Fiona M. Lewis Vulval symptoms in children are not uncommon, but there is much less scientific study of vulval disorders in children and often they are included in case series with adults. Several dermatoses will affect the vulva in adults and children, but there are different patterns of disease, with some conditions specific to, or more common in, the paediatric age group. Children should be seen in an environment that is appropriate for their needs, ideally in specific paediatric vulval clinics where trained nursing staff and other specialities relevant to their management are easily accessible. Adapting the principles of history taking and examination in children is discussed in Chapter 5. The common inflammatory conditions seen in girls and adolescents are dealt with in this chapter. Napkin or diaper dermatitis is a general term used to describe rashes that involve the napkin area [1,2]. Although the majority of these are related to eczema or psoriasis, there are other important diagnoses to consider, especially if there is a lack of response to treatment. A better term is therefore napkin/diaper eruptions. The main causes are listed in Table 49.1. It is important to take a full history including details about hygiene practices, nappies used, and bowel and bladder function [2]. Table 49.1 Causes of napkin/diaper eruptions. There are basic principles which apply to the management of all napkin eruptions and, indeed, other inflammatory problems. The main aim of treatment is to protect the barrier function of the skin. All irritants should be avoided, and this includes soaps, bubble baths, wet wipes, perfumed ‘baby’ products, and tight clothing in older children. An emollient ointment can be used as a soap substitute and a barrier preparation applied if there is urine contact with the skin. Issues with incontinence and constipation must be addressed as these will cause an irritant dermatitis and aggravate any inflammatory dermatosis. The commonest problem seen in paediatric vulval clinics is eczema [3]. Atopic eczema tends to spare the napkin area as it is well hydrated and so may present only in later childhood. Seborrhoeic eczema is more common in infants, older children, and adolescents. The most common form of eczema presenting in childhood is irritant eczema, accounting for about 40% of cases. However, this is more common in those with a history of atopy. Allergic contact dermatitis is rare in children. Napkin eruptions and irritant reactions are very common. Interestingly, there are geographic differences which relate to cultural and cleansing practices. The lowest rate is in China, where the use of barrier preventative preparations is high [4]. Maternal oestrogens will influence the neonatal vulva for the first few weeks of life, but as levels fall, the skin becomes more vulnerable to irritants and infection. The area is moist and occluded and subject to several irritants, particularly urine and faeces in young children who are not toilet trained. Proteases and lipases in faeces are highly irritant and will lead to problems. Poor hygiene and the short anal–vulval distance allows irritants to easily pass to the vulva. Once a rash is noted, there is a tendency to overuse topical treatments, particularly anti‐candidal and anti‐fungal preparations, which can then add to the irritant effects. The design of nappies is aimed at reducing irritation and modifications are constantly made, the principle of modern nappies being that fluid is drawn through hydrophilic material into a superabsorbent layer which aims to keep the skin as dry as possible. The main symptom is pruritus, but soreness and dysuria are also common complaints. The outer labia majora and buttocks are most frequently affected as these are the convex areas most in contact with any irritants (Figure 49.1). Diffuse erythema, sometimes with mild scaling at the edges is seen. In severe cases, erosion, fissuring, and secondary infection are seen. Figure 49.1 Irritant eczema affecting convex labia majora. Seborrhoeic eczema, psoriasis, tinea, and streptococcal infection can all present with similar features. The erythema in psoriasis tends to be very well defined and is a useful sign. The general principles of avoidance of irritants and the use of emollients are key to management. An ointment‐based emollient used as a soap substitute is helpful. A mild topical steroid such as 1% hydrocortisone ointment can be used for short periods to control inflammation. The problem may relapse and remit, and ongoing care of the area with regular emollients must be emphasised to the parents. Allergic contact dermatitis is less common in children, but has been reported to rubber components [5], additives [6], and dyes [7]. There is often a distinct distribution across the buttocks and hips called the ‘Lucky Luke’ pattern as it resembles a cowboy holster. Other products such as preservatives in wet wipes and fragrances can cause a contact allergy, and patch testing may be required. If there is a chronic and severe irritant reaction, usually in the setting of urine and faecal incontinence, erosions and ulceration can occur. This has previously been called Jacquet’s erosive dermatitis. Nodular, granulomatous reactions can also occur, which have been termed granuloma gluteale infantum. A similar entity of pseudoverrucous papules and nodules is described, but it is very likely that these three conditions are all a part of the same spectrum of severe irritancy [8]. With the development of disposable nappies, these severe reactions became rare, although there is no evidence that these prevent napkin eruptions in systematic reviews [9]. However, as cloth nappies are used more in an effort to be more eco‐friendly, cases are re‐emerging [10,11]. The most important treatment is to remove the cause. Barriers and emollients need to be applied regularly. It is thought that the use of potent topical steroids may be a factor in the development of these reactions, and so they are best avoided. Topical tacrolimus was helpful in one case [12]. Psoriasis is a common dermatosis in adults and children, and the clinical features are similar in both groups [13]. It can be a challenging diagnosis as there are frequently overlapping features with other dermatoses such as eczema. There is little information about the incidence of genital psoriasis, but in a large study of 1262 paediatric cases, 8.9% had anogenital disease [14]. A family history is frequently reported, but no specific genetic patterns have been established. The most common symptom is pruritus, but dysuria will occur if there is fissuring. Discomfort with defaecation in perianal disease may lead to constipation. The mons pubis and labia majora are most commonly involved with well‐defined, symmetrical plaques (Figure 49.2), but inflammation of the inguinal folds, perianal skin, and gluteal cleft is often seen. Scaling does not occur due to the moist environment, but fissuring is found more frequently in flexural psoriasis. In infants, the napkin areas may be the only site involved. In older children, lesions at other sites such as the scalp, nails, extensor surfaces, and the umbilicus may offer clues to confirm the diagnosis. Figure 49.2 Extensive psoriasis with maceration in inguinal folds and fissuring. Eczema, tinea infection, and other infections can look similar. The erythema in psoriasis is well defined, but in eczema, it is often diffuse, and this may be a useful distinguishing sign. It is also possible that psoriasis may koebnerise after an irritant reaction, and the two may coexist. Secondary bacterial infection can be seen, and affected 12.1% of children with genital psoriasis [13]. S. aureus and streptococci were the organisms responsible. Treatment of psoriasis can be challenging. General measures are important, with avoidance of irritants and the use of emollients. Many of the topical treatments used for psoriasis elsewhere such as Vitamin D analogues are too irritant on the genital skin. A mild to moderately potent steroid can be used daily initially, followed by tapering down the frequency of application. Weak tar preparations were used as maintenance therapy in one study, with over 90% responding [13]. Calcineurin inhibitors may be helpful but can sting when applied. As with any chronic disease, psoriasis may relapse and remit and require treatment from time to time.
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Inflammatory Dermatoses of the Vulva in Children
Napkin (diaper) eruptions
Disease
Sub‐types
Genetic
Epidermolysis bullosa
Inflammatory
Eczema/dermatitis
Irritant
Atopic
Seborrhoeic
Allergic contact dermatitis
Psoriasis
Infection
Scabies
Staphylococcal scalded skin
Streptococcal infection
Associated with systemic disease
Crohn’s disease
Kawasaki disease
Metabolic
Acrodermatitis enteropathica
Malignant
Langerhans cell histiocytosis
General principles of management of napkin eruptions
Vulval eczema (dermatitis)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Treatment
Prognosis and follow‐up
Allergic contact dermatitis
Severe irritant reactions
Psoriasis
Epidemiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis and follow‐up
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