Pediatric Vulvar Disorders

CHAPTER 26 Pediatric Vulvar Disorders





Congenital vulvar abnormalities


Female genital tract developmental abnormalities are rare. They may involve only the external genitalia or the whole reproductive tract (Tables 26.1 and 26.2).


Table 26.1 Ambiguous External Genitalia































Clinical Presentation Diagnosis Etiology
Clitoromegaly or variable phallus formation with ventral opening; variable labial formation with opening or scrotal sac formation Female pseudohermaphroditism Congenital adrenal hyperplasia Enzyme deficiencies (recessive) 21-Hydroxylase deficiency 11-Hydroxylase deficiency (rare)
  Exogenous hormones Maternal androgen-producing tumor
    Exogenous androgen exposure
  Male pseudohermaphroditism Lack of gonadotropin
    Enzyme defect in testosterone synthesis
    Target tissue androgen receptor defect

Table 26.2 Abnormalities of External Genitalia























Disorder Clinical Presentation Treatment
Labial hypertrophy Long labia minora Surgery
Hymenal abnormalities Variable hymenal openings or complete closure Surgery
Hemangiomas Red to purple vascular macules, nodules or plaques with or without erosions or ulcers Reassurance; laser destruction if extensive or ulcerated
Congenital nevi Small to large pigmented macules or plaques Biopsy if atypical; close observation as indicated


Ambiguous external genitalia


At birth the external genital organs in these conditions are not clearly male or female, thus the sexual ambiguity. Eighty percent of ambiguous genitalia are due to female pseudohermaphroditism with androgenization of the female fetus. Such infants present with an enlarged phallus alone or associated with some degree of labioscrotal fusion because of a recessive congenital enzymatic defect of adrenal steroid biosynthesis. The most common cause is a 21-hydroxylase deficiency resulting in overproduction of cortisol and androgen that virilizes the fetal female external genitalia. Rarely this can be caused by an 11-hydroxylase deficiency. Exogenous hormones from a maternal androgen-producing tumor or maternal ingestion of androgen can also produce virilization of a female fetus.


Male pseudohermaphroditism is far less common and represents only 15% of cases of ambiguous genitalia. In these infants there is a partial or complete block in the masculinization process during development due to a lack of gonadotropins, an enzyme defect in testosterone biosynthesis, or a defect in the androgen-dependent target tissue response (e.g., androgen receptor defect or 5α-reductase deficiency). Clinically these children present with varying degrees of phallus formation, scrotal sac formation, and with varying genital openings (Figures 26.1 and 26.2)13.






Labial adhesions


Superficial fusion of the labia minora occurs in 1–3% of prepubertal girls. It starts posteriorly and usually involves the posterior two-thirds of the labia minora. Only rarely is there almost complete labial agglutination .This is an acquired condition seen mostly in children 2–3 years of age4. The cause is unknown, but local irritation, poor hygiene, genital trauma, and lack of estrogen may all play a role5. The result is inflammation and adherence of skin surfaces on either side of the labia, giving the vulva a flat appearance. Localized posterior fusion is often minor and asymptomatic. Although this condition resolves spontaneously with pubertal estrogenization, extensive fusion leaving only a pinhole opening can result in urine retention, urinary infection, genital irritation, and burning. The first line of therapy is topical estrogen cream, used two or three times a day with gentle traction for 2–3 weeks if needed6. If extensive, surgery may be required1,710.




Hemangiomas


Hemangiomata are the most common vascular lesions, affecting 5% of newborns with a predominance in females (a male-to-female ratio of 1:3). These benign tumors are characterized by endothelial cell hyperproliferation and they form pink to reddish papules or plaques anywhere on the body. They usually start within a few days of birth with a flat red patch that gradually enlarges. The proliferative phase of rapid growth lasts about 8–9 months, then the lesion stabilizes for a time. Involution starts at about 2 years of age, and most of these lesions flatten into a whitish scar by the age of 9 years. Symptoms depend on size: large tumors can create considerable discomfort with ulcers, erosions, and bleeding (Figures 26.6 and 26.7). Treatment depends on the degree of involvement, and asymptomatic lesions may be left to resolve on their own. Corticosteroids may be used for problematic hemangiomata, intralesionally for small lesions and systemically for large ones. Hemangiomata that bleed or ulcerate may respond well to early laser treatment. For severe cases, co-management with a pediatric dermatologist is recommended1318.






Vulvar dermatoses


Many childhood vulvar problems are associated with estrogen depletion (Table 26.3). Estrogen is an important factor in vulvar skin reactions to irritants and infection. Estrogen is present and effective during the first 3 months of life and then again 2–3 years before menarche. In the interval, the vulva is relatively estrogen-deficient. In this state, the labia minora and the vulvar epithelia are thin with a visible capillary network. The result is a weakened barrier function and the tissue is more susceptible to irritants but resistant to Candida. The estrogenized vulva is resilient and pink with plump labia minora and well-moisturized vulvar introital epithelium. This results in resistance to chemical and physical irritants but increased susceptibility to Candida.





Diaper dermatitis


This term means any dermatitis in the diaper area, usually due to repeated contact irritation from urine, feces, and friction. This is the commonest skin condition in infancy, with a prevalence of 7–35%. Babies aged 7–12 months are most commonly affected. The skin is pink to red, with shiny or chapped areas of friction. Typically the convex surfaces of the mons pubis, the labia majora, and the buttocks are irritated, but the skin folds are usually spared (Figure 26.9). Children with atopic dermatitis or a tendency to psoriasis are more susceptible. With time, a mixed pattern develops due to secondary infection with bacteria and Candida. The skin in the folds and on the convex surfaces may become red, chapped, and raw with scattered satellite pustules or desquamating papules and, if severe, open ulcers may develop. Treatment is to remove the irritants and change the diapers more frequently and, when possible, to leave the infant without a diaper. Superabsorbent disposable diapers are best to reduce irritation. Routine cleansing should be with bare or vinyl-gloved hands in plain water with a soapless cleanser (Cetaphil, unscented Dove bar or superfatted soap) followed by a thorough rinse, then 1% hydrocortisone ointment. An imidazole cream (clotrimazole) is added for secondary yeast. For diaper changes away from home, cleansing with plain water or light mineral oil on a soft tissue or cotton ball is permitted2329. “Baby wipes” are best avoided.







Psoriasis


Psoriasis is a common, hereditary, red, scaly rash of the skin. In infants, it may present as a chronic, sometimes itchy, diaper dermatitis with bright red, sharply outlined plaques around the mons pubis and labia majora through the perineum and often up into the gluteal cleft with varying degrees of fissuring. In young infants it often presents as a diaper dermatitis that is resistant to therapy. The white scales typical of psoriasis are not present in body folds, but may occur on elbows, knees, or scalp. A history of psoriasis in other family members helps to make a diagnosis3739.


Treatment involves avoiding trauma such as irritating soaps and lotions, and keeping the area cool and dry. Cleanse with a bland product such as Cetaphil cleanser, unscented Dove bar, or superfatted soap. A low-potency steroid ointment (hydrocortisone 2.5%, desonide 0.05%) may be used twice a day initially, tapering to 1% hydrocortisone ointment with response. In more severe cases, mid- or even high-potency topical corticosteroids may be necessary for 1–2 weeks followed by long-term management with intermittent use of low-dose hydrocortisone. For concurrent secondary infection antiyeast topical clotrimazole and antibacterial mupirocin ointment may be needed. The topical calcineurin inhibitors can be useful as steroid-sparing agents: pimecrolimus 1% cream for milder psoriasis and tacrolimus 0.03% and 0.1% ointment for more severe. These can be used twice a day intermittently for psoriasis and atopic dermatitis (see below)4042.



Apr 29, 2016 | Posted by in Dermatology | Comments Off on Pediatric Vulvar Disorders
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