The pathogenesis of acne fulminans remains obscure despite extensive investigations discussed in this review, but evidence points toward an immune mediated phenomenon (eg, hypergammaglobulinemia, depressed complement, circulating immune complexes, hematuria, lytic bone lesions, or inflammatory myositis). Whether acne fulminans begins as an autoimmune process de novo, or whether the process is somehow triggered by minimal inflammation in susceptible individuals remains to be elucidated. Acne fulminans is a rare, disfiguring, and disabling disease. It is associated with many problems, including abnormalities revealed in the laboratory or on roentgenogram. We have reviewed past and present therapies. In conclusion, we emphasize the importance of early diagnosis and treatment to decrease the significant morbidity of acne fulminans.