Localized Childhood Vulval Pemphigoid Treated with Tacrolimus Ointment

Abstract

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.

British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012

“…In a case of mild vesicular pemphigoid, reduction of oral steroids and use of potent topical steroids led to new vesicles; these ceased with the substitution of topical steroids with tacrolimus ointment 0·1% (twice daily for 2 weeks), allowing withdrawal of the oral steroids 88 . In other reports, topical tacrolimus has been used as the sole agent for localized disease and also in vulval pemphigoid in a child 89–91 . The use of topical tacrolimus is limited by local irritation and its price compared with topical steroids.…”

The management of mucous membrane pemphigoid and pemphigus

“…Significant response to the use of topical tacrolimus (Protopic®) twice daily has been reported for the treatment of MMP (73–76). We have found topical corticosteroids to be more efficacious.…”

Childhood cicatricial pemphigoid confined to the vulva.

“…Topical tacrolimus has been reported to be effective in treating childhood vulval CP within 3 months of application. It was well tolerated and no relapse was seen on cessation of therapy 5 …”

“…Nine cases of vulval CP in children have been reported in the literature, only two of which had solely vulval involvement 1–5 . The other childhood cases had associated oral involvement or more widespread disease.…”