Localized childhood vulval pemphigoid treated with tacrolimus ointment

Background: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. Observation: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. Conclusion: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.