History of Present Illness

A 14-year-old female presents to your office complaining of a one-year history of vaginal discomfort and irritation. She reports her labia have been enlarging to the point where she now has discomfort wearing swimsuits or certain clothes. She feels a rubbing sensation when she walks, and sometimes her labia stick to her underwear. She usually removes all hair from her vulva by shaving. She does not have any rash, redness, or vaginal discharge. Her mother has seen the area and thinks it does not look normal.

Privately, the patient tells you that she has never been sexually active.

She denies any history of medical problems or any surgical history. She reports her menarche was at age 12 years, and her periods are regular. She is in eighth grade and is active in volleyball.

Physical Examination

General appearance

Well-developed adolescent in no distress

Respiratory

nonlabored respirations

Abdomen

soft, non-tender, non-distended

Breast

Tanner V, symmetric, no lumps

Pelvic

Normal external genital anatomy without rashes or lesions

• 
  

  Tanner IV pubic hair

  
  
• 
  

  Normal intact annular hymen

  
  
• 
  

  No vaginal discharge or erythema

  
  
• 
  

  Mildly asymmetric labia minora, right greater than left

  
  
• 
  

  On stretch, the right labium measures 5 cm from midline and the left labium measures 4 cm from midline

  
  
• 
  

  Wet prep: negative for clue cells, hyphae, or motile trichomonads

How Would You Manage the Patient?

This patient has mild labial hypertrophy, which is a normal variation and is not pathologic. In addition, she has no evidence of yeast vaginitis or other condition that may cause vulvovaginal discomfort.

The initial management involved reassuring the patient and her mother of the wide variety of normal vulvar anatomy. She was directed to resources online and in print to view images of normal vulvar anatomic variations. For her discomfort and vulvar irritation, she was advised to make some behavioral modifications, such as wearing looser clothing and supportive underwear. In addition, she was advised to avoid irritants in her personal hygiene regimen, such as harsh or scented soaps, scented pads or panty liners, detergents, and certain hair removal methods that can cause vulvar irritation. She was also advised to use a topical, over-the-counter barrier ointment such as petroleum jelly or zinc oxide to reduce chaffing.

At her follow-up visit, her symptoms of irritation had resolved, and she had no additional concerns.

Labial Hypertrophy in Adolescents

Increasing numbers of girls and women are seeking care due to concerns about genital appearance. Possible reasons for the increased attention to this area include increased access to images of genital anatomy online, trends in removal of pubic hair with resultant increased awareness of variation in genital anatomy, and awareness of the availability of labial cosmetic surgery [1, 2].

Usual patient concerns include pain or chronic irritation of the vulva, discomfort wearing certain clothing, concern that there is a “bulge” appearance that can be seen through clothing, embarrassment when changing clothes in front of peers, or self-consciousness with sexual activity. Parents may bring children to be seen for this condition when the adolescent’s anatomy appears different from her parent’s expectation of normal.

Labial hypertrophy is a poorly defined and subjective condition. When measured on stretch from the midline, labial size is known to vary greatly, from approximately 1 to 5 cm [3]. There is no consensus on what labial size constitutes hypertrophy or “abnormal” labia; however, most studies on labiaplasty have defined their own minimum measurements that diagnose labial hypertrophy, with variation between 3 cm and 5 cm [4].

In adolescents with symptoms of external vaginal irritation, the differential diagnosis includes vulvovaginitis due to yeast, bacterial vaginosis, or trichomoniasis, as well as vulvar dermatoses such as lichen sclerosus. In patients who complain solely of anatomic concerns or “extra skin” in the vulvar area, the differential includes anatomic abnormalities such as clitoromegaly, overgrowth of mucosal tissues such as hymenal tags or hymenal septa, and vulvar cysts such as Bartholin’s gland cysts.

The initial workup for vulvar irritation in the setting of concern for labial hypertrophy should include a thorough history, with specific attention to symptoms of irritation, itching, and effect of symptoms on daily activities. Physical examination should include a thorough external genital exam with careful attention to all vulvar and vaginal anatomy and the relationship between vulvar structures. An evaluation for yeast, bacterial vaginosis, and trichomoniasis should be performed with a wet prep in patients who have symptoms of discharge or irritation.

Once the diagnosis has been confirmed and underlying infectious or dermatologic conditions have been ruled out, the management of patients presenting with labial hypertrophy should be tailored to the presenting complaint. Patients who present with symptoms of discomfort can be counseled to wear clothing that is less form-fitting, wear supportive undergarments, make adaptations during exercise to increase comfort, improve personal hygiene to decrease irritation, or use topical emollients if there is chronic external irritation. The current trends of pubic hair removal and resultant exposure and irritation of labia can be discussed, and patients who shave or otherwise remove their pubic hair should be discouraged from continuing this practice.

If the primary concern is about labial appearance, the first step should be patient reassurance about a wide variety of normal vulvar appearances. Patients can be referred to a number of resources such as websites that have a variety of photographs of normal vulvar anatomy for comparison [11, 12]. Patients can also be reassured that increases in labial size during the pubertal years are common. Informative handouts and displays can help patients view variations in labial anatomy [5–7]. If there is concern for excessive preoccupation with a minor physical abnormality, the patient should be screened for body dysmorphic disorder using a standardized questionnaire or referred to a mental health professional [8, 13].

Labial Surgery in Adolescents

In recent years, increasing awareness about labiaplasty in the general population has led to an increase in requests for this procedure. In adolescent patients, surgery is performed only in extreme cases, as in the patient depicted in Figure 63.1, and should be performed by a practitioner with experience in labial surgery. Labial surgery exclusively for cosmetic purposes should not be performed in an adolescent population [1, 9].

Figure 63.1 An adolescent patient with severe right labial hypertrophy (over 10 cm on stretch) that caused significant quality of life issues (preventing her from wearing jeans or riding a bike) despite conservative measures, including use of topical emollients.

There are multiple labial reduction techniques described in the literature. Two common variations are of linear resection (either straight or curvilinear) along the edge of the labia minora and wedge resection or de-epithelialization. The linear resection techniques will result in a linear scar along the exposed edge of the labia minora. Wedge resection techniques have the benefit of preserving the natural edge of the labia minora [9, 10] but may have a higher rate of complications. Figure 63.2 demonstrates the immediate postoperative appearance of a patient who underwent a linear resection of the right labia minora.

Figure 63.2 The immediate postoperative appearance of the patient in Figure 63.1 after linear resection. Note the entire labia minora was not removed but was resected to be similar to the left side.

Typical surgical risks include scarring, dyspareunia, altered sensation during sexual intercourse, dissatisfaction with appearance, and regrowth of labia minora, especially if the procedure is performed at a young age. Typical complications would include bleeding, wound breakdown, and infection. There are studies reporting an overall low rate of complications and high patient satisfaction; however, these studies are often retrospective surveys of complications and satisfaction rates and lack long-term follow-up data [9].

Key Teaching Points

• 
  

  There is a wide variation in normal external genital anatomy.

  
  
• 
  

  Labial hypertrophy is poorly defined but typically considered when the labia minor measure greater than 5 cm on stretch.

  
  
• 
  

  In adolescents, the first approach to concerns about labial hypertrophy should be reassurance about anatomic variations and conservative measures aimed at symptom management.

  
  
• 
  

  Body dysmorphic disorder should be considered in individuals presenting with concerns about genital appearance and be screened for using available screening questionnaires.

  
  
• 
  

  Labial surgery in adolescents is reserved for extreme, medically indicated cases and should not be performed for cosmetic purposes alone.

History of Present Illness

A 16-year-old old nulligravid female presents to your office for acne, excessive hair growth, and irregular menses. She reports menarche at age 13, with initial regular bleeding every 28 days that would last for 5 days. For the last 12 months, her menses have become irregular, with intervals between 28 and 90 days. Her menses have also become heavier and last 7–9 days. Her last menstrual period was six weeks ago. She denies any recent or prior sexual activity.

Over the past year, she reports an increase in hair growth on her face, lower back, and around her umbilicus, and she has to shave her legs more often. She denies deepening of the voice, but has noticed her acne is worsening. She currently has facial and upper back acne that is not responding to oral antibiotics or over-the-counter topical therapy. She reports a weight gain of 15 pounds in the past year, although she denies significant changes in her diet or activity level.

Her family history is significant for hypertension on her father’s side and diet-controlled diabetes in her mother. She denies any other significant cardiovascular disease or history of deep vein thrombosis.

Physical Examination

General appearance

Well-appearing, no acute distress, overweight

Temperature

98.5°F (36.9°C)

Blood pressure

110/65 mmHg

Pulse

64 beats/minute

Respiration

20 breaths/minute

Weight

185 pounds

Height

5 ft 3 in

BMI

32.8 kg/m²

Cardiovascular

Regular rate and rhythm without murmurs, rubs, or gallops

Lungs

Clear to auscultation bilaterally, no wheezing

Abdomen

Soft, non-tender, non-distended. Bowel sounds are present

Pelvic

Deferred

Skin

Moderate inflammatory acne lesions in different stages of healing on the face, neck, upper back, and upper chest, with signs of scarring around the mandible. Acanthosis nigricans of the back of the neck and the folds of the arms. Increased amount of hair in the upper lip and chin, as well as the sideburns. Shaved hair follicles around the areola bilaterally, suprapubic and upper abdomen, as well as on her lower back. Both arms and legs are shaved.

Urine pregnancy test

Negative

FSH

4.8 mIU/mL (4.7–21.5 mIU/mL)

Testosterone

78 ng/dL (< 55 ng/dL)

17-hydroxy progesterone

80 ng/dL (<200 ng/dL)

DHEAS

250 mcg/dL (37–307 mcg/dL)

TSH

1.55 uIU/mL (0.51–4.94 uIU/mL)

Prolactin

19 ng/mL (2.8–29.2 ng/mL)

How Would You Manage This Patient?

This patient underwent laboratory and imaging examinations to rule out specific medical conditions that could cause irregular menses and androgen excess. Given the lack of specific findings and the mild elevation of testosterone, the diagnosis of polycystic ovarian syndrome (PCOS) can be presumed. Although she has acanthosis nigricans, suspicious for hyperinsulinemia, insulin level testing was not performed, as it would not significantly change initial management. She was, however, tested for metabolic syndrome, including screening for diabetes with a two-hour glucose tolerance test and lipid profile screening, which were both negative.

The patient was prescribed combined oral contraceptive pills to regulate her menses and decrease the circulating free testosterone that causes her acne and hirsutism. She was advised on nonmedical interventions for hair removal, including depilation (above the skin) and epilation (beneath the skin) methods to help remove the already present hair. In addition to the hormonal management, the patient was counseled regarding the importance of healthy lifestyle, including calorie intake restriction and modification, as well as increase in physical activity. A referral to a dietician and the physical therapy group at the local gym was made.

At her six-month follow-up, she reports regular, light menses without cramping. She is following a low carbohydrate diet and walking an extra 30 minutes a few times a week, and she has lost 5 pounds. She reports her acne is much improved, as it is now only on her face and responds to topical therapy. She has not seen an increase in hair growth, and after undergoing frequent waxing, she has seen a better cosmetic result on her facial and abdominal hair.

Polycystic Ovarian Syndrome in Adolescents

Polycystic ovarian syndrome (PCOS) affects 6–15 percent of reproductive-age women and is responsible for more than 70 percent of cases of hyperandrogenism [1, 2]. Although its origin is not clear, evidence suggests PCOS is caused by an interaction of inherited traits, environmental factors, alterations in steroids and metabolism, and adaptations of excess energy supply [3, 4]. Although the criteria in adults have been well established to include polycystic ovarian morphology (PCOM), anovulation, and hyperandrogenism [5], the diagnosis of the condition in adolescents is controversial. The findings associated in adults are common normal findings in adolescents and are not necessarily diagnostic of PCOS. In order to establish better guidelines in the diagnosis and management of patients with suspected PCOS, multiple medical societies and its representatives created consensus guidelines in an attempt to unify specialists caring for these patients [6].

The evaluation of patients with suspected PCOS should include a complete personal and family history as well as appropriate laboratory testing to rule out other conditions, including nonclassical adrenal hyperplasia (see Box 64.1). Androgen excess in adolescents generally presents with hirsutism, moderate inflammatory acne, and menstrual irregularities. Mild facial hirsutism and acne are normally present in adolescents in their postpubertal stage and are not considered clinical evidence of hyperandrogenism. On the other hand, moderate or severe acne and hirsutism require laboratory evaluation before initiating therapy. Available assays to evaluate androgen levels have not been consistently reliable to measure levels in children and women. Each available measuring system has established normative levels, and abnormal values would depend on the assay utilized. Measurement of total and/or free testosterone seems the most widely available test for androgen assessment and can be used to determine hyperandrogenism in adolescents. Elevated androgens, without clinical findings, should not be considered hyperandrogenism in adolescents [6].

Irregular menses are most often the result of anovulatory cycles, which are common within the first two years after menarche and are not necessarily caused by PCOS or androgen excess. Patients who have persistent cycle lengths of less than 20 days or greater than 45 days after the first two years post-menarche warrant an evaluation. Similarly, patients with recurrent cycles greater than 90 days apart, or who started menses after age 15 (or 2–3 years after thelarche), should be assessed for PCOS. Patients presenting with oligomenorrhea or amenorrhea may benefit from follicle-stimulating hormone (FSH) testing to evaluate for premature ovarian insufficiency.

Unlike adults, the presence of multiple follicles in the ovary is not considered a diagnostic criterion for PCOS and can be a normal finding in adolescents. Data regarding ovarian morphology come from ultrasounds performed via the transvaginal approach, which is not commonly performed in non-sexually active adolescents. Obesity in these patients makes the transabdominal images not reliable, and could provide inaccurate information. For these reasons, ultrasound is not included as diagnostic criteria in this population during the evaluation for PCOS.

Patients suspected of having PCOS may also have clinical (acanthosis nigricans) and laboratory evidence of hyperinsulinemia. This condition is common in adolescents and should not play a role in the diagnosis of PCOS; however, detection of impaired glucose tolerance may be an opportunity to address the long-term effects of the condition, including diabetes and metabolic syndrome. This will allow initiating evaluations for these comorbidities, and placing interventions to improve the metabolic disturbances, including lifestyle modifications with diet and exercise. To address these concerns, screening for glucose intolerance and lipid abnormalities is indicated. A fasting and two-hour 75 g load glucose tolerance test and a fasting lipid profile will allow for early identification and management of the comorbidities.

Management of menstrual irregularity and the symptoms related to androgen excess is generally the main concern for these patients [8, 9]. Cyclic administration of combined estrogen-progesterone oral contraceptive pills (COC) is considered the first-line management for most adolescents [10]. By suppressing ovarian function, androgen production decreases. In addition, circulating estrogen increases the production of steroid hormone binding globulin (SHBG), thus decreasing free testosterone and its end organ effects. The cyclic shedding of the endometrium prevents the long-term effects of chronic anovulation, including endometrial hyperplasia and cancer, and provides reliable scheduled menses. Those with contraindications to estrogen may use progesterone-only products, but the reduction in circulating androgen is less.

Although COC has a significant impact in new hair growth and future outbreaks of acne, hair that is already present may still require mechanical and chemical treatment. For refractory cases, other medications, such as spironolactone, may be utilized as an adjuvant to further decrease hirsutism. Adjuvant use of chemical and mechanical hair removal methods is also recommended.

Along with hormonal management, lifestyle modifications, including weight loss, are beneficial to manage PCOS and the long-term effects of the condition. Menstrual irregularities respond well to weight loss through diet and exercise, and should also be utilized as first-line treatment, particularly in patients with obesity. A reduction of as little as 6.5 percent of total body weight has been shown to return menstrual regularity [11]. The use of insulin sensitizing agents, such as metformin, has been used in adolescents to manage the metabolic disturbance present in adolescents, and is best reserved for those with concomitant glucose impairment [8]. Contraceptive needs for the adolescent should also play a role in the treatment selection, as many of these patients may wrongly perceive they are unable to conceive given their anovulatory cycles.

Key Teaching Points

1. 
   

   1. There is significant overlap between the normal changes associated with puberty and the findings associated with PCOS, making its diagnosis difficult in adolescents.

   
   
2. 
   

   2. PCOS is a diagnosis of exclusion, and the proper evaluation for the other conditions presenting in a similar fashion should be performed.

   
   
3. 
   

   3. When selecting management options, addressing both the immediate needs (menstrual regulation and decrease in acne and hair growth) as well as the potential long-term effects of the adolescent (metabolic syndrome, infertility) are warranted.

   
   
4. 
   

   4. The use of combined oral contraceptive pills is considered first-line therapy for the treatment of adolescents with PCOS.

   
   
5. 
   

   5. Lifestyle modifications (diet and exercise) should also be included in the initial approach to PCOS.

   
   
6. 
   

   6. Obesity, hyperinsulinemia, and insulin resistance are not part of the diagnostic criteria for adolescents with suspected PCOS, but they should be screened for and addressed in the context of long-term disease prevention.

History of Present Illness

An eight-year-old girl presents with her mother with complaints of persistent vulvar itching. The girl has been seen by her pediatrician several times for this complaint without improvement of her symptoms. The girl states that she just cannot stop scratching because her vulva is so intensely itchy. Her mother reports that the patient has been scratching and rubbing her vulva more frequently over the last few months, and the discomfort seems to be worse at night. The mother and daughter deny any odor or discharge on the girl’s underwear. The girl has been treated empirically for yeast infections twice and for bacterial infection once, but her mother states that the pediatrician was unable to find a source of the itching and that all the swabs and cultures have been negative. None of the treatments have helped, and the mother thought that the cream to treat a yeast infection made the girl’s vulva burn and feel more painful.

The girl has had no medical problems. Prior surgery includes tonsillectomy at age five years. She has no known allergies and does not take any medications other than a daily vitamin. She was delivered vaginally at full term and has met all her developmental and physical milestones. She is doing well in third grade. She lives at home with her mother, father, and younger brother, and there is no concern for sexual abuse.

Physical Examination

General appearance

Well-appearing, well-nourished child

Temperature

37.0°C

Pulse

85 beats/min

Respiratory rate

22 breaths/min

BP

90/64 mmHg

Weight

64 pounds

Height

49 inches

BMI

18.7 kg/m²

Abdomen

Soft, non-tender, non-distended

Breasts

Tanner 1

External genitalia

Prepubertal, Tanner Stage 1. Hypopigmentation from clitoral hood to perineum and spreading around perianal region with sharp borders. Tissue appears paper thin (see Figure 65.1).

Labia

Normal appearing in size and shape. Mild ecchymosis and superficial erosions noted on lower aspect of labia minora/majora.

Urethral meatus

Normal size and location, no lesions, no prolapse.

Urethra

No masses, no tenderness.

Bladder

No masses, no tenderness.

Vagina

Visualized, no abnormal discharge. Hymen appreciated with normal prepubertal appearance, appropriate hygiene.

Anus/perineum

Thin, hypopigmented skin.

Vaginal bacterial culture

Normal flora

Fungal culture

Negative

How Would You Manage This Patient?

The patient has lichen sclerosus (LS), the diagnosis of which is based on clinical symptoms and physical examination. When LS is diagnosed in a child, the physician must first describe the disease to the patient and her parent and explain how to treat it. The goal is to preserve normal anatomy and help minimize pruritic symptoms. Vulvar biopsy is not indicated at this time and should only be done if LS is refractory to treatment or the diagnosis is in doubt.

This patient was treated with a high-potency corticosteroid ointment, clobetasol 0.05 percent, applied in a thin layer to the affected area twice daily. In addition to this pharmacological treatment, good vulvar care was recommended, including minimizing the use of any harsh soaps, body wash, harsh detergents, and/or dryer sheets. Further, oral diphenhydramine (25 mg every 6 hours) was recommended to relieve her pruritus symptoms. She was instructed to follow-up four weeks later, at which time her symptoms and clinical exam findings were resolved. The topical corticosteroids were tapered to a lower-potency steroid for two weeks, followed by 1 percent hydrocortisone ointment, to use daily. The patient was instructed to follow up every 6–12 months for intermittent monitoring of disease and to call if symptoms returned. This patient continued to have an excellent response to the steroid ointment taper and remained symptom-free six months later.

Childhood Lichen Sclerosus

LS is a benign inflammatory dermatologic skin condition primarily affecting the anogenital region in children. It is chronic, progressive, and characterized by marked inflammation, epithelial thinning, and skin changes. Although genital LS primarily occurs in postmenopausal women, about 10–15 percent of cases are in prepubertal children [1]. This bimodal age distribution appears to correlate with hypoestrogenic physiologic states. The etiology of LS is unclear, but it is accepted as an autoimmune disorder. LS has been associated with other autoimmune diseases such as alopecia, vitiligo, type 1 diabetes, and autoimmune thyroiditis [2].

Children with LS typically present with symptoms of intense pruritus, vulvar irritation or pain, dysuria, bleeding from excoriations from scratching, and painful bowel movements. The itching and irritation can be so intense that the patients cannot stop scratching. The irritation and discomfort also tend to be worse at night. Oftentimes, these patients are misdiagnosed as having other conditions with similar symptoms, such as urinary tract infections, pinworms, and/or yeast infections.

The diagnosis of LS in the pediatric population is based on history and physical exam. The differential diagnosis for LS includes lichen planus, vitiligo, psoriasis, eczema, and contact dermatitis. Classic vulvar LS appears as hypopigmented (white or pink) plaques with distinct borders. The tissue can become paper thin and is sometimes described as being similar to parchment paper or cigarette paper. The affected region most frequently surrounds the labia majora and minora but can also extend past the perineum and surround the anus. This pattern is typically referred to as a key-hole or figure-of-eight pattern. Figure 65.1 demonstrates the typical appearance of LS in a pediatric patient.

Figure 65.1 Lichen sclerosus in prepubertal girl.

Courtesy of Diane Merritt

Scratching or rubbing may induce vulvar purpura with ecchymoses or fissures. If left untreated, scarring can occur, resulting in labial fusion, buried clitoris, and narrowing of the vaginal introitus [3]. The vagina is not affected by LS. In children, biopsy should be conducted only if the condition is not improved with treatment or if the diagnosis is unclear [4]. Of note, LS is sometimes mistakenly diagnosed as sexual abuse, as they can share similar physical exam findings. As these two diagnoses can coincide, it is important to take a thorough history, and if there are any concerns for abuse, the child should be evaluated by a health-care provider trained in child abuse evaluation and management.

Treatment goals for LS are primarily medical and include suppression of symptoms and resolution of the signs of the disease, including atrophy, hyperkeratosis, fissuring, and ecchymosis. The most commonly used medication for treatment of LS is a topical super-potency corticosteroid ointment, such as clobetasol propionate 0.05 percent or betamethasone valerate 0.1 percent [5]. Few studies have evaluated the efficacy of topical steroids for LS in the pediatric population; however, a 2011 Cochrane Review by Chi et al. included a small number of pediatric patients [6]. Although the authors were unable to make specific recommendations for children, the evidence indicated that topical steroids were more effective than placebo, and clinical experience and subsequent studies appear to confirm that topical corticosteroids should be considered first-line therapy in these patients. To improve absorption and decrease risk of contact dermatitis, ointments rather than creams are recommended for the application of these steroids. The ointment should be applied sparingly to the affected area twice daily.

After a month of use, the patients typically become asymptomatic, as occurred in the above patient, and can begin to taper off the clobetasol ointment. This can be done in several ways, and there is no clear consensus on the optimal regimen. Some advocate decreasing to daily application of the clobetasol ointment for two weeks, then twice a week for two weeks. The patient is then instructed to either use a once weekly maintenance dose or to resume treatment with any flares. Other alternatives to a clobetasol taper include switching to a mid-potency steroid for two weeks, followed by a daily maintenance dose of hydrocortisone 1 percent ointment, with methylprednisolone aceponate 0.1 percent ointment used on the weekends [7]. It is thought by some experts that continued low- or mid-potency topical corticosteroid steroid use should be continued until puberty to prevent recurrence, progression, and scarring.

Topical calcineurin inhibitor therapy with tacrolimus or pimecrolimus has been reported as a successful adjunctive treatment for LS, particularly for maintenance therapy; however, it has not been shown to be effective as initial therapy. In addition, an FDA black box warning indicated a possible relationship between its long-term use and skin cancer and lymphoma.

Surgical management is not indicated for first-line treatment of LS. However, surgery may be an option for patients with long-standing untreated or refractory disease who have developed significant scarring that causes pain, urinary tract retention, or dyspareunia. As this is a rare procedure to treat the end-stage tissue damage of LS, it is best to refer to a specialist with experience in treating this complication. It is advised that the underlying LS be well-controlled prior to moving forward with surgical management, and the patient should be educated on the need to continue topical steroid ointment therapy after surgery to prevent recurrence of disease.

LS is a chronic dermatologic condition of the vulva that can recur even after appropriate treatment. The rates of recurrence for LS in children have been reported to range from almost half to nearly 80 percent after treatment [8]. Even during symptom-free periods of time, changes in the appearance of the vulva can be noted. In adults, there is a small increased risk of developing squamous cell carcinoma with LS. It is unknown if there is a similar risk for young girls diagnosed with LS [9]. Close follow-up is therefore recommended for patients with vulvar LS. In children with LS, long-term follow-up with a gynecologist is recommended every 6–12 months to monitor for symptoms and evaluate for recurrence.

Key Teaching Points

1. 
   

   1. LS can be diagnosed in the pediatric population based on symptoms and appearance on physical exam, with vulvar biopsy reserved only for those cases refractory to treatment or atypical appearing lesions.

   
   
2. 
   

   2. Initial therapy for LS should begin with a high-potency topical steroid ointment, such as clobetasol 0.05 percent, and continued until symptoms are resolved. Steroids may then be tapered, and maintenance therapy with a low-potency topical steroid may be considered to prevent recurrence.

   
   
3. 
   

   3. Surgical management is rarely indicated and should only be performed in patients with severe architectural changes that result in significant pain or recurrent infection.

   
   
4. 
   

   4. Follow-up for children with LS should occur every 6–12 months with a gynecologist to monitor symptoms and evaluate for recurrence.

History of Present Illness

A four-year-old girl is referred from her pediatrician for evaluation of vaginal discharge. The discharge has been occurring on and off for several months. She also complains of itching and occasional burning pain associated with the discharge. She denies vaginal bleeding, abdominal pain, dysuria, or any other associated symptoms, and she has no recent history of upper respiratory infection. She is accompanied by her mother who confirms this history and adds that the discharge has a foul odor. The patient has been treated with oral fluconazole as well as oral metronidazole, but there has been no improvement in her symptoms.

She lives at home with her younger brother, mother, and father. The mother and patient both deny any abuse history or concerns for victimization. She attends preschool during the day. She is healthy and takes no medications.