Treatment aims to improve symptoms
LS is usually diagnosed clinically (especially in children, as a biopsy is a great challenge to perform). However, when the clinical presentation is unclear, a skin biopsy will demonstrate the diagnostic findings of thinning of the epidermis, loss of rete pegs, hyperkeratosis, and dermal fibrosis with a T-lymphocyte-dominant inflammatory infiltrate.1,2,4,5
LS is a remitting and relapsing condition with no cure. The goals of treatment are to provide symptom relief and minimize scarring and atrophy,2 but it is unknown whether treatment reduces the risk of malignancy.9
First-line treatment for both genders and all ages is ultrapotent topical corticosteroids; clobetasol propionate 0.05% is most commonly used.1,6 Regimens vary, but the vast majority of patients improve within 3 months of once-daily treatment.4
For refractory LS, calcineurin inhibitors such as tacrolimus may be used. Although it has a black box warning regarding a potential cancer risk, long-term studies of children using tacrolimus for atopic dermatitis have not demonstrated an increased risk of malignancy.6,9 Because of a considerable adverse effect profile, oral retinoids are limited to refractory cases in adults.6 Surgery is reserved for scarring and adhesions.4
Follow-up plays an important role in management
Historically, it was believed that pediatric LS had an excellent prognosis, with patients achieving complete resolution after puberty.1,4 Recent findings have shown mixed results, with LS persisting in many patients beyond puberty.2,4 Therefore, regular follow-up is recommended every 6 to 12 months.
For uncomplicated LS, specialist follow-up is not indicated. Female patients should regularly conduct self-examinations and, at a minimum, undergo annual examinations by their primary care physician. Those who require specialist follow-up include patients with difficult-to-control symptoms, hypertrophic lesions, a history of SCCV or differentiated vulvar intraepithelial neoplasia (dVIN), or pathology showing possible dVIN.15
Our patient. We prescribed clobetasol propionate 0.05% ointment to be used once daily for 8 weeks. We stressed the importance of genital self-examinations using a mirror to monitor for any concerning changes such as skin thickening. We showed the patient and her mother photos of normal female genitalia to help normalize the genital exam, and taught the patient how to find her plaques in the mirror. We set expectations by emphasizing the chronic nature of LS and the likelihood of recurrence. We also encouraged HPV vaccination in the upcoming years to prevent both cervical cancer and HPV-related SCCV.
CORRESPONDENCE
Somya Abubucker, MD, University of Hawaii, 1356 Lusitana Street, 7th floor, Honolulu, HI 96813; sabubuck@hawaii.edu.