An unusually severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) with a fatal outcome in an 82-year-old woman is reported. After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. Skin biopsies showed the typical histopathological changes of PLEVA. Early recognition of this severe variant of PLEVA is important, since the fulminating course can lead to death.