Journal of Environmental Pathology, Toxicology and Oncology

ISSN for PRINT: 0731-8898

For Online Access

Best Paper Award Selection - Editorial Board Site

2008, Volume27

Issue 3

89 pages

Issue price - $202.00

Richard F. Edlich
Biomedical Engineering and Emergency Medicine, University of Virginia Health System, Trauma Specialists LLP, Legacy Verify Level I Shock Trauma Center for Pediatrics and Adults, Legacy Emanuel Hospital, Portland, OR, USA

Catherine L. Cross
Legacy Emanuel Hospital, Portland, OR, USA

Jill J. Dahlstrom
Legacy Verified Level I Shock Trauma Center, Legacy Emanuel Hospital, Portland, OR, USA

William B. Long III
Trauma Specialists, LLP, Legacy Verified Level I Shock Trauma Center for Pediatrics and Adults, Legacy Emmanuel Hospital Portland, OR, USA

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The purpose of this collective review is to provide modern concepts on the diagnosis and treatment of neonatal purpura fulminans, idiopathic purpura fulminans, and acute infectious purpura fulminans. There are three forms of this disease that are classified by the triggering mechanisms. First, neonatal purpura fulminans is associated with a hereditary deficiency of the natural anticoagulants Protein C and Protein S as well as Antithrombin III. Idiopathic purpura fulminans usually follows an initiating febrile illness that manifests with rapidly progressive purpura. Deficiency of Protein S is considered to be central to the pathogenesis of this form of the disease. The third and most common type of purpura fulminans is acute infectious purpura fulminans. The mortality rate has decreased with better treatment of secondary infections, supportive care, and new treatments, but it remains a disabling condition often requiring major amputations.

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