The classic signs of vulvovaginitis—introital irritation and vaginal discharge—are said to account for 80% to 90% of outpatient visits of children to gynecologists (7). Much of this is attributed to nonspecific vulvovaginitis due to poor hygiene. The pediatrician, family medicine practitioner, or nurse practitioner often sees girls with vaginitis, labial adhesions, and vulvar pruritus due to pinworms. The dermatologist, on the other hand, is likely to see a different population of girls with vulvar complaints and thus diagnose specific dermatoses. Fischer reported on 130 prepubertal girls who presented with a vulvar complaint to a pediatric dermatology clinic. The most common diagnosis was atopic dermatitis followed by lichen sclerosus and psoriasis (Table 5-1) (8). In another study of 38 prepubertal girls with chronic vulvitis (excluding lichen sclerosus), genital examination showed that 71% had psoriasis and 24% had atopic dermatitis (4). In contrast, a dermatologic study by Paek and colleagues found that vulvar pruritus in children was mostly nonspecific in nature (9).
Reaction to this study prompted Fischer and Margesson to recommend a vigorous search for specific causes, rather than attribution to nonspecific causes, when the complaint in children is vulvar pruritus (10).

Rather than choose sides (nonspecific vs. specific causes of vulvar pruritus or vulvitis)—since either may be correct depending on the clinical situation—good rules of thumb to consider are as follows: (a) if what seems like nonspecific vulvovaginitis persists or is unresponsive to treatment, a primary vulvar skin disease should be considered; (b) if a primary vulvar skin disease seems evident but is not clearing, nonspecific causes such as hygiene issues as well as vaginitis should be reconsidered; and (c) if a vulvar skin disease seems apparent, the clinician should at a minimum consider those vulvar dermatoses that are commonly reported in children: atopic dermatitis, lichen sclerosus, and psoriasis.

A core group of medications can be used for most pediatric and adolescent vulvar dermatoses.

In this procedure, a dermal curette is used to scrape or scoop out a skin lesion. A dermal curette is an instrument with a loop, square, or cup tip. Tips range in size from 1 to 7 mm. A 3-mm loop-tip curette is invaluable for removing lesions of molluscum contagiosum; this technique can quickly be learned. Multiple lesions can be removed in a few minutes. EMLA cream may be applied beforehand to reduce pain; hemostasis is achieved with pressure and application of aluminum chloride (this may sting; applying the aluminum chloride before curettage seems to decrease stinging).

Freezing vulvar molluscum contagiosum and condyloma acuminata with liquid nitrogen is indispensable in the office setting and would benefit clinicians practicing office gynecology. Liquid nitrogen is stored in a large tank and may be dispensed into a liquid nitrogen spray unit. Alternatively, liquid nitrogen is dispensed into a small thermos or a Styrofoam cup; a cotton-tipped applicator may be dipped in the liquid nitrogen and then touched to the lesion. EMLA cream may be applied beforehand as with curettage. Lesions should be frozen for 15 to 30 seconds. The lesion eventually crusts and sloughs off. The patient should be warned that blistering may occur.

Sebaceous glands can easily be seen as an incidental finding along the medial and lateral aspects of the labia minora, as well as the medial aspects of the labia majora (21). Known as Fordyce spots, these glands are not associated with hair follicles. Some variability exists in their size; their number may range from a few (Fig. 5-4) to many and in some women may completely cover the inner labia minora and take on a “cobblestone” appearance. Their color may be white, yellowish, cream-colored, pink, or more pigmented in individuals with darker skin. Their function is to secrete sebum, an oily substance that lubricates genital skin. Fordyce spots also occur on the buccal mucosa and vermillion border of the lip; on the female areolae they are known as Montgomery tubercles.

Vestibular papillomatosis describes the presence of multiple small (1 to 3 mm) papillae of the vulvar vestibule (21). Their pigmentation is similar to the patient’s general skin pigmentation (Figs. 5-5 and 5-6). The weight of evidence does not support a causal role for HPV. Thus, they should be considered physiologic and no treatment or biopsy is necessary.

Irritant diaper dermatitis is a nonspecific term that describes a spectrum of symptoms in the diaper area caused by inflammatory skin reactions. Four main factors contribute: wetness, friction, urine and feces, and microorganisms (22). Wet diapers result in excessive hydration and maceration of the stratum corneum. This leads to impairment of the already less than perfect barrier function of vulvar skin, which can result in enhanced penetration by irritants and microbes and makes vulvar skin more susceptible to frictional trauma. Mechanical trauma from skin to diaper contact further disrupts and macerates the stratum corneum and exacerbates barrier dysfunction. Fecal bacteria degrade urea in urine, thus releasing ammonia and increasing pH. Ammonia itself does not irritate intact skin; it may cause irritation by contributing to a higher local pH. Fecal lipases and proteases are activated by increased pH; this further disrupts the epidermal barrier. The warm, humid area of the perineum is ideal for the proliferation of microbes; in addition, the innate antimicrobial flora cannot survive the higher pH environment.

Perineal erythema is the hallmark of irritant diaper dermatitis (Fig. 5-7), often accompanied by maceration, erosions, and ulcerations. Unlike candidal diaper dermatitis, irritant diaper dermatitis usually spares the inguinal skin folds since these have less contact with the diaper and urine and feces. Similar findings will be seen in older girls and adolescents with urinary and fecal incontinence. The differential diagnosis includes candidal diaper dermatitis, seborrheic dermatitis, and, in severe cases, rarer entities such as Langerhans cell histiocytosis and acrodermatitis enteropathica. No specific tests are necessary. If candidal superinfection is suspected, a surface skin culture can be obtained. Bacterial cultures may be difficult to interpret since fecal bacteria invariably will be recovered. However, a culture can be helpful if there is suspicion of pathogens such as Staphylococcus aureus and Streptococcus pyogenes or if vaginitis is present, especially in the older child.

Treatment should focus on frequent diaper changes to maintain dryness and loose-fitting diapers to minimize contact between skin and urine and feces. Brief intervals without diapers are also beneficial. Cleansing should be gentle with an irritant- and fragrance-free soap or cleanser followed by patting dry and liberal application of a barrier preparation. Infant wipes usually consist of a nonwoven carrier that is soaked with an emulsion-type water or oil lotion. Preservative- and fragrance-free wipes should be chosen and are well tolerated in infants. Restoring the barrier function of perineal skin is vital for healing of irritant diaper dermatitis and is a continuous theme in the treatment of all vulvar inflammatory dermatoses. Pastes are the most desirable barriers followed by ointments; ointments are superior to creams and lotions, which are poorly adherent, are minimally occlusive, and may contain preservatives. The barrier pastes should be applied thickly and can be covered by petroleum jelly to avoid sticking to diapers. A group VI or VII topical corticosteroid ointment is often necessary in moderate to severe irritant diaper dermatitis. The agent can be applied twice a day for several days to a week. More potent topical corticosteroids as well as combination steroid/antifungal preparations should be avoided in infants (see Medications section). Secondary candidal infection is common, especially if the eruption has been present for several days or is severe. A topical azole antifungal agent, nystatin, or ciclopirox can be used. With treatment, irritant diaper dermatitis should resolve over 1 to 2 weeks. Failure of the eruption to resolve should prompt a complete review of all prescribed and actual treatment measures and consideration of other potential diagnoses.

Candidal diaper dermatitis is a superficial fungal infection of perineal skin; vaginal involvement is not common in infants. Candida species may flourish in the wet, warm environment of the diaper area. The examination shows beefy red erythema on perineal skin, the buttocks, the lower abdomen, and inguinal areas (Fig. 5-8). Pinpoint vesicles and pustules—satellite lesions—may be seen at the periphery. An antecedent history of oral antibiotics may be obtained. Concomitant oral thrush and maternal nipple candidal infection may occur. A KOH preparation showing budding yeast and pseudohyphae
confirms the diagnosis; if KOH capability is not present, a surface culture can be obtained and empiric treatment begun.

Treatment includes maintaining dryness with frequent diaper changes or not using diapers for short periods. An antifungal cream (an azole cream, nystatin, or ciclopirox) is used twice a day until the eruption clears. The addition of a topical antibiotic such as mupirocin ointment two to three times a day is effective for concomitant bacterial infection. The eruption may take 1 to 3 weeks to resolve. If erythema from irritation persists beyond 10 to 14 days, a brief course of topical hydrocortisone 1% cream may be applied followed in a few hours by application of the topical antifungal agent. Baby powders may help prevent recurrence by absorbing moisture.

Jacquet erosive dermatitis is a form of severe irritant dermatitis (24,25). This occurs in infants in the setting of frequent loose stools, poor hygiene, and infrequent diapers changes, but has also been reported in older children and adolescents with urinary or fecal incontinence, for example, in the setting of spina bifida or after surgery for Hirschsprung disease (26,27). Lesions appear as punched-out ulcers with elevated, indurated borders (Figs. 5-9 and 5-10). Other dermatoses that cause erosions and ulcers should be considered including HSV infection, erosive candidiasis, allergic contact dermatitis, and Langerhans cell histiocytosis. While the diagnosis can be made clinically, surface cultures for Candida, HSV, and bacteria may be obtained. Treatment is the same as for irritant diaper dermatitis, but this eruption make take longer to heal. Vigilance is required in home skin care. Close follow-up and reassurance are necessary as this eruption evokes a great deal of parental concern.

This is a nodular eruption that may arise in the setting of irritant diaper dermatitis (28). The etiology is unknown but several factors have been suggested including Candida albicans, topical use of fluorinated corticosteroids, and as a response to long-standing infection. Lesions present with erythematous pseudoverrucous papules and nodules on groin and buttock skin. The differential diagnosis includes condyloma acuminata, HSV infection, and Langerhans cell histiocytosis. Treatment is the same as for irritant diaper dermatitis. Lesions should resolve over the course of weeks to months; residual scarring is not unusual.

Seborrheic dermatitis is an inflammatory dermatosis that may occur in infants as well as in adolescents and adults. The exact cause of seborrheic dermatitis is unknown. The yeast Malassezia furfur has been implicated. In infants, perineal lesions appear as well-circumscribed, greasy, erythematous plaques (Fig. 5-11) (29). Scale tends to be less prominent in the diaper area due to occlusion and moisture but is commonly seen with involvement of the scalp, eyebrows, ears, and trunk. Adolescent involvement is usually of the scalp, eyebrows, glabella, and nasolabial folds. Treatment with a group VI or group VII corticosteroid ointment or lotion twice a day for 1 to 2 weeks should allow rapid healing. Moisturizing is also important for healing as well as for preventing recurrences. While recurrences are common, the overall eruption usually resolves by about 6 months of age.
If the eruption persists longer, consider the diagnosis of atopic dermatitis, especially if there is a family history of atopy. There is no clear link between infantile and adolescent or adult seborrheic dermatitis. Infants with generalized seborrheic dermatitis and failure to thrive should be evaluated for complement C5 immunodeficiency.

This is a group of idiopathic diseases characterized by proliferation of histiocytes and includes the classic descriptions of eosinophilic granuloma (of bone), Hand-Schüller-Christian disease (multifocal), and Letterer-Siwe disease (disseminated) (30). Solitary cutaneous disease presents with oral, perineal, vulvar (31), or retroauricular lesions. Skin lesions may also be seen in classic multifocal Langerhan’s cell histiocytosis (LCH) and acute disseminated LCH, which has multiorgan involvement including hepatosplenomegaly (32). A congenital form manifests as skin lesions at birth or during the early postnatal period; organ involvement may also occur. Skin lesions appear as closely set petechiae and yellow–brown papules topped with scale and crust; the papules may coalesce to form an erythematous, weeping, or crusted eruption (Figs. 5-12 and 5-13). Hemorrhagic infiltrated nodules and plaques may be seen as well as erosions and ulceration.

Perineal lesions of LCH may mimic seborrheic dermatitis; however, lesions of LCH tend to be hemorrhagic and more infiltrative in nature. Erosive candidiasis lacks hemorrhagic lesions. HSV infection of atopic dermatitis (eczema herpeticum) may cause ulcerated papules and plaques. Acrodermatitis enteropathica may cause perineal erosions. Skin biopsy will show the presence of CD1 a and S-100 surface markers and characteristic racquet-shaped bodies in the cytoplasm (Birbeck granules). Surface cultures for Candida species and HSV may be performed if those diagnoses are entertained. A systemic workup is needed to determine extent of the disease. Localized skin disease is treated with moderate to potent topical corticosteroids; other treatment modalities include psoralen plus ultraviolet A light therapy (PUVA) and ultraviolet B excimer laser. Multifocal and disseminated disease are treated with chemotherapy. The congenital form tends to resolve spontaneously within weeks to months. Prognosis is excellent for patients with single-system disease; multifocal and disseminated disease have variable prognoses (30).

Acrodermatitis enteropathica is an autosomal recessive disorder caused by zinc deficiency (33). The cause of acrodermatitis enteropathica is an inability to absorb sufficient zinc from the diet. Secondary zinc deficiency, as may occur with long-term parenteral nutrition without supplemental zinc or with chronic malabsorption, may have similar clinical findings. Skin findings include vesicobullous, eczematous, dry, scaly, or psoriasiform plaques symmetrically distributed in the perioral, acral, and perineal areas (Fig. 5-14) and also on the cheeks, knees, and elbows. Erosions and superinfection with C. albicans may occur. The clinical features as well as detection of a low plasma zinc level make the diagnosis. Treatment is with oral zinc therapy. The cutaneous manifestations of the disease are rapidly abolished with therapy. Measures used for the treatment of irritant dermatitis may help restore the skin barrier.

Hemangiomas are the most common tumor of infancy (34). They are benign neoplasms that arise from the rapid proliferation of endothelial cells. Risk factors include female gender, white race, premature birth, and maternal chorionic villous sampling. Hemangiomas of infancy (HOI) have been classified
as superficial, deep, or mixed; another classification looks at hemangiomas as localized, segmental, or indeterminate. Vulvar hemangiomas tend to be superficial and localized (Fig. 5-15). They may be associated with urogenital and anorectal anomalies such as anterior or vestibular anus, imperforate anus, and atrophy or absence of the labia minora.

Hemangiomas start within a few weeks after birth as a macular erythema, a telangiectatic patch, or an area of pallor; this undergoes a period of rapid growth followed by slow involution. Graying of the surface is an early sign of involution. Perineal hemangiomas are prone to ulceration and infection due to frictional and chemical trauma (35). Large perineal hemangiomas may interfere with micturition or defecation. Ulcerated hemangiomas (Fig. 5-16) may result in pain, infection, bleeding, and scarring. Bleeding may be recurrent or life-threatening (36). Hemangiomas have also been reported of the vaginal wall with resultant vaginal bleeding. The diagnosis of a vulvar hemangioma is usually apparent clinically. Vascular malformations are usually more poorly circumscribed, do not change in size rapidly but grow in proportion to the child, and do not involute. Tests are generally not needed for diagnosis. However, imaging tests such as magnetic resonance imaging (MRI) may help differentiate hemangiomas from other vascular lesions and may determine the depth of the lesion. The presence of multiple cutaneous hemangiomas should prompt a search for visceral hemangiomas. If a lesional biopsy is necessary, positive staining for the immunohistochemical marker glucose transporter protein 1 (GLUT-1) can be used to help distinguish HOI from other vascular tumors and malformations. Other markers that stain positive are the placenta-associated antigens FcγRII, merosin, and LeY (34).

Nonintervention is appropriate for most small lesions without functional impairment. Lesions with functional impairment or that ulcerate or bleed need treatment. Superabsorbent diapers will minimize contact of the hemangiomas with urine. For ulceration, wound care may be performed with compresses (saline or Burrow solution) followed by application of a topical antibiotic (metronidazole gel or mupirocin ointment), followed by application of petroleum jelly–impregnated gauze or a barrier ointment. Topical platelet-derived growth factor (becaplermin gel, Regranex) has shown healing in ulcerated hemangiomas (37); it is not approved by the U.S. Food and Drug Administration (FDA) for this purpose. Other reported treatments include imiquimod (38) and topical and intralesional steroids.

Pain control may be needed with acetaminophen, acetaminophen with codeine, and judicious use of topical lidocaine as this agent carries the risk of systemic toxicity. Systemic steroids such as prednisone or prednisolone 2 to 3 mg/kg/d as an early morning dose have been used; if responsive, cessation of growth or the onset or involution should occur after about 2 weeks (34). Side effects of systemic steroids include hypertension, gastrointestinal upset and bleeding, adrenal suppression, and immunosuppression. Use of propranolol for infantile hemangiomas soared after growth arrest of an infant’s hemangiomas was incidentally noted when propranolol was started for
obstructive hypertrophic cardiomyopathy (34,39

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