Purpura Fulminans

A severe, rapidly fatal reaction occurring most commonly in children following an infectious illness. It is characterized by large, rapidly spreading skin hemorrhages, fever, or shock. Purpura fulminans often accompanies or is triggered by DISSEMINATED INTRAVASCULAR COAGULATION.

Also Known As:

Networked: 289 relevant articles (7 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sepsis (Septicemia)
2.	Septic Shock (Toxic Shock Syndrome)
3.	Purpura
4.	Adrenal Insufficiency (Adrenal Gland Hypofunction)
5.	Protein C Deficiency

Experts

1.	Schlievert, Patrick M: 3 articles (01/2014 - 04/2005)
2.	Leclerc, F: 3 articles (11/2001 - 09/2000)
3.	Fourier, C: 3 articles (11/2001 - 09/2000)
4.	Esmon, C T: 3 articles (08/2001 - 05/2001)
5.	Alhenc Gelas, Martine: 2 articles (02/2015 - 11/2014)
6.	Knöfler, Ralf: 2 articles (02/2015 - 11/2014)
7.	Nowak-Göttl, Ulrike: 2 articles (02/2015 - 11/2014)
8.	Zieger, Barbara: 2 articles (02/2015 - 11/2014)
9.	Junker, Ralf: 2 articles (02/2015 - 11/2014)
10.	Holzhauer, Susanne: 2 articles (02/2015 - 11/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Purpura Fulminans:

1.	Protein CIBA 05/01/2006 - "The aim of this study was to document the effects of supplementation with a plasma-derived protein C concentrate in adult patients with infectious purpura fulminans. " 05/01/2004 - "Treatment with protein C concentrate is followed by an improvement of the coagulopathy and is safe in children with purpura fulminans; however, a large trial involving a high dose is required to determine its effect on mortality and morbidity. " 07/01/2003 - "Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic study." 12/01/2000 - "An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia." 11/01/2015 - "Purpura fulminans (PF) may be the presenting symptom in a patient with protein C (PC) deficiency. "
2.	Antithrombin IIIIBA 10/01/1994 - "Meningococcal purpura fulminans treated with antithrombin III concentrate: what is the optimal replacement therapy?" 12/01/2002 - "Significant advances have been made in understanding the physiopathology of the meningococcal purpura fulminans especially about the deleterious role of the deficiency of the protein C and the antithrombin III. It is too soon to advance that the prognosis of these diseases has been improved by these new therapeutic approaches but the results of preliminary clinical studies are encouraging. " 01/01/1990 - "Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates." 12/01/1997 - "Personal experience is reported on the efficacy of heparin in combination with antithrombin III in the treatment of purpura fulminans in two preterm neonates who did not have inherited deficiency of protein C or S and developed the disease possibly following prolonged labor (36 hours) in the first case, and maternal neoplasia, in the second. " 10/01/2013 - "Purpura fulminans is a life-threatening hematologic emergency characterized by extensive skin necrosis with hemorrhagic infarction, hypotension and gangrene which may arise from severe sepsis; mostly gram negative sepsis though also associated with some gram positive organisms, similarly viral infections like varicella and measles have been implicated; it may also arise from congenital deficiency of the anticoagulants protein C, S, and antithrombin III; and it may also be idiopathic. "
3.	drotrecogin alfa activated (Xigris)FDA Link 02/01/2003 - "Drotrecogin alfa (activated) for the treatment of meningococcal purpura fulminans." 08/01/2005 - "We report data from adult and pediatric patients with severe sepsis from studies evaluating drotrecogin alfa (activated) (DrotAA) and presenting with purpura fulminans (PF), meningitis (MEN), or meningococcal disease (MD) (PF/MEN/MD). " 08/01/2005 - "Drotrecogin alfa (activated) in patients with severe sepsis presenting with purpura fulminans, meningitis, or meningococcal disease: a retrospective analysis of patients enrolled in recent clinical studies." 05/01/2005 - "Treatment with protein C concentrate or recombinant activated protein C (Drotrecogin alfa, activated) has been shown to be beneficial in sepsis-induced purpura fulminans. " 02/01/2004 - "Based on case reports, treatment with drotrecogin alfa (activated) or substitution of protein C have been recommended for adjuvant treatment of sepsis-related purpura fulminans."
4.	Heparin (Liquaemin)FDA LinkGeneric 08/01/1970 - "[Value of heparin in the treatment of meningococcal purpura fulminans. " 06/01/1968 - "[Successful treatment with heparin of Schönlein-Henoch disease with a course of purpura fulminans]." 08/01/1966 - "Heparin therapy of purpura fulminans." 01/01/1997 - "In one neonate purpura fulminans occurred, and heparin-induced thrombocytopenia type II was additionally documented. " 10/01/1994 - "This small, retrospective series suggests that heparin therapy may limit digit and extremity necrosis when used early and in therapeutic doses in meningococcal purpura fulminans. "
5.	SteroidsIBA 08/01/1975 - "She was successfully treated with adrenal steroids and heparin for her purpura fulminans. " 10/07/1967 - "Purpura fulminans complicating varicella: recovery with low molecular weight dextran and steroids." 11/01/2007 - "Although it may be prudent to provide stress-dose steroids to children with septic shock who are clinically at risk for adrenal insufficiency (chronic or recent steroid use, purpura fulminans, etomidate or ketoconazole administration, hypothalamic, pituitary, adrenal disease), the safety and efficacy of stress-dose steroids as general adjunctive therapy for pediatric septic shock have not been established. "
6.	CortisoneIBA 12/01/1959 - "[Purpura fulminans and cortisone]." 02/13/1955 - "[Two cases of superacute meningococcal purpura resembling clinically the purpura fulminans, treated by cortisone and cured]." 02/13/1955 - "[Cortisone in severe infections; two observations: one of meningococcal purpura fulminans, the other of staphylococcal pachypleuritis in a young infant]."
7.	Ketoconazole (Nizoral)FDA LinkGeneric 11/01/2007 - "Although it may be prudent to provide stress-dose steroids to children with septic shock who are clinically at risk for adrenal insufficiency (chronic or recent steroid use, purpura fulminans, etomidate or ketoconazole administration, hypothalamic, pituitary, adrenal disease), the safety and efficacy of stress-dose steroids as general adjunctive therapy for pediatric septic shock have not been established. "
8.	EtomidateFDA LinkGeneric 11/01/2007 - "Although it may be prudent to provide stress-dose steroids to children with septic shock who are clinically at risk for adrenal insufficiency (chronic or recent steroid use, purpura fulminans, etomidate or ketoconazole administration, hypothalamic, pituitary, adrenal disease), the safety and efficacy of stress-dose steroids as general adjunctive therapy for pediatric septic shock have not been established. "
9.	Dacarbazine (DIC)FDA LinkGeneric 06/01/2008 - "Several case series demonstrated beneficial effects in infectious purpura fulminans and DIC, but no controlled studies for these indications exist. " 03/01/1996 - "Major complications were: DIC (28%), ARDS (26%), purpura fulminans (21%). " 09/01/1992 - "One infant was treated every 48 hr for 2 years without significant purpura fulminans or DIC complications. " 10/01/1990 - "Although the pathogenesis is not fully understood, the DIC in purpura fulminans appears to involve the skin selectively. " 10/01/1990 - "Purpura fulminans is at least in part a cutaneous manifestation of the syndrome of systemic DIC. "
10.	Adrenal Cortex Hormones (Corticosteroids)IBA 03/01/2014 - "A total of 1981 IMD cases were identified during the 8-year study, 805 cases (712 [88.5%] bacterial meningitis and 93 [11.5%] purpura fulminans) had steroid treatment data (33.8% received corticosteroids). "

Therapies and Procedures

1.	Hyperbaric Oxygenation 09/01/2000 - "Improvement in limb perfusion was achieved after hyperbaric oxygenation in patients with purpura fulminans caused by different pathogens. " 05/01/2006 - "Hyperbaric oxygen therapy for purpura fulminans-comment." 07/01/2005 - "Hyperbaric oxygen therapy for purpura fulminans: comment." 03/01/2003 - "Currently wound treatment options of amputation stumps due to purpura fulminans include healing by secondary intention from wound debridement, split-thickness skin grafting, tissue and muscle flaps, plantar skin free transfer, skin expansion, artificial skin, and hyperbaric oxygen therapy. "
2.	Amputation 07/01/2012 - "[Purpura fulminans: functional results in two paediatric patients after suffering multiple amputations]." 01/01/2011 - "Although this disease is rare, infants with suspected idiopathic purpura fulminans should be rapidly diagnosed and immediately anti-coagulated to prevent adverse catastrophic outcomes such as amputation and significant developmental delay. " 04/01/2009 - "Inpatient rehabilitation of survivors of purpura fulminans with multiple limb amputations: a case series." 09/01/2006 - "The authors report a case of nasal reconstruction in a 5-year-old boy who had undergone subtotal amputation of the nose 8 months before in the context of meningococcus-induced purpura fulminans. " 09/01/2006 - "Suitable age for nasal reconstruction after subtotal amputation in a child, with respect to a case involving purpura fulminans."
3.	Catheters 11/01/2012 - "Catheter-directed thrombolysis in a patient with purpura fulminans." 03/28/1987 - "In order to investigate the haemodynamic changes involved at the very beginning of meningococcal purpura fulminans, a prospective study was conducted in 10 children in whom a Swan-Ganz catheter was introduced on admission into the pulmonary artery. " 06/01/2006 - "Thrombotic complications range from arterial and venous catheter thrombosis to purpura fulminans. " 11/01/2013 - "She developed purpura fulminans at birth and a catheter-related venous thrombosis complicated by massive pulmonary embolism at the sixth day of life. "
4.	Transplants (Transplant) 01/01/1988 - "[Purpura fulminans in a newborn infant subsequently treated with free dermal-epidermal transplants]." 09/01/2001 - "The most severe presentation, called purpura fulminans, has a death rate of 20-25%; 5 to 20% of the survivors need skin grafts and/or amputations. " 08/01/1994 - "Thirteen (72%) of the 18 patients with purpura fulminans needed one or more surgeries including skin grafts, local debridement, microvascular flaps or amputations. " 01/01/2010 - "Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. "
5.	Splenectomy 01/01/2008 - "A devastating case of purpura fulminans in a young adult seven years after a splenectomy: a case report." 04/10/2007 - "[Saved case of purpura fulminans in pneumococcal sepsis after splenectomy]." 09/07/1970 - "Purpura fulminans in a child with pneumococcal septicemia two years after splenectomy." 11/01/1989 - "Purpura fulminans associated with overwhelming sepsis and disseminated intravascular coagulation following splenectomy."