousal and orgasm. Surgical procedure development and counsel about surgical risks related to labioplasty and surgical flaps created from labial tissue may be based on inadequate information. Methods: Labial samples from 10 normal girls (aged 2–9 years) who underwent surgery for labial fusion utilized waste tissue strips for immunohistochemical identification of S-100 and neuronal nitric oxide synthase (nNOS) in the labia minora. Results: Vascular and lymphatic plexus lie within the reticular dermis, which contains a dense mesh of nerve fibers with a higher concentration of nerve fiber at the level of the subepithelial plexus. Dense innervations are located at the epidermis, extending along the basal and spinous layers of the epithelium of labia minora. Nerve bundles in the papillary dermis are associated with sebaceous and eccrine glands and nerve terminals located throughout the epithelium. The introital epithelium of the labia minora is highly innervated with widespread and intense staining, detected in the introital border of the labia minora versus the external one. The dermis appeared to display S-100 and nNOS immunolabelling. S-100 was also immunopositive in the epidermis. Conclusion: Labia minora is highly innervated along its entire edge. Related vascular compartment tissue involved in engorgement during sexual arousal makes this tissue important for sexual response. Labioplasty risks removal of tissue with an important contribution to sensory sexual arousal. Movement of labial tissue during genitoplasty may have different sensory outcomes dependent on which labial surface is used. [ABSTRACT FROM AUTHOR]

Abstract: Background: Isolated absence of the clitoris is a rare entity with medical and sexual implications for patients. Case: As part of an examination for alleged sexual abuse, a 13-year-old female was noted to have an apparent absence of the clitoris. Congenital absence of the clitoris was felt to be the most likely diagnosis. The differential diagnosis and medical implications are reviewed. Conclusion: Isolated absence of the clitoris is a rare entity. When faced with the finding, a detailed medical evaluation should be performed to clarify the nature of the finding, rule out other potential anomalies and help in the counseling of the patient. [Copyright &y& Elsevier]

For complex cloacal reconstructions, urogenital separation is best for a short urethra (<2cm) or if there is concern that a total urethra mobilization may move the bladder neck out of the urogenital diaphragm. For such cases, the single perineal orifice is left to be the meatus. After separation from the vagina(s), the common channel is used with the urethra to construct a neourethra. This orifice, however, is anatomically high relative to the typical external female genitalia, leading to poor cosmesis and potential discomfort with intermittent urethral catheterizations. This case report describes a method of urethral meatoplasty during a cloacal repair with urogenital separation in which the distal common channel is used to create an anatomically correct placement of the urethral orifice relative to the vestibule. A 4-month girl with a cloaca was referred to the interdisciplinary surgical team of colorectal, gynecologic, and urologic surgeons. A cloacagram showed a 2.16 cm common channel, 1.5 cm urethra, and longitudinal vaginal septum, thus a urogenital separation was planned. After maintaining the common channel for the urethra, the vaginas were separated from the urinary tract. The vaginas and rectum were mobilized via laparotomy. Following septum resection, the vagina was tubularized and elongated. The labia minora, which were fused to the common channel, were separated. The posterior wall of the very distal common channel was opened 3mm and sutured to the anterior vaginal wall horizontally. Thus, the urethral meatus was more posterior to its original location, and the introitus lay next to the urethral opening's lateral walls. With this meatal modification, the urethral and vaginal orifices are natural anatomic locations within a mucosal vestibule. There is a paucity of literature regarding cosmetic outcomes following cloacal repair; focus has been on a catheterizable, visible urethra. For a cloaca requiring a urogenital separation, leaving the common channel orifice in its original location leaves an anteriorly mislocated urethral meatus, closer in proximity to the clitoris. Our proposed technique for meatoplasty and introitoplasty yields urethral and vaginal orifices within a mucosal vestibule, improves cosmesis, and may improve comfort for future intermittent catheterizations as the urethral meatus is separate from the clitoris. [ABSTRACT FROM AUTHOR]

Background Hyperhidrosis (excessive sweating) affects 1%-3% of the population. Primary focal hyperhidrosis most commonly affects the axilla, palms, and soles. There are few case reports of hyperhidrosis of the genital region, typically described as inguinal hyperhidrosis or Hexsel's hyperhidrosis. Case A 17-year-old girl presented with 3 years of copious, clear “vaginal” discharge causing significant emotional distress. After extensive gynecologic and urologic workup was negative, further review of her history was notable for excessive axillary sweating. Inguinal hyperhidrosis was suspected and she was treated with topical aluminum chloride hexahydrate with complete resolution of her symptoms. Summary and Conclusions Inguinal hyperhidrosis, compared with other sites, is not widely described in the literature. Awareness of inguinal hyperhidrosis is important because it causes significant social embarrassment but is a treatable condition. [ABSTRACT FROM AUTHOR]

Abstract: Study Objective: To help determine the long-term course of girls diagnosed with lichen sclerosus before puberty. Design: Retrospective chart review and follow-up interview. Setting: Washington University pediatric gynecology and dermatology clinics. Participants: Premenarchal girls diagnosed with lichen sclerosus from 1989-2010. Interventions: Telephone interview. Main Outcome Measures: Resolution of symptoms, specifically pain and/or pruritus. Results: Follow-up was available for 36 premenarchal girls. The mean age at lichen sclerosus (LS) diagnosis was 7 years (range: 3-14 years). The mean duration of follow-up was 5.3 years (range: 2 months-15 years). Treatment with topical steroids (primarily 0.05% clobetasol propionate ointment) resulted in improvement in symptoms within an average of 14 weeks (range: 2 weeks-2 years) in 33 girls. Eighty-three percent of patients (n = 30) experienced remission after initial treatment. Sixteen patients reported relapses requiring an average of 3.1 years of intermittent maintenance therapy. The mean length of remission to date was 3.6 years (range 1 months-10 years). 72% of patients reported remission at the time of the phone interview. Of note, 7 out of 9 patients in our study who continue to report symptoms are still premenarchal. One postmenarchal patient was asymptomatic but had signs of LS on physical exam. Conclusion: The prognosis and long term course of LS diagnosed prior to puberty is unclear. Although remission may occur prior to menarche in some cases, once children reach menarche with active disease, complete remission may be less likely. Treatment duration of LS in our study had a wide range, but 3 months appears to be adequate for most patients to obtain remission. [Copyright &y& Elsevier]

: BackgroundLichen 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 reportA 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.: ConclusionPimecrolimus 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. [Copyright &y& Elsevier]

Background Mastocytosis is a rare benign disorder characterized by the finding of mast cells in the skin and other organs, occurring in adult as well as pediatric age groups. The cutaneous form is the most common presentation in childhood and is rarely located on the vulva. Case A 9-year-old prepubertal girl presented with bilateral vulvar nodules found incidentally by her pediatrician. Biopsy and histopathologic review confirmed mastocytosis. Her lesions significantly diminished over the following year. Summary and Conclusion Mastocytosis should be on the differential for lesions on the vulva in children, in addition to the more common disorders such as condyloma acuminata and molluscum contagiosum. It is a benign condition in children, and conservative, symptomatic management is recommended. [ABSTRACT FROM AUTHOR]

Background River tree rope swings are popular for recreation along inland lakes and rivers, but not without hazard. In a comprehensive review of injuries related to rope swings, genital injuries accounted for nearly 3 percent. Case We describe significant genital injuries, with laceration and avulsion of the labia and a large vulvar hematoma in 2 young women as a consequence of rope swing use. Summary and Conclusions Visitors to inland waterways need to be cognizant of the hazards of rope swings and health care professionals should consider this mechanism of injury when confronted with vulvar trauma. [ABSTRACT FROM AUTHOR]

A rare case of a giant vulvar lipoma that developed in an adolescent is presented. A review of the world literature of 15 cases that occurred prior to adulthood confirmed that they tend to occur on the right side of the vulva. [ABSTRACT FROM AUTHOR]

Abstract: Background: Granular cell tumor is an uncommon benign neoplasm with a predisposition for upper aerodigestive tract, skin and soft tissue involvement. Malignant and atypical granular cell tumors account for less than 2% of the lesions and in the pediatric population they are extremely rare and atypia has not been previously reported. Case: We present a case of a rapidly growing granular cell tumor of the vulva of a 12-year-old girl exhibiting atypical histology. The lesion demonstrated prominent Ki-67 proliferation index (up to 20%), localized areas of spindling of tumor cells, scattered apoptotic bodies and p53 overexpression. Conclusion: The current histologic diagnostic criteria of atypical granular cell tumors are evaluated while physician awareness and the need for follow-up of patients for potential recurrences of this rare entity are emphasized. [Copyright &y& Elsevier]

Abstract: Background: Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are aggressive malignancies characterized by translocation involving the EWS gene. Primary extraskeletal ES/PNET rarely affects the female genital tract. Case: We report the case of a 20-year-old female, who presented with a rapidly growing painful mass arising from the left labium majus for six months. Multiple lesions in bones and nodules in lungs were considered metastasis. The diagnosis was established by immunohistochemical stain and reverse transcriptase polymerase chain reaction analysis. The patient ceased treatment and died of respiratory failure one month after discharge. Conclusion: Vulvar ES/PNET mainly affects adolescent females. ES/PNET should be considered in the differential diagnosis of tumors involving the lower gynecologic tract in adolescent female. [Copyright &y& Elsevier]

Abstract: Background: Large vulvar hematomas in the pediatric population resulting from straddle-type injuries often require surgical incision and drainage. We report an innovative use of a Word catheter to facilitate recovery following hematoma evacuation. Case: A 14-year-old female presented with a 6-cm right labial hematoma and was initially treated with ice packs and analgesia. The hematoma did not resolve over the following weeks, and she subsequently underwent incision, drainage, and placement of a Word catheter. It was removed painlessly on postoperative day 8, and follow-up revealed no signs of reaccumulation, infection, or scar tissue formation. Summary and Conclusion: Placement of a Word catheter following evacuation of vulvar hematomas is a comfortable alternative to traditional drains to prevent reaccumulation and facilitate recovery. [Copyright &y& Elsevier]

Abstract: Background: Ectopic breast tissues can be found along the embryonic mammary ridges and can occur in the vulva. While ectopic breast tissue is not uncommon, functional breast with overlying nipple located within the vulva is exceedingly rare. Case: A 17-year-old with undiagnosed hypothyroidism presents with vulvar mass draining milky white fluid. A small lesion with appearance similar to a skin tag is noted and milky fluid expressed. Biopsy and excision of this mass confirmed the presence of a functional supernumerary nipple. Summary and Conclusions: This appears to be the first reported case of a supernumerary nipple with symptomatic lactation in a non-pregnant adolescent. Supernumerary nipple should be considered in the differential diagnosis of a vulvar mass. Ectopic breast tissue in the vulva can undergo malignant transformation, therefore excision of this tissue is generally recommended. [Copyright &y& Elsevier]