Epidermolysis bullosa aimplex is a dominantly inherited syndrome characterized by the dissolution of the basal epidermal cells with mechanical stress. A 23 year-old man developed recurrent bullous eruptions with pain on hands, feet and face since childhood, which healed spontaneously without scarring. Grandmother of his father's side, father, aunt of his father's side and two of his three brothers had suffered similar skin lesions. Light and electron microscopy showed degeneration and bulla formation in the basal cell layer of epidermis and intact basement membrane attached to dermis.