ABSTRACT
ABSTRACT: Purpura fulminans can result in significant full-thickness wounds, posing a challenge in the pediatric population, given the paucity of donor sites for reconstruction. The authors present the case of an 11-month-old patient for whom a split-thickness skin allograft (TheraSkin) was successfully implemented as a temporizing measure for a large leg wound.
Subject(s)
Lower Extremity/physiopathology , Necrosis/surgery , Plastic Surgery Procedures/methods , Purpura Fulminans/complications , Child , Female , Humans , Necrosis/etiology , Pediatrics/methods , Purpura Fulminans/physiopathology , Purpura Fulminans/surgery , Skin Transplantation/methods , Texas , Wound Healing/physiologySubject(s)
Purpura Fulminans/mortality , Child , Child, Preschool , Cohort Studies , Female , France , Humans , Male , Multivariate Analysis , Prospective Studies , Purpura Fulminans/physiopathology , Risk FactorsABSTRACT
Scrub typhus and Queensland tick typhus (QTT)-rickettsial infections endemic to tropical Australia-can cause life-threatening disease. This retrospective study examined the clinical course of all patients with laboratory-confirmed scrub typhus or QTT admitted to the intensive care unit (ICU) of a tertiary referral hospital in tropical Australia between 1997 and 2019. Of the 22 patients, 13 had scrub typhus and nine had QTT. The patients' median (interquartile range [IQR]) age was 50 (38-67) years; 14/22 (64%) had no comorbidity. Patients presented a median (IQR) of seven (5-10) days after symptom onset. Median (IQR) Acute Physiology and Chronic Health Evaluation II scores were 13 (9-17) for scrub typhus and 13 (10-15) for QTT cases (P = 0.61). Following hospital admission, the median (IQR) time to ICU admission was five (2-19) hours. The median (IQR, range) length of ICU stay was 4.4 (2.9-15.9, 0.8-33.8) days. Multi-organ support was required in 11/22 (50%), 5/22 (22%) required only vasopressor support, 2/22 (9%) required only invasive ventilation, and 4/22 (18%) were admitted for monitoring. Patients were ventilated using protective lung strategies, and fluid management was conservative. Standard vasopressors were used, indications for renal replacement therapy were conventional, and blood product usage was restrictive; 9/22 (41%) received corticosteroids. One patient with QTT died, and two (8%) additional patients with QTT developed purpura fulminans requiring digital amputation. Death or permanent disability occurred in 3/9 (33%) QTT and 0/13 scrub typhus cases (P = 0.055). Queensland tick typhus and scrub typhus can cause multi-organ failure requiring ICU care in otherwise well individuals. Queensland tick typhus appears to have a more severe clinical phenotype than previously believed.
Subject(s)
Acute Kidney Injury/physiopathology , Intensive Care Units , Respiratory Distress Syndrome/physiopathology , Scrub Typhus/physiopathology , Spotted Fever Group Rickettsiosis/physiopathology , APACHE , Acute Kidney Injury/etiology , Acute Kidney Injury/therapy , Adrenal Cortex Hormones/therapeutic use , Adult , Aged , Amputation, Surgical , Anti-Bacterial Agents/therapeutic use , Azithromycin/therapeutic use , Cohort Studies , Doxycycline/therapeutic use , Female , Fluid Therapy/methods , Hospitalization , Humans , Hypotension/etiology , Hypotension/physiopathology , Hypotension/therapy , Hypoxia/etiology , Hypoxia/physiopathology , Hypoxia/therapy , Length of Stay , Male , Middle Aged , Multiple Organ Failure/etiology , Multiple Organ Failure/physiopathology , Multiple Organ Failure/therapy , Organ Dysfunction Scores , Purpura Fulminans/etiology , Purpura Fulminans/physiopathology , Queensland/epidemiology , Renal Replacement Therapy/methods , Respiration, Artificial/methods , Respiratory Distress Syndrome/etiology , Respiratory Distress Syndrome/therapy , Retrospective Studies , Scrub Typhus/complications , Scrub Typhus/therapy , Spotted Fever Group Rickettsiosis/complications , Spotted Fever Group Rickettsiosis/therapy , Tertiary Care Centers , Vasoconstrictor Agents/therapeutic use , Young AdultSubject(s)
Anti-Bacterial Agents/administration & dosage , Debridement/methods , Gangrene , Purpura Fulminans , Biopsy/methods , Disseminated Intravascular Coagulation , Early Diagnosis , Female , Gangrene/etiology , Gangrene/surgery , Humans , Middle Aged , Purpura Fulminans/blood , Purpura Fulminans/diagnosis , Purpura Fulminans/physiopathology , Purpura Fulminans/therapy , Skin Ulcer/etiology , Skin Ulcer/pathology , Time-to-TreatmentABSTRACT
Protein C (PC) deficiency is a heritable or acquired risk factor for thrombophilia, with presentations varying from asymptomatic to venous thromboembolism to neonatal purpura fulminans, a life-threatening disorder. Hereditary PC deficiency is caused by mutation in the PC (PROC) gene located on chromosome 2q14.3. Heterozygous and acquired PC deficiencies are more common than homozygous deficiency. The recommended initial laboratory test measures PC activity using either clot-based or chromogenic methods. There are numerous potential interferences in PC activity testing that may result in either false-positive (falsely low activity) or false-negative (falsely normal or elevated activity) results. In the present review, we discuss common clinical presentations; laboratory testing, with a focus on potential assay interferences; treatment options; and prognosis in patients with PC deficiency.
Subject(s)
Protein C Deficiency/diagnosis , Purpura Fulminans/etiology , Thrombophilia/etiology , Venous Thromboembolism/etiology , Blood Coagulation Tests , Humans , Mutation , Protein C Deficiency/complications , Protein C Deficiency/physiopathology , Protein C Deficiency/therapy , Purpura Fulminans/physiopathology , Purpura Fulminans/therapy , Thrombophilia/physiopathology , Thrombophilia/therapy , Venous Thromboembolism/physiopathology , Venous Thromboembolism/therapySubject(s)
Heart Transplantation/adverse effects , Purpura Fulminans/diagnosis , Adult , Escherichia coli/drug effects , Escherichia coli/pathogenicity , Extracorporeal Membrane Oxygenation/methods , Heart Transplantation/methods , Humans , Male , Multiple Myeloma/complications , Multiple Myeloma/surgery , Purpura Fulminans/physiopathology , Shock, Cardiogenic/etiology , Shock, Cardiogenic/physiopathologyABSTRACT
We report a dramatic case of meningococcal sepsis manifesting as purpura fulminans in an elderly diabetic woman. Hemodynamic instability and severe bilateral cutaneous lesions involving her hands and feet developed rapidly. Specific antibiotic therapy and the administration of inotropic and vasopressor drugs were initiated. The severity and extension of the cutaneous lesions (attributed to purpura fulminans) worsened because of the need for vasoconstrictors for the treatment of septic shock. Bilateral transmetatarsal and metacarpal amputations were required to stabilize the patient.
Subject(s)
Meningococcal Infections/diagnosis , Purpura Fulminans/diagnosis , Sepsis/diagnosis , Aged , Amputation, Surgical , Diabetes Mellitus/physiopathology , Female , Humans , Meningococcal Infections/physiopathology , Meningococcal Infections/therapy , Purpura Fulminans/physiopathology , Sepsis/physiopathology , Sepsis/therapy , Severity of Illness IndexABSTRACT
Meningococcal sepsis and purpura fulminans is a rare but highly lethal disease process that requires a multidisciplinary team of experts to optimise morbidity and mortality outcomes due to the breadth of complications of the disease. The surgical perspective involves the critical care management which utilises all currently available measured outcomes of critical care management as well as experimental therapies. Limb loss is common, and is reflective of the high incidence of compartment syndrome compounded by the significant soft tissue loss secondary to purpura and limb ischaemia, presumptively due to digital microemboli. A multidisciplinary approach involving current standards in critical care and early surgical evaluation are important in improving patient outcomes and limb salvage.
Subject(s)
Bacteremia/surgery , Critical Care/methods , Limb Salvage/methods , Meningococcal Infections/surgery , Purpura Fulminans/surgery , Skin/blood supply , Adult , Amputation, Surgical , Anti-Bacterial Agents/administration & dosage , Bacteremia/microbiology , Bacteremia/physiopathology , Female , Humans , Interdisciplinary Communication , Meningococcal Infections/complications , Meningococcal Infections/physiopathology , Purpura Fulminans/etiology , Purpura Fulminans/physiopathology , Treatment OutcomeABSTRACT
Protein C (PC) deficiency is a rare but life-threatening bleeding disorder that can present in the immediate neonatal period. This article presents the case of a baby girl with acute and progressive neonatal purpura fulminans as the presenting feature of PC deficiency. Other common complications of this disease include ophthalmic problems and central nervous system (CNS) changes. Management consists of correcting the coagulopathy, intensive wound care including negative-pressure dressings and skin grafting, and supportive care for the ophthalmic and CNS issues. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.
Subject(s)
Protein C Deficiency/physiopathology , Purpura Fulminans/physiopathology , Female , Humans , Infant, Newborn , Protein C Deficiency/therapy , Purpura Fulminans/therapyABSTRACT
We describe here the case of a boy who presented 2 days after birth with purpura fulminans on his feet and scalp. Laboratory investigations revealed signs of disseminated intravascular coagulation. An underlying coagulation disorder was suspected, and therapy with recombinant tissue plasminogen activator, fresh-frozen plasma, and unfractionated heparin was started. On the basis of plasma protein C activity and antigen levels of 0.02 and 0.03 IU/mL, respectively, after administration of fresh-frozen plasma, a diagnosis of severe protein C deficiency was established, and therapy with intravenous protein C concentrate (Ceprotin [Baxter, Deerfield, IL]) was started. Because of difficulties with venous access, we switched to subcutaneous administration after 6 weeks. The precise dosing schedule for subcutaneously administered protein C concentrate is unknown. In the literature, a trough level of protein C activity at >0.25 IU/mL is recommended to prevent recurrent thrombosis. During 1 year of follow-up our patient frequently had protein C activity levels at <0.25 IU/mL. Clinically, however, there was no recurrent thrombosis, and we kept the dosage unchanged. This report highlights 2 important points: (1) subcutaneously administered protein C concentrate is effective in treating severe protein C deficiency; and (2) in accordance with previous studies, after the acute phase trough levels of protein C activity at >0.25 IU/mL may not be necessary to prevent recurrent thrombosis. However, further research on the dosing, efficacy, and safety of protein C concentrate for prophylaxis and treatment of severe protein C deficiency is needed.
Subject(s)
Protein C Deficiency/diagnosis , Protein C Deficiency/drug therapy , Protein C/therapeutic use , Purpura Fulminans/drug therapy , Dose-Response Relationship, Drug , Drug Administration Schedule , Follow-Up Studies , Humans , Infant, Newborn , Infusions, Intravenous , Infusions, Subcutaneous , Long-Term Care , Male , Protein C Deficiency/complications , Protein C Deficiency/genetics , Purpura Fulminans/etiology , Purpura Fulminans/physiopathology , Risk Assessment , Severity of Illness Index , Treatment OutcomeABSTRACT
BACKGROUND: Acute infectious purpura fulminans (AIPF), a rare syndrome of infection-induced ischemic necrosis of the extremities (symmetric peripheral gangrene), is due most often to Neisseria meningitidis in the US and the UK, but is not endemic to Japan. PURPOSE: We reviewed clinical AIPF manifestations in Japan. METHODS: (A) We retrospectively analyzed six cases of AIPF in the last seven years. (B) We reviewed the medical literature up to 2008 using PubMed search and Japana Centra Revuo Medicina (Igakuchuozasshi) findings. RESULTS: (A) None of the 6 subjects-5 men and 1 woman aged 44 to 69 old- had a history of splenectomy. Except for one case, their disease was community-acquired and immunocompetent. Causative organisms were Streptococcus pneuomoniae (66.7%), Gram negative rods, and Legionella pneumophilla. No localized infectious focus was apparent except for one of Legionella pneumonia. Systemic purpura progressed rapidly within 12 to 96 hours of initial fever or influenza-like symptoms. Main AIPF manifestations are shock and disseminated intervascular coagulation, but not all subjects had both. Six-month mortality was 33.3%, with death within two days of admission. Survivors all required at least two limbs to be amputated. (B) The Japanese literature reported AIPF occurring in 64 cases- 11 neonatal due to congenital protein C deficiency, seven pediatric, and 46 adults. Organisms most commonly involved were S. pneumoniae at 41.3%, and Neisseria meningitidis at 15.2%. The most common pathogen reported in a PubMed search was Neisseria meningitidis. CONCLUSIONS: In Japan, S. pneumoniae is the most frequent isolate associated with AIPF, and progression to AIPF occurs mainly in adults, who commonly suffer frequent amputations and high mortality. Rapid progressing systemic purpura following common-cold-like symptoms is the key to early diagnosis, even if the patient is immunocompetent, not splenectomized, or has no apparent infectious foci.
Subject(s)
Neisseria meningitidis/isolation & purification , Purpura Fulminans/microbiology , Purpura Fulminans/physiopathology , Streptococcus pneumoniae/isolation & purification , Adult , Aged , Female , Humans , Legionella pneumophila/isolation & purification , Male , Middle Aged , Retrospective StudiesSubject(s)
Gram-Negative Bacterial Infections/drug therapy , Infant, Premature, Diseases/drug therapy , Protein C/therapeutic use , Shock, Septic/drug therapy , Enterobacter cloacae/isolation & purification , Enterococcus faecalis/isolation & purification , Escherichia coli/isolation & purification , Gram-Negative Bacterial Infections/microbiology , Humans , Infant , Infant, Newborn , Infant, Premature , Infant, Premature, Diseases/microbiology , Microcirculation , Purpura Fulminans/physiopathology , Retrospective Studies , Sepsis/drug therapy , Sepsis/microbiology , Shock, Septic/microbiology , Treatment OutcomeABSTRACT
Purpura Fulminans is a severe disorder of acute onset with high morbidity and mortality. It is characterized by DIC with thrombocytopenia, hyofibrinogenemia, hypothrombinemia and anemia. It most often occurs in young with sudden appearance of symmetrical, tender, ecchymotic skin lesions usually involving the lower extremities. An infectious and noninfectious etiology has been proposed. Early recognition and early therapy with appropriate antibiotics and heparin is known to limit both morbidity and mortality. This article reports 5 cases of Purpura Fulminans treated at our centre with review of etiology, pathogenesis, clinical features and treatment.
Subject(s)
Purpura Fulminans/physiopathology , Anti-Bacterial Agents/therapeutic use , Anticoagulants/therapeutic use , Basal Ganglia Diseases/etiology , Female , Gangrene/etiology , Gangrene/surgery , Humans , Infant , Male , Purpura Fulminans/complications , Purpura Fulminans/drug therapy , Skin Diseases/etiology , Skin Diseases/surgeryABSTRACT
A 19 years male presented with fever, oliguria and purpuric lesions involving both hands. The patient was diagnosed as a case of purpura fulminans with disseminated intravascular coagulation due to complicated falciparum malaria. The case is presented to sensitize the physicians to keep malaria as a differential in cases of fever with purpura fulminans.