Papulosis atrophicans maligna associated with extensive cerebral vasculitis of small arteries and arterioles, encephalomalacia, and myelomalacia without other visceral involvement is reported. The predominant involvement of the cerebral gray matter, spinal cord white matter, cranial nerves, and spinal cord nerve roots is described. The similarity of this type of necrotizing vasculitis with thrombosis to polyarteritis nodosa is observed. The previously reported articles are recorded in the bibliography.