We describe a 75-year-old woman with a chronic, blistering eruption on the left leg whose clinical and immunopathologic features were consistent with a diagnosis of localized pretibial pemphigoid. After a disease-free interval of 5 years she developed a generalized prurigo nodularis-like eruption. Immunofluorescence studies revealed deposition of IgG and C3 along the dermoepidermal junction, and circulating autoantibodies against the dermoepidermal junction were demonstrated. Indirect immunoelectron microscopic examination of saponin-treated skin samples showed deposits of immunoreactants over the intracellular part of the hemidesmosomes. By Western immunoblotting the 230 kd bullous pemphigoid antigen was recognized by circulating autoantibodies. Thus our patient had two unusual clinical variants of bullous pemphigoid: localized pretibial pemphigoid and pemphigoid nodularis.