Electron microscopic findings are presented in a patient with localized cicatricial pemphigoid (Brunsting-Perry). These findings revealed subepidermal separation below the basal lamina. The basal lamina and anchoring fibrils were well preserved and attached to the intact epidermis to form the roof of the blister. These findings support the concept that localized cicatricial pemphigoid, cicatricial pemphigoid, and disseminated cicatricial pemphigoid are closely related diseases and may explain the occurrence of scar formation in localized cicatricial pemphigoid. A review of 42 cases reported in the world literature is included, with emphasis on clinical, histologic, and immunofluorescence microscopic findings.