Atopic and irritant dermatitis are the most common causes of prepubertal vulvar itch, and they often occur together [2]. These conditions manifest as fluctuating, poorly defined erythematous patches and plaques involving the vulvar area that are exacerbated by excessive washing and overuse of antifungal creams [3]. The labia majora may have scale and slight rugosity, while the labia minora can present with desquamation and redness [3]. The pruritus is often so intense that scratching is difficult to control. As such, disturbances in sleep and parental embarrassment are not uncommon.

While superinfection by Staphylococcus aureus may occur, it rarely yields positive cultures [3]. Management of atopic and irritant dermatitis focuses on the reduction of irritant exposures (commonly urine, feces, soaps, and bubble baths), as well as application of low-dose topical steroids [2]. Secondary skin infections can be treated with topical antimicrobials such as mupirocin 2 % ointment, although more severe cases may require oral antibiotics.

Vulvar psoriasis more commonly affects children than adults [2]. These lesions first present as a persistent diaper rash in babies. As children age, the lesions become pruritic, well demarcated, symmetric red plaques without scale in the vulvar and perianal regions [3]. When psoriasis is limited to the vulva, diagnosis may be difficult. In such cases, the presence of other manifestations of psoriasis such as nail pitting, history of cradle cap, and scalp/postauricular rashes can help confirm the etiology [3]. Vulvar psoriasis is managed with high-potency topical steroids or topical tacrolimus.

Lichen sclerosus (LS) affects approximately 1 in 900 girls in the United States, with 7–15 % of all cases found in the prepubertal age group [4]. This chronic, autoimmune, mucocutaneous inflammatory dermatosis of unknown etiology classically presents as a white plaque with secondary atrophy and subcutaneous hemorrhage of the vulvar and perianal skin [2] in a figure of eight configuration. Extragenital lesions include pale “confetti” spots, which can occasionally be seen on the inner wrists or elsewhere on the body; these macules are often asymptomatic. The vulvar rash is characterized by severe pruritus, which may lead to subsequent soreness, dysuria, and chronic constipation [3]. The presence of petechiae and purpura can trigger inappropriate investigations into sexual abuse in this age group [2]. LS typically first presents between the ages of 4 and 5 years old, but diagnosis often lags for at least 1 year after onset of symptoms [4]. Complications include loss of genital architecture secondary to scarring and subsequent effacement of the labia minora and clitoris [5]. While some pediatric LS resolves with puberty, other cases may silently progress into adulthood. Long-standing LS is associated with a less than 5 % chance of squamous cell carcinoma later in life [6–8]. Treatment of LS includes high-potency topical steroids and topical calcineurin inhibitors with frequent follow-up [5].

Infectious vulvovaginitis in the female prepubertal age group typically occurs secondary to group A beta-hemolytic streptococcal infection [9]. Streptococcal bacterial infections of the vulva may present in acute or subacute forms. In the more severe, acute form, patients present with erythematous, painful, edematous plaques with discharge. Alternatively, they may present with subacute inflammation, manifested as pruritic erythematous patches and plaques in the vulvar and perianal regions. Streptococcal infections are diagnosed via culture and sensitivity of vaginal swabs. Treatment includes oral antibiotics such as penicillin, amoxicillin, or cephalexin (if penicillin allergic). Although streptococcus accounts for most cases of infectious vulvovaginitis in these prepubertal girls, more rare infections include staphylococcus, haemophilus, and shigella [2]. Beyond bacterial infections, pinworm is a common etiology of vulvar and perianal pruritus and may be associated with an eczematous rash. Mebendazole is the treatment of choice for pinworm [3]. Although tinea infections can occasionally be seen in girls, vulvovaginal candidiasis does not occur before menarche in immunocompetent patients.

Most reproductive-age women experience at least one episode of vulvovaginal candidiasis (VVC) in their lifetime, and approximately half of these women endure multiple episodes [13]. Estrogen mediates the colonization of the vulvovaginal region with yeast. Because of this hormonal control, vulvovaginal candidiasis occurs almost exclusively in the reproductive years. Since estrogen levels are highest in the premenstrual period, candidal infections occur more commonly during the second half of the menstrual cycle [13]. Among women of different age groups, vulvovaginal candidal colonization has been estimated to have a prevalence of 11–22 % [14–16]. Certain conditions and medications may increase estrogen levels and lead to more frequent colonization as well as infections. These include pregnancy, antibiotic use, the use of hormonal birth control methods, hormone replacement therapy, and tamoxifen [2, 13–16]. Changes in immune regulation can also cause yeast infections, including diabetes, HIV, thyroid disease, lupus, corticosteroid use, and inheritance of a polymorphism associated with low production of mannose-binding lectin [13].

The typical presentation of VVC includes itching and burning of the vulva, with or without white discharge and vulvovaginal redness. Some patients also experience dysuria and dyspareunia. It should be noted that VVC is largely overdiagnosed in women with vulvar itch; self-diagnosis is poor, particularly in cases of recurrent vulvovaginal symptoms [13]. Diagnosis is usually made by wet prep to visualize fungal elements. However, when wet prep is indeterminate, culture or PCR may be used along with a normal vaginal pH to rule out bacterial vaginosis, atrophic vaginitis, and trichomoniasis [13]. Treatment involves topical or systemic antifungal azoles, with expected resolution of symptoms in 2–3 days [13]. Older proven therapies include topical treatment with Silvadine 1 %.

Recurrent VVC is defined as the occurrence of at least four episodes within 1 year or at least three episodes in 1 year not associated with antibiotic use [2]. These women are usually otherwise healthy and develop a hypersensitivity-like reaction to Candida. Recurrent vulvovaginal candidiasis (RVVC) may require a long-term treatment regimen of a weekly or biweekly suppressive azole antifungal [18].

Most VVC cases are associated with Candida albicans, although other species such as C. glabrata, C. tropicalis, and C. parapsilosis should be considered in relatively treatment-resistant cases. In such circumstances, fungal culture, rather than wet mount alone, is necessary for diagnosis [17].

Approximately 50 % of chronic vulvovaginal pruritus cases are due to allergic and irritant contact dermatitis [13]. These dermatoses present in a nonspecific fashion, with sudden or gradual onset of itching, burning, and erythema of the vulvovaginal region. The common offending agents in allergic and irritant dermatitis are displayed in Table 10.2.

In acute irritant contact dermatitis, redness can be localized to the area of contact (if in solid or cream form) or may be diffuse (if in water soluble or liquid form). Subacute and chronic cases are characterized by erythema, swelling, and lichenification of the affected area. While vesicles and bullae occur with irritant dermatitis on other parts of the body; they are uncommon on the vulva [13]. New popular types of cosmetic hair removal may make the skin more sensitive to a host of irritants that were previously unknown.

Allergic contact dermatitis of the vulva has a similar presentation to irritant contact dermatitis, but may be delayed or intermittent in nature. Continuous exposure to an allergen triggers an itch-scratch cycle that leads to the development of the typical thickened plaques of lichen simplex chronicus. Medical history is the key to accurate diagnosis. While patch testing is not routinely performed, it can be helpful in certain cases. Allergic contact dermatoses are treated by removing the offending agent, avoiding overwashing, and applying topical steroids.

Lichen simplex chronicus (LSC) occurs as a result of chronic rubbing and scratching of the skin. On the vulva, the skin becomes thickened, lichenified, and often hyperpigmented. Various etiologies of LSC exist; the condition can occur secondary to pruritic conditions such as LS or allergic contact dermatitis (ACD). Alternatively, it may be part of a systemic neuropathy or, in some cases, is a primary psychogenic process [13]. When LSC is suspected in the vulvar area, it is important to rule out neuropathic itch associated with sacral spinal compression: a lumbar X-ray may be helpful in identifying possible involvement of the dorsal root ganglia [19]. Other types of neuropathic itch include postherpetic neuralgia and diabetic neuropathy. To successfully treat neuropathic itch and LSC, patients must break the cycle of itching and scratching. This may be accomplished with behavior modification, anti-itch medications, anticonvulsants such as gabapentin and pregabalin [20], and topical steroids.