Vulvo-Perineal Localization of Dermatologic Disorders

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.