Fig. 1.1
Streptococcus pyogenes vulvovaginitis
Staphylococcus aureus may cause opportunistic skin infections which may appear as impetiginous, bullous, or suppurative.
In case of suspected sexually transmitted disease, evaluation for Neisseria Gonorrhoeae and Chlamydia Trachomatis can be obtained sending a urine or vaginal sampling for NAAT (nucleic acid amplification testing). Trichomonas vaginalis can be identified in vaginal secretions by wet mount examination, culture or NAAT. When sexually transmitted diseases are detected and mother to child transmission can be excluded, sexual abuse should be considered.
Physical examination is usually sufficient to diagnose viral genital infection, since they show characteristic lesions.
Feces and urine culture may be useful respectively in cases of diarrheal illness and dysuria.
1.4.1 Vulvar Lesions
As symptoms of vulvovaginitis, vulvar dermatosis, and systemic disease with vulvar manifestations may often overlap, recovery of specific vulvar lesions may give a clue to diagnosis. Hence, awareness of vulvar lesion is essential for differential diagnosis. It has been calculated that, in a series of 130 children presenting with vulvar symptoms to a dermatologic clinic, 33% had atopic or irritant dermatitis, 18% had lichen sclerosus, 17% psoriasis, 15% hemangiomas, less than 20% had infection, and 10% had streptococcal infection; vulvar manifestations of systemic disease were rare [13]. Systemic diseases with vulvar manifestations include: measles, chickenpox, varicella, scarlet fever, mononucleosis, Stevens-Jonhnson syndrome, Kawasaki disease, Langherans cell histiocytosis, Crohn disease, Behçet’s syndrome, Staphylococcal scaled skinsyndrome, Henoch-Schönlein purpura, zinc deficiency [7, 13].
Skin lesions may be differentiated in primary and secondary types (Table 1.1) [22, 23].
Table 1.1
Primary and secondary skin lesions
Primary lesions | Definitions |
|---|---|
Macule | Small area (<1.5 cm) of color change; no elevation and no substance on palpation |
Patch | Large area (>1.5 cm) of color change; no elevation and no substance on palpation |
Papule | Small (<1.5 cm) elevated and palpable lesion |
Plaque | Large (> 1.5 cm elevated, palpable, and flat topped lesion |
Nodule | Large (>1.5 cm) papule; often hemispherical or poorly marginated; may be located on the surface, within, or below the skin; nodules may be cystic or solid |
Vesicle | Small (<0.5 cm) fluid filler blister; the fluid is clear (blister: a compartmentalized, fluid filled elevation of the skin or mucosa) |
Bullae | A large (>0.5 cm) fluid filled blister; the fluid is clear |
Pustula | Pus-filled blister; the fluid is white or yellow |
Secondary lesions | Definitions |
Eczema | A group of inflammatory diseases that are clinically characterized by the presence of itchy, poorly marginated red plaques with minor evidence of microvesiculation and seven or more frequent surface disruption |
Lichenification | Thickening of the tissue and increase prominence of skin markings. Scale may or may not be detectable in vulvar lichenification. Lichenification may be bright-red, dusky-red, white, or skin colored in appearance |
Excoriation | Surface disruption (notably excoriation) occurring as a resultof the “itch-scratch” cycle |
Erosion | A shallow defect in the skin surface; absence of some, or all, of the epidermis down to the basement membrane; the dermis is intact |
Fissure | A thin, linear erosion of the skin surface |
Ulcer | Deeper defect; absence of the epidermis and some, or all, of the dermis |
For clinical purpose, it may be helpful to adopt the 2011 ISSVD (International Society for the Study of Vulvovaginal Disease) Terminology and Classification of Vulvar Dermatological Disorders as suggested by Lynch [23]. Vulvar diseases may be clustered into groups with similar clinical presentations. As some skin disease are polymorphic they may be listed in more than one group. For each group, a list of diseases which may occur in childhood is reported below (adapted from [23]):
1.4.1.1 Skin Colored Lesions
Papules and nodules may be caused by Human Papilloma Virus, Molluscum contagiosum, skin tag, epidermal and mucinous cyst, scar, and nevus. If the lesion is single and appears on the median rafe of perineum the differential diagnosis include the infantile perianal pyramidal protrusion, which is a peduncolated congenital protrusion typically located anterior to the anus [7]. Plaques are present in lichen simplex chronicus and other lichenified disease.
The hallmark of Human Papilloma Virus (HPV) infection is condyloma acuminata which may appear as papule or nodule, solitary or multiple. Lesion may be skin colored, red, white, or dark colored. Acquisition of the virus may occur by mother during gestation or delivery. Postnatal infection may be acquired through inoculation from nongenital mucocutaneous lesion or fomite transmission [24, 25].
Umbilicated and caseous-plug containing papules are typically caused by Molluscum contagiosum. Lesions may be single or, more often, multiple; skin colored, white, red, dark colored. Size of lesions ranges from 1 to 5 mm (giant lesions up to 1.5 cm are rare, may be present in HIV infection) [26]. Transmission may occur by casual contact, fomite spread, autoinoculation, and sexual contact. Treatment options include: watchful waiting (spontaneous resolution may take month or years), off-label application of imiquimod or tretinoin, curettage, and diatermocoagulation [7].
1.4.1.2 Red Patches and Plaques
Clinicians should consider allergic/irritant/atopic dermatitis, eczematous changes superimposed on other vulvar disorders, candidiasis, psoriasis, lichen simplex chronicus, lichen planus, hemangioma of infancy, cellulitis, pityriasis rosea, zinc deficiency, Langherans cell histiocytosis.
Psoriasis is a chronic immune-mediated inflammatory disease which may involve skin and/or joints [27]. Lesions are red-pink, symmetric, well-demarcated plaques. Scales are seen at the periphery. Skin lesions affecting the scalp and extensor surfaces of the limbs (elbows and knees) are typical of adulthood, whereas vulvar and anogenital localization are the most frequent in childhood [26].
Atopic dermatitis is a skin chronic disease with complex pathogenesis (genetic, immunologic, and environmental factors are involved). Lesions are erythematous scaly plaques with undefined margins. Lichenifications may compare with time and scratching. The disease may be indistinguishable from contact dermatitis (Fig. 1.2).
Fig. 1.2
Atopic dermatitis
Contact dermatitis (irritant and allergic) may present as acute or subacute/chronic disease. Acute manifestations may be erythema, edema, and vesicle which evolve into painful erosions and ulcerations. Subacute and chronic dermatitis may appear with erythema, lichenification, red plaques, excoriations, and fissures [7].
A particular condition frequently found in pediatric age is irritant diaper dermatitis. It commonly affects infants with a peak incidence between 9 and 12 months. It appears as perineal erythema, restricted to the area covered by diapers but usually spares inguinal areas [28, 29]. Macerations, erosions, and ulcerations may be associated. It is caused by overhydration of the skin, maceration, prolonged contact with urine and faces, retained diaper soap and is a prototypical example of irritant contact dermatitis [30]. Moreover, the onset of secondary infection caused by Candida albicans or bacteria such as Bacillus faecalis, Proteus, Pseudomonas, Staphylococcus, and Streptococcus is frequent. Candidal diaper dermatitis is a superficial infection of skin, involving perineal skin, buttock, lower abdomen, inguinal areas, characterized by beefy red erythema often associated to maceration, erosions, and ulcerations [7].
Piritiasis rosea is a papulosquamous eruption usually presenting on the trunk along the skin tension lines. In children lesions may involve the pubic, inguinal and axillary areas. Viral etiology is suspected [7, 14].
Hemangioma of infancy is a benign vascular neoplasm, the most common tumor of infancy. Usually not evident at birth, it grows rapidly during the first year of life, appearing as a flat or raised lesion, red or blue colored. Sometimes ulcerate and lead to infection. It may be associated with urogenital an anorectal malformations.
Zinc deficiency is a rare inherited (acrodermatitis enteropathica) or aquired disorder characterized by the inability of absorb sufficient zinc from diet. Skin findings include red, erosive plaques that may contain vesicles and pustules, simmetrically distributed in the perioral, acral, and perineal areas [7, 31].
1.4.1.3 Red Papules and Nodules
Red papules and nodules may be caused by folliculitis, HPV, Molluscum contagiosum, hidradenitis suppurativa, urethral prolapse, hemangioma of infancy, pityriasis rosea.
1.4.1.4 White Lesions
The most frequent white lesion in childhood is caused by lichen sclerosus. Other diseases are vitiligo, Molluscum contagiosum, and HPV.
Lichen sclerosus is a chronic autoimmune skin disease, in adulthood often associated with other autoimmune disease. Genetic and hormonal factors are involved also [12]. It affects all areas of the body in both sexes and all ages, but in children extragenital lesions are rare and average age of onset is 5 years [14, 31]. The key feature of lichen is a well-demarcated white plaque in a figure of eight distribution surrounding the vulva and perineal areas. Petechiae and ecchymoses may be present (Fig. 1.3). Skin is usually wrinkled (Fig. 1.4). The vulvar rash is extremely pruritic, and irritation, pain, dysuria, bleeding, constipation, and dyschezia may associate. In contrast, many cases are asymptomatic. Generally the diagnosis is made by clinical examination, therefore biopsy is not necessary. Symptoms usually improve at menarche. Many patients have relapses and remits over time. It has been reported that lichen sclerosus may increase the risk of vulvar squamous cell carcinoma. Treatment of choice, whose objective is to relief symptoms and avoid scars, is topical corticosteroids (clobetasol propionate 0.05% or betamethasone valerate 0.05%). Off-label treatment with topic tacrolimus or pimecrolimus may be an option as second-line treatment [20].
Fig. 1.3
Lichen sclerosus
Fig. 1.4
Lichen sclerosus
Lichen simplex chronicus may be present in all ages and may be distinguished in primary (arising from normal appearing skin) or secondary (superimposed on some other underlying dermatological disorders). It is characterized by the presence of itch-scratch cycle and clinically by a palpable thickening of the tissue and increased prominence of skin markings. Color of lesions may vary from white to skin colored or red [23].
Vitiligo is an aquired, autoimmune disorder due to an absence of epidermal melanocytes. Lesions are asymptomatic, sharply demarcated patches with complete loss of pigment, often involving periorificial areas in a symmetric manner [7].
1.4.1.5 Dark-Colored Lesions
Acanthosis nigricans, nevi, lentigines, HPV, and seborrheic keratosis may appear as dark-colored lesions.
Acanthosis nigricans is characterized by thickening and hyperpigmentation of the skin. Symptoms are absent. It usually involves the skin of axillae, posterior neck, groins, bell line, dorsal surface of fingers, mouth and around areolas. It is considered as markers of insulin resistance.
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