Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.