Paediatric vulvar skin conditions are relatively common but often cause diagnostic difficulties for clinicians, which can lead to anxiety within the children’s families. Vulvar skin conditions can be caused by various underlying aetiologies. Most are general dermatologic conditions that occur in the vulvar area, such as eczema, psoriasis, skin lesions or infections. However, other conditions such as lichen sclerosus and napkin dermatitis (‘nappy rash’) only affect the genital region.
Every affected child needs a sensitive evaluation of the complaint including an assessment of the impact of the condition on the patient and her family. Paediatric vulvar disease often influences bowel and bladder habits and may lead to behavioural problems.
General measures such as avoidance of irritants or soap substitution and regular use of emollients are helpful for all patients. Specific therapy depends upon the underlying diagnosis.
Time spent reassuring the families, reinforcing regimens and providing written documentation can all be very helpful in successful management of paediatric vulvar skin conditions.
Introduction
Vulvar skin conditions are a frequent complaint in the paediatric population and vulvovaginitis is said to be one of the most common gynaecological problems in prepubertal girls , although it is much less common in children than in adults . Such conditions are infrequently reported in the literature, leading to a reduced therapeutic evidence base.
‘Vulvitis’, ‘vaginitis’ and ‘vulvovaginitis’ are terms which are often used interchangeably to define inflammatory conditions of the lower genital tract . This article focuses upon conditions of the prepubescent vulva. It should be noted that in the absence of stimulation by estrogens, the vagina is rarely the source of problems in this group of patients.
Vulval skin conditions can cause considerable distress for children and their families leading to behavioural disturbances at school and home. Adding to this distress is that vulvovaginal conditions can be difficult to manage and are often poorly understood by non-specialist physicians.
The cause of vulvar disease in childhood can be due to common skin conditions such as eczema or psoriasis or dermatoses that are specific to the vulvar skin, such as lichen sclerosus and nappy dermatitis.
This article aims to provide a practical and when available, evidence-based approach to the aetiology, diagnosis and management of vulvar skin conditions in the prepubescent girl.
Methods
To identify relevant articles we searched the Medline, Embase and CENTRAL (Cochrane Central Register of Controlled Trials) databases combining the free text terms ‘ vulva* ’ and ‘ vulvo* ’ with the medical subject heading (MeSH) terms ‘ child ’ and ‘ skin disorder’ . Databases were searched from the time of inception until 10th April 2013. No randomised controlled trials were identified and most articles were reviews, case reports or small case series.
Methods
To identify relevant articles we searched the Medline, Embase and CENTRAL (Cochrane Central Register of Controlled Trials) databases combining the free text terms ‘ vulva* ’ and ‘ vulvo* ’ with the medical subject heading (MeSH) terms ‘ child ’ and ‘ skin disorder’ . Databases were searched from the time of inception until 10th April 2013. No randomised controlled trials were identified and most articles were reviews, case reports or small case series.
The vulva in infancy and childhood
The vulva changes as children reach menarche. It is important to appreciate these changes to i) understand the normal appearance of the vulva in childhood and ii) understand the reason why certain conditions occur more frequently in children than in adults.
During the first few weeks of life, the vulva and vagina are under the influence of maternal hormones that are received transplacentally . In the absence of oestrogenic stimulation, the vaginal mucosa becomes thin and atrophic, and lacks protective antibodies. There are no labial fat pads or pubic hair, and the vulvar skin is thin, delicate and in close proximity to the anus. The skin is generally more vulnerable to irritants such as soaps and bubble baths. This predisposes prepubertal female genitalia to bacterial infection, especially in the presence of poor hygiene. Poor hygiene can result from habits such as ineffective hand washing, wiping from back to front following bowel movements and inadequate washing of the area.
In childhood, the labia minora are relatively prominent, the hymen is thickened and the epidermal skin is thin making the vaginal introitus bright red in its normal state; these features may be mistaken as abnormalities . Occasionally, labial adhesions are present, which can mimic ambiguous genitalia . The pH is between neutral and alkaline which facilitates growth of normal skin bacteria rather than candida, explaining why streptococcal vulvitis is much more common than Candida infection before puberty.
At puberty, the size of the labia minora and mons pubis increase due to fat deposition, pubic hair develops, the clitoris becomes more prominent and the hymenal opening increases in diameter. The pH becomes more acidic in the presence of oestrogens and the vulvar tissue can subsequently be affected by a different range of conditions that are not discussed in this article.
Principles of management of vulvar skin conditions
Before discussing the specific conditions seen in the vulvar area, it is important to understand initial principles of management. These apply to vulvar dermatoses affecting both adults and children.
It is appreciated that in children and young people it might be difficult, especially in those who are very young to adhere to recommended measures. However, it is important to make the families aware of these specific recommendations and reinforce treatment plans at each visit. Extra time should be taken to address the disease process, discussing general vulvar care measures and managing expectation . Information leaflets, relevant patient-oriented websites and written instructions for how to use topical agents are helpful for parents. The goal of therapy is to correct the skin’s barrier function, which is usually disrupted and for in skin conditions, to reduce inflammation which usually lead to the child’s symptoms.
A range of environmental modifications can be undertaken to protect the skin’s barrier ( Box 1 ). Soap and other routine cleaning agents (e.g., wipes) can act as irritants and should be avoided . Urinary and faecal incontinence need to be addressed as these will exacerbate, or can be the cause of the symptoms. ‘Soap substitution’, that is, using an emollient instead of soap or shower gel, with a bland cream or ointment-based emollient like petrolatum is best for cleansing. The same agent can then be used as an emollient to soothe the area and can be applied often as necessary.
| Washing | Use soap substitute with an emollient; Bathe rather than shower/supervise showering to make sure vulva is properly washed; Do not shampoo hair in the bath; Avid bubble bath. |
| Wiping | Wipe front to back; Avoid perfumed toilet paper. |
| External irritants | Avoid antifungal creams, perfumed products including wet wipes or any other cream which causes stinging. |
| Clothes | Avoid tight clothing, for example, lycra ballet clothes or leotards; Wear only cotton underwear; Change nappies regularly. |
Inflammation reduction is achieved by topical corticosteroids. These are often ineffectively used in the vulvar area due to concerns from about side effects, particularly skin thinning. This should not be the case if used responsibly and it should be made clear to the patient and caregivers. The treatments of individual inflammatory conditions are considered in the specific sections of this article.
Vulvar-specific skin conditions
Lichen sclerosus
Lichen sclerosus occurs almost exclusively in the genital area. Two peaks of presentation occur; these are in prepubertal girls and post-menopausal women. It is not an uncommon condition in prepubertal girls with a reported prevalence of one in 900 and this figure appears to be rising . There is an increasing body of evidence to suggest that lichen sclerosus is autoimmune in nature although the exact pathogenesis remains unclear. Other autoimmune diseases are more common in adult patients with lichen sclerosus compared with controls and in a case series of 70 paediatric patients, autoimmune disease occurred in 14% of patients and a family history of autoimmunity in 64% .
Itch and soreness are the most common presenting symptoms. These may result in dysuria and constipation. Clinical findings are typically those of well-demarcated white plaques in a figure-of-eight distribution surrounding the vulva and perianal areas. Fig. 1 demonstrates lichen sclerosus in the vulval and perineal area whilst Fig. 2 demonstrates it affecting predominantly the perianal area. Skin is usually wrinkled and may show telangiectasia that bleed to cause ecchymoses ( Fig. 1 ). Fissuring and erosions are common. In lichen sclerosus, the vagina is not affected. Diagnosis in this age group is made by the typical clinical features and a diagnostic biopsy is not usually necessary since this can often be traumatic to the youngster.
Given the clinical appearance, it is not uncommon for questions of sexual abuse to be raised in affected children . Powell et al. found that in their case series of 70 girls, 77% of families were queried as to the possibility of sexual abuse . Lichen sclerosus does have a predilection for traumatised surfaces, and clearly the presence of lichen sclerosus does not rule out sexual abuse. Features that should arouse suspicion include lichen sclerosus presenting in older prepubertal girls, poor response to treatment and coexisting sexually transmitted infections .
Extra genital lesions are extremely rare but present as well-demarcated atrophic plaques on the skin elsewhere.
Lichen sclerosus does not usually remit at puberty, although symptoms may seem to settle. Silent progression may occur with recurrence of symptom activity at a later stage of life . There is an association with the development of vulvar squamous cell carcinoma in later life and has been reported in relatively young women with lichen sclerosus since childhood .
Although there are no randomised controlled trials to support the use of specific therapies in this age group, case series suggest that potent or superpotent topical steroids are more effective than moderate potency preparations and do not cause side effects if used responsibly . Powell suggests that once-daily application of a potent/superpotent topical steroid for 2–3 months should induce rapid alleviation of symptoms which should be followed by ‘as necessary’ applications which typically needs to be once or twice per month . Topical steroids should be complemented with the regular use of an emollient, which will soothe the skin and can be used as frequently as desired (see Box 1 for additional environmental modifications that may be helpful).
As with previously discussed, vulvar skin is vulnerable to steroid atrophy if overused and abused. The recommended regimen is required to induce remission of the inflammation and should not be compromised for fear of potential side effects just because the patient is a child.
Second-line therapy with topical calcineurin inhibitors, for example, pimecrolimus 1%, has been used successfully in four girls with childhood lichen sclerosus but long-term follow-up data are not available. There are theoretical concerns that topical calcineurin inhibitors could contribute towards malignant change at a later stage. In January 2006, the US Food and Drug Administration issued a boxed warning requirement based on a theoretical risk of malignancy (including lymphoma) with topical calcineurin inhibitors use. However, in the years since, analyses of epidemiologic and clinical data have failed to demonstrate a causal relationship between the use of topical calcineurin inhibitors and malignancy or lymphoma risk .
Napkin dermatitis
Napkin dermatitis is a common inflammatory skin reaction caused by the specific environmental conditions created in the area covered by the nappy (diaper). In some countries, this is otherwise known as ‘diaper rash’. Features of this dermatitis include high humidity, maceration, friction and contact with urine and faeces . The condition generally affects infants aged 9–12 months and has an estimated incidence of 7–35% . The skin is affected in a characteristic distribution where the nappy is in closest contact and this includes the lower abdomen, lower lumbar region, gluteal area, genitalia and inner aspects of the thighs. ‘High-risk’ groups include those with Hirschsprung’s disease or anorectal malformations.
A particular unusual and potentially severe form of napkin dermatitis has been reported as a case series as re-emerging since the use of reusable nappies . Children present with skin-coloured umbilicated papules or nodules with minimal erythema and can develop into an erosive dermatitis, known as Jacquet erosive dermatitis. This is most likely a result of reusable nappies being less absorbent than disposable ones and may be worsened by the presence of diarrhoea. However, a Cochrane systematic review in 2006 failed to find sufficient high-quality evidence to support or refute the use and type of disposable napkins for the prevention of napkin dermatitis in infants .
It is suggested that barrier preparations can help to prevent napkin dermatitis and there are numerous over the counter preparations marketed for this purpose. A systematic review however, found no randomised controlled trials that compared barrier preparations in the prevention of the condition and so it is not possible to state which agent should is superior. A subsequent randomised controlled trial of 229 infants received petrolatum jelly, or nothing, after every nappy change, found 33% less cases of napkin dermatitis in the intervention group, although this was not statistically significant . The authors do state that the intervention group had more risk factors for developing the condition though, and it is not clear whether a bigger sample size would have shown a different result.
The systematic review asked a second question, which was whether one barrier preparation is better than another in treating napkin dermatitis. They found that zinc oxide impregnated into the nappy, or zinc oxide cream, and petroleum products do have some protective effect and can be used to protect the skin from irritation.
Finally, a further Cochrane systematic review assessed the usefulness of topical vitamin A preparations in the treatment of napkin dermatitis, which had been postulated as a potential treatment strategy, and found no evidence to support their use in the condition .
On the basis of these studies, it is impossible to draw any firm conclusions about the prevention of the condition; however, experts recommend that careful cleaning of skin and regular nappy changes are imperative . It remains to be proven whether barrier creams are effective on clinically normal skin to prevent the development of napkin dermatitis.
Labial adhesions
Labial adhesion is the term used when the labia minora fuse together in prepubescent girls. Other nomenclature used to describe the condition includes labial agglutination , labial fusion and vulvar synechiae . The incidence of labial agglutination has been reported as approximately 0.6–3.0% and occurs most often in younger children up to the age of 2 years . The aetiology of the condition is unknown but it is believed to be associated with the low oestrogenic state in prepubertal girls . Less commonly, it occurs secondary to vulvar inflammation and irritation in which the skin becomes excoriated and denuded leading to fusion of the labial edges during the healing process . Agglutination of the labia majora or minora may occur in a variable degree from the clitoris to the posterior fourchette. There is a flattened appearance to the vulva and it is not possible to see the entire vaginal opening . Labial fusion is usually asymptomatic since urine can pool behind the fusion leading to maceration, secondary dermatitis, cystitis and urethritis.
Management includes explaining to the parents how the fusion has arisen. Reassurance that it is a self-limiting condition and that the internal anatomy is normal is helpful for most parents. Fusion usually resolves by the age of 6 years . No further treatment is necessary in an asymptomatic patient. General hygiene advice should be given as described in other parts of this article .
General skin conditions affecting the vulvar area
Dermatitis
The terms dermatitis and eczema are often used interchangeably. There are different types of dermatitis that can affect the vulvar area, in children these are predominantly atopic (i.e., patients who suffer from a tendency to eczema, asthma or hay fever) or irritant dermatitis. Atopic disease in the genital area (‘atopic vulvitis’) is part of a more widespread complaint of eczema, often combined with asthma and hay fever, or a positive family history for these complaints. Irritant dermatitis will be localised to the area where skin is in contact with the irritant. Common causes of irritant disease in children tend to relate to unintentional poor hygiene such as inadequate wiping and showering. Additionally, neglecting to rinse residual soap products left on the skin (this happens less after bathing) can contribute to irritant dermatitis. Urine and faeces are common irritants so incontinence can also be a causative factor. Napkin dermatitis is a specific pattern of irritant dermatitis in the genital area and is considered separately in this article.
Dermatitis is the most common cause of vulvar symptoms in prepubertal girls. In a case series of 130 girls presenting to an Australian secondary care outpatient clinic between 1996 and 1998, 33% (41/130) had atopic or irritant dermatitis . A further case series study by the same authors found that 24% (9/38) of girls with ‘vulvovaginitis’ had clinical signs of atopic dermatitis on general skin examination .
A personal or family history of atopic disease is an important predictor for the development of dermatitis in children , especially atopic dermatitis. Fischer et al. demonstrated that 85% of 41 girls with atopic or irritant dermatitis had a personal or family history of atopy.
Dermatitis of any type presents as an itchy eruption which takes a relapsing-remitting course. Clinical examination shows erythematous skin that may be scaly at the peripheries (but often shiny in the flexural creases themselves) in association with leathery thickening (lichenification) and often secondary infection. Fig. 3 demonstrates features of atopic vulvitis with poorly demarcated erythema surrounding the vulvar area, minor scale and some slight thickening.
