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Neonatal Purpura Fulminans

Neonatal Purpura Fulminans (homozygous Protein C Deficiency)


Presentation

  • A four-day-old neonate presented with multiple necrotic skin lesions with abnormal coagulation profile. Skin lesions responded to repeated plasma transfusions but the neonate developed bilateral retinal detachment.[ncbi.nlm.nih.gov]
  • Alerts and Notices Synopsis   Neonatal purpura fulminans is a rare but life-threatening disorder that presents in a neonate with rapidly progressive intravascular thrombosis and hemorrhagic infarction of the skin.[visualdx.com]
Cyanotic Congenital Heart Disease
  • The hemorrhagic diathesis associated with cyanotic congenital heart disease. In: Lusher JM, Barnhart M, eds. Acquired bleeding disorders in children. Vol. 1. Abnormalities of hemostasis. New York: Masson Publishing, 1981:91–8.[nejm.org]
Easy Bruising
  • There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums.[medical-dictionary.thefreedictionary.com]
Saddle Nose
  • Most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). Almost all patients with WG have c-ANCA, but not vice versa. Current treatment of choice is cyclophosphamide.[findzebra.com]
Cardiomegaly
  • Progressive cardiac failure with tachypnea, hepatomegaly, cardiomegaly, and pulmonary hypertension developed in spite of increasing doses of digitalis and diuretics, necessitating open-heart surgery on day 43.[nejm.org]
Systolic Murmur
  • A systolic murmur due to a large infundibular septal defect and persistence of the ductus arteriosus was detected on the 10th day of life.[nejm.org]
Purpura
  • The authors present a neonate with extensive purpura fulminans due to group B streptoccoccal septicemia and evaluated the attributable clinical mortality and morbidity of this potentially lethal syndrome.[ncbi.nlm.nih.gov]
Cutaneous Manifestation
  • PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth.[ncbi.nlm.nih.gov]
  • Purpura fulminans: a cutaneous manifestation of severe protein C deficiency . Arch Dermatol 1988 ;124: 1387 –91. 10. Hermsen VM, Conahan JB, Koops BL, Cunningham RD. . Persistent hyperplastic primary vitreous associated with protein C deficiency .[nejm.org]
Hyperkeratosis
  • Histopathology revealed epidermal hyperkeratosis with epidermal-dermal splitting, vessels showing fibrin occlusion with red blood cell extravasation into the perivascular areas in dermis along with dermal necrosis.[ijpd.in]
Keratosis
  • In a patient with Citrobacter infections, the bacteria can be transmitted vertically from mother or horizontally from carriers or other hospital sources. [2] On histopathological examination, classical findings of epidermal keratosis with epidermal-dermal[ijpd.in]
Slurred Speech
  • Resulting complications can include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems.[findzebra.com]

Workup

  • Other features include a Chief Complaints section addressing the workup and treatment of 50 signs and symptoms, plus a medication index, syndromes glossary, surgical glossary, laboratory values, and tables all for quick reference saving you time and helping[books.google.com]
  • The initial workup revealed disseminated intravascular coagulopathy, and both blood and CSF cultures grew GBS. He had normal levels of protein C and protein S for his age.[ncbi.nlm.nih.gov]
  • A diagnosis of protein C deficiency was suspected and thrombophilia workup was done for the baby and her parents. Doppler ultrasound of the head and abdomen was normal. There was no family history of venous thrombotic disease.[ispub.com]
Insulin Increased
  • On the contrary, the information centre on hospital admissions recorded a decrease in the number of admissions for severe hypoglycaemia during 1983-88, the period when the market share of human insulin increased from 4-1% to 82.1% These data conflict[kundoc.com]

Treatment

  • The treatments given during surgery are shown in Figure 1.[nejm.org]
  • Treatment is mainly removing the underlying cause and degree of clotting abnormalities and with supportive treatment (antibiotics, volume expansion, tissue oxygenation, etc.). Thus, treatment includes aggressive management of the septic state.[flipper.diff.org]
  • Early clinical recognition, timely investigation and treatment is utmost important.[jpma.org.pk]
  • Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout.[books.google.com]

Prognosis

  • This is crucial considering the poor prognosis and the need for multidisciplinary care to address the ophthalmological and neurological sequelae of this dreaded condition.[indianpediatrics.net]
  • ( i ) it occurs in patients with a history of administering certain medications; ( ii ) and with no prodromal infections; ( iii ) serum protein C and protein S levels may be normal; ( iv ) it responds to corticosteroids; ( v ) generally with a good prognosis[medicaljournals.se]
  • Protein C deficiency does NOT appear to cause arterial thrombosis. [ 5 ] Prognosis There is a 10- to 15-fold risk of VTE. [ 5 ] However, there is a marked variation in risk among families with protein C deficiency, that cannot be explained by the genetic[patient.info]
  • The prognosis for these patients is guarded. References [1] Neonatal Purpura Fulminans, a rare genetic disorder due to protein C deficiency: A case report., Irfan Kazi SG,Siddiqui E,Habib I,Tabassum S,Afzal B,Khan IQ,, JPMA.[statpearls.com]

Etiology

  • Etiology of neonatal purpura fulminans Purpura fulminans was first described in a neonate in 1962 and the etiology was presumed an inherited disorder as three siblings had similar skin lesions.[flipper.diff.org]
  • Epidemiology History : first described by Guelliot in 1884 Etiology Acute Infectious Purpura Fulminans Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus , [[Capnocytophaga Canimorsus]]) Epidemiologic Exposure : dog bite Risk Factor for Bacteremia[mdnxs.com]
  • However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF.[e-ijd.org]
  • If an infant presents with purpuric and necrotic lesions, consider the possibility of congenital or infective etiology of purpura fulminans and investigate thoroughly by doing protein S and C levels, blood tests (D-dimer, aPTT, PT, complete blood count[ijpd.in]
  • Etiology In older children, purpura fulminans may have several causes.[drmhijazy.com]

Epidemiology

  • Epidemiology of invasive group B streptococcal disease in the United States, 1999–2005. JAMA. 2008; 299 :2056–65. [ PubMed ] [ Google Scholar ] 18. Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: recognition, diagnosis and management.[ncbi.nlm.nih.gov]
  • Epidemiology History : first described by Guelliot in 1884 Etiology Acute Infectious Purpura Fulminans Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus , [[Capnocytophaga Canimorsus]]) Epidemiologic Exposure : dog bite Risk Factor for Bacteremia[mdnxs.com]
  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
  • […] disseminated intravascular coagulation (DIC) Associated with a congenital or acquired protein C and/or protein S deficiency Life-threatening condition that requires prompt diagnosis and judicious replacement therapy to decrease morbidity and mortality Epidemiology[unboundmedicine.com]
  • Genatlas disease for PROC Gene thrombosis,recurrent (pro-C type I and type II deficiency) Relevant External Links for PROC Genetic Association Database (GAD) PROC Human Genome Epidemiology (HuGE) Navigator PROC Atlas of Genetics and Cytogenetics in Oncology[genecards.org]
Sex distribution
Age distribution

Pathophysiology

  • While three distinct presentations of purpura fulminans are recognized, the underlying pathophysiology stems from a qualitative or quantitative deficiency in protein C, an important regulator of the clotting cascade.[emedicine.medscape.com]
  • (Overview of pathophysiology and assessment of DIC.) Piette, W, Bolognia, JL, Jorizzo, JL, Rapini, RP. “Cutaneous manifestations of microvascular occlusion syndromes”. Dermatology. 2008. pp. 331-45.[dermatologyadvisor.com]
  • Pathophysiology Common features of purpura fulminans: DIC: Endothelial injury from bacterial endotoxin or other trigger may initiate secretion of inflammatory cytokines or activation of coagulation and complement proteins.[unboundmedicine.com]

Prevention

  • Prevention of perinatal group B streptococcal disease: a public health perspective. Centers for Disease Control and Prevention. MMWR Recomm Rep. 1996; 45 :1–24. [ PubMed ] [ Google Scholar ] 16. Eberly MD, Rajnik M.[ncbi.nlm.nih.gov]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Prevention of hypothermia is also of utmost importance in patients suffering from protein C deficiency, as hypothermia may trigger thrombosis.[ijaweb.org]
  • […] either alone, or in combination with coumarins or low molecular weight heparin is main stay of management. 10 Neonatal Purpura Fulminans is a life threatening condition although it is very rare but early diagnosis and prompt management is crucial to prevent[jpma.org.pk]
  • Early diagnosis and adequate therapy with the protein C concentrate may have been lifesaving and decisive in preventing irreversible organ damage in this patient.[nejm.org]

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