Localized Pemphigus Foliaceus: Diverse Presentations, Treatment, and Review of the Literature

The clinical presentation of localized pemphigus foliaceus (PF) often involves photo exposed areas. We describe five cases of localized PF, two of which were rare locations for the disease in non-photo exposed areas, namely the genitalia and back. Patients were treated with topical corticosteroids and calcineurin inhibitors as well as systemic treatment with corticosteroids and dapsone. Each patient responded to treatment, with two achieving remission. No relapses occurred in any of these cases over a mean follow-up time of 3.7 years. A review of the English literature using MEDLINE(®) yielded 18 reported cases of localized PF. Most occurred in photo exposed areas such as the nose, cheeks, scalp, and other areas of the face. Two patients progressed to generalized involvement without treatment. Treatment regimens had much variation and included both topical and systemic agents. Localized PF is rare, and our findings suggest it may be controlled with topical therapy and systemic dapsone.