Vulvo-Perineal Localization of Dermatologic Disorders
Systemic disorders may be localized in the vulvo-perineal region. Here, we will only emphasize those in which this localization suggests the diagnosis or is of particular importance.

In Langherans’ cell histiocytosis (

fig. 7), lesions initially are erythematous, purpuric papules and they then become scaly, crusted and sometimes confluent. The preferential localizations are the scalp, the retroauricular folds, and the trunk. The napkin area may be involved, such as the inguinal folds and the perianal region. Involvement of the female genital tract may occur, including the vulva and vagina. In case of persistent inguinal fold involvement in a child, clinical examination should extend to other potential localizations of this disease. If purpuric or crusted papules are present, a histologic examination has to be performed.

Fig. 7. Histiocytosis.

Acrodermatitis enteropathica (

fig. 8) is a biochemical disorder of zinc metabolism. Cutaneous manifestations are localized in perioral and perianal regions. The clinical picture is a vesiculobullous, pustular hyperkeratotic dermatitis with failure to thrive, diarrhea, apathy, alopecia and fine brittle hair. Differential diagnosis should include biotinidase deficiency and some types of organoacidemia.

Lesions of inflammatory bowel disease (

fig. 9 - Fig. 9. Aphtous lesion in Behçet’s disease.): Perianal involvement is quite common in Crohn’s disease, and perianal skin tags, anal and vaginal fissures, abscesses and fistulas are frequent. Isolated swelling of the labia majora may be present for months, before the appearance of other dermatological or gastrointestinal symptoms. Cutaneous biopsies may help the diagnosis by revealing the characteristic granulomatous features. Genital and anal aphthae may occur in Behçet’s and Crohn’s disease. Given the lack of a pathognomonic laboratory test or histologic findings, clinical criteria must be used to make the diagnosis of Behçet’s disease. Genital aphthae usually occur on the vulva and are painful; in the initial presentation, herpes virus must be considered in the differential diagnosis. Several patients with neonatal Behçet’s disease have been reported, all of whose mothers were affected with the same disease. Treatment depends on severity, and topical and intralesional corticosteroids can be used for genital aphthae, as can applications of viscous lidocaine. Other treatments include colchicine, dapsone, thalidomide and topical tacrolimus.

In Kawasaki’s disease (

fig. 10- Perineal desquamation in Kawasaki disease), an erythematous, desquamating perineal rash may occur in the second week of symptom onset, almost at the same time as palmoplantar desquamation. This is of importance for the diagnosis of this syndrome, as early diagnosis and treatment is the best way to prevent coronary aneurysms.

Any bullous disease may be localized in the perineal area. In the newborn, a sample for bacterial and viral studies must be taken. Staphylococcal and viral diseases require rapid treatment. Genetic bullous disorders are mainly epidermolysis bullosa, congenital and incontinentia pigmenti (

Fig. 11. Incontinentia pigmenti: hyperpigmentation in Blaschko linear distribution.) (with distribution of the vesiculobullar lesions along Blaschko’s lines). In childhood, autoimmune bullous disorders are uncommon. IgA dermatitis includes blisters in the perineal region and erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis may involve the perineal area. Coexisting erosions of the other mucous membranes (eyes, mouth) and cutaneous rash help the diagnosis. In case of involvement of the vaginal introitus, post-inflammatory stenosis can occur; local care several times a day is needed to prevent this.

Many dermatoses may involve the perineal area in girls. The age at which lesions occur is very important clue for the diagnosis. In the case of congenital lesions, we should first consider infectious, malformative and genetic diseases. The most common problem of diaper-wearing infants is primary irritant dermatitis due to occlusion and friction; in cases of persistence, rare diagnoses such as acrodermatitis enteropathica or histiocytosis should be considered. It is important to distinguish between an isolated vulvitis (which is very common) and vulvo-vaginitis. Lichen sclerosus seems to be quite a frequent pathology in girls and diagnosis is often delayed. Tumors are rare in childhood but, when suspected, diagnosis is urgent to treat them quickly.

Cutaneous and mucosal cultures are needed to adequately manage genital diseases. In atypical cases where sexual abuse is suspected, we recommend a multidisciplinary approach.

References

Dominique Hamel-Teillac
Service de Dermatologie, Hôpital Necker-Enfants Malades