Vulvar lichen sclerosus in a prepubertal girl

A 7-year-old girl consulted the pediatrician for itching and ecchymosis in the perineum, denying any other symptoms. Perineum examination revealed skin discoloration in the area involving the vulva and perianal region, with areas of ecchymosis (Fig. 1a). The clinical diagnosis of lichen sclerosus (LS) was established, and the patient initiated treatment with topical 0.05% fluticasone propionate ointment once a day for 2 weeks, then on alternate days for an additional 2 weeks. After 4 weeks of treatment the patient’s skin lesions and symptoms had subsided completely (Fig. 1b). At re-evaluation 6 months after treatment, the patient remained asymptomatic. LS is a chronic inflammatory disease of the skin, predominantly affecting the anogenital region. It is more common in female patients (10:1), with two peaks of incidence: prepubertal or postmenopausal [1]. The pathogenesis is unknown, but autoimmune mechanisms seem to be involved [2]. LS may be asymptomatic, but usually manifests as pruritus, pain, burning sensation in the perineum, constipation or urinary symptoms. The presence of ecchymosis and bleeding lesions may raise the suspicion of sexual abuse. The pathognomonic characteristic is the presence of a depigmented lesion in the shape of a ‘figure 8’, involving the anogenital region. The chronicity of inflammation can result in scarring, with a disruption of the normal anatomical structures, sensory abnormalities, psychosexual problems and progression to squamous cell carcinoma, specially in adults. A correct diagnosis and early aggressive treatment are crucial to prevent complications. Topical steroids are the mainstay of treatment. Potent or very potent topical steroids should be applied once a day for 6–12 weeks [1, 2], with progressive weaning. Patients should be advised to use emollient as a barrier and as soap substitute and avoid mechanical triggers. LS usually improves at puberty, but may persist into adulthood, a regular follow-up at least until puberty should be maintained [2].