Of 144 patients with bullous or cicatricial pemphigoid, nine with localized pemphigoid were seen at the Mayo Clinic between 1968 and 1975. In two patients the disease had become generalized before presentation, and in one it had evolved into bullous pemphigoid, the only case with positive indirect immunofluorescence. One additional patient showed mucosal lesions. Direct immunofluorescence was performed in five patients and showed linear deposition of C3 and fibrin but not immunoglobulins in three; in two patients immunofluorescence was negative. Localized pemphigoid can be divided into two types: that with scarring plaque-like lesions usually occurring on the head and neck predominantly in males, and that with localized bullous lesions usually occurring on the lower part of the legs of females.