Childhood vulvar lichen sclerosus. The course after puberty.

OBJECTIVE

To identify girls with vulvar lichen sclerosus (LS) and to follow them through puberty, documenting the course of the disease.

STUDY DESIGN

Twenty-one postpubertal girls were identified from a cohort of 75 girls with LS presenting prepubertally and attending a pediatric vulvar clinic. Details of current symptoms, findings on examination and treatment needs were recorded. A database of 263 women with LS was reviewed for onset of LS premenarche.

RESULTS

Of the 21 postpubertal girls, 16 reported an improvement in symptoms, but 11 stated that they still experienced occasional pruritus, requiring intermittent topical steroid application. Although the disorder appeared less active in most cases, definite physical signs persisted in 16 patients (75%); in 5 patients no physical signs of the disease remained. Of 251 postmenopausal women with LS, < 5 could recall symptoms in childhood. Of 12 young adult premenopausal patients with vulvar LS, 4 could recall symptoms in childhood. One of these, a 32-year-old with well-documented LS in childhood resolving at puberty, presented with and died of vulvar squamous cell carcinoma (SCC).

CONCLUSION

Patients should be aware that LS may improve symptomatically but usually does not entirely resolve at puberty and that the disease in women may be associated with development of vulvar SCC. Ideally, long-term follow-up should be the standard of care.