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: 401 relevant articles (11 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sepsis (Septicemia)
2.	Septic Shock (Toxic Shock Syndrome)
3.	Meningococcal Infections
4.	Purpura
5.	Neoplasms (Cancer)

Experts

1.	Kang, Dongchon: 4 articles (11/2021 - 06/2009)
2.	Schlievert, Patrick M: 4 articles (01/2021 - 04/2005)
3.	Contou, Damien: 3 articles (01/2022 - 12/2019)
4.	Ohga, Shouichi: 3 articles (11/2021 - 06/2009)
5.	Levin, M: 3 articles (09/2002 - 09/2000)
6.	Esmon, C T: 3 articles (08/2001 - 05/2001)
7.	Guerreschi, P: 2 articles (12/2021 - 10/2016)
8.	Hotta, Taeko: 2 articles (11/2021 - 04/2014)
9.	Ishiguro, Akira: 2 articles (11/2021 - 04/2018)
10.	Ishimura, Masataka: 2 articles (11/2021 - 06/2009)

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. " 02/01/2022 - "Purpura fulminans (PF) is a hematological emergency that can be caused by severe congenital protein C (PC) deficiency. " 01/01/2021 - "Compound heterozygous or homozygous variants in the Protein C gene (PROC) lead to severe deficiency of protein C and affected neonates typically present shortly after birth with purpura fulminans. " 12/01/2020 - "Compound heterozygous protein C variants undetectable by common laboratory testing causing purpura fulminans after the neonatal period."
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.	SteroidsIBA 08/01/1975 - "She was successfully treated with adrenal steroids and heparin for her purpura fulminans. " 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). " 01/01/2023 - "A diagnosis of purpura fulminans was made, and steroids, therapeutic plasma exchange and empiric therapy, including antibiotic and anticoagulation therapy, were initiated immediately. " 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. "
4.	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."
5.	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 - "The role of heparin in the prevention of extremity and digit necrosis in meningococcal purpura fulminans."
6.	CortisoneFDA Link 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.	CoagulantsIBA 05/23/2011 - "Another novel feature of our patient's presentation is that discontinuation of anti-coagulation has invariably led to recurrence and formation of new lesions, which is unexpected in idiopathic purpura fulminans because clearance of autoimmune factors should be followed by restoration of anti-coagulant function. "
10.	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. " 05/19/2022 - "Her hospital course rapidly progressed to multiorgan dysfunction, DIC and purpura fulminans. " 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. "

Therapies and Procedures

1.	Therapeutics 01/01/1993 - "A 32-year-old pregnant woman developed meningococcemia associated purpura fulminans and quickly improved with therapy. " 04/01/2020 - "[Treatment failure after postinfectious purpura fulminans despite interdisciplinary surgical and intensive medical therapy]." 06/01/2006 - "Infectious purpura fulminans is associated with high mortality and morbidity despite standard antimicrobial therapy. " 09/01/2004 - "Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. " 10/10/1991 - "[Purpura fulminans--aspects of etiopathogenesis, clinical aspects, diagnosis and therapy]."
2.	Hyperbaric Oxygenation 09/01/2000 - "Improvement in limb perfusion was achieved after hyperbaric oxygenation in patients with purpura fulminans caused by different pathogens. " 01/01/2023 - "Use of hyperbaric oxygen therapy of purpura fulminans in an extremely low birth weight preterm: A case report." 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. "
3.	Free Tissue Flaps 12/01/2021 - "We examined the efficacy of free flaps for lower limb salvage in infectious purpura fulminans survivors by evaluating surgical management, walking ability and quality of life. " 12/01/2021 - "Use of the free flap in patients with infectious purpura fulminans, after multidisciplinary reflection, is an appropriate procedure that preserves limb length. " 12/01/2021 - "Adult purpura fulminans survivors who received a free flap for lower limb salvage were included. " 12/01/2021 - "Free flap for lower limb salvage in infectious purpura fulminans." 09/01/2006 - "Salvaging limbs in cases of severe purpura fulminans: advantages of free flaps."
4.	Surgical Amputation (Amputations) 12/01/2021 - "Infectious purpura fulminans is a disabling disease often leading to amputations. " 12/01/2020 - "One patient with QTT died, and two (8%) additional patients with QTT developed purpura fulminans requiring digital amputation. " 01/01/2020 - "Fulminant Neisseria meningitidis septicaemia with purpura fulminans requiring limb amputation." 01/01/2019 - "She developed purpura fulminans (PF) ultimately resulting in amputation of both lower extremities. " 07/30/2018 - "This included cardiorespiratory and acute hepatorenal failure and purpura fulminans that eventually necessitated four-limb amputation. "
5.	Splenectomy 02/26/2020 - "Half of pneumococcal purpura fulminans episodes occurred more than 20 years after splenectomy. " 01/01/2008 - "A devastating case of purpura fulminans in a young adult seven years after a splenectomy: a case report." 01/01/2022 - "We report here what we believe to be the first case of overwhelming post-splenectomy infection (OPSI) with purpura fulminans due to Enterococcus cecorum in a 51-year-old man. " 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."