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Images in neonatal medicine
Neonatal purpura fulminans due to protein C deficiency A 3-day-old term male neonate born of third-degree consanguineous marriage was admitted with purplish black, oval tender lesions with erythematous rim on both soles and left hip. He was normothermic, haemodynamically stable and feeding well. However, new gangrenous lesions appeared on his fingers soon after admission. C-reactive protein was negative. Complete blood count revealed a platelet count of 73 000/mm3 with normal haematocrit and leucocyte count. Prothrombin time and activated partial thromboplastin time were prolonged (PT: 26 s; INR: 2.03; aPTT>100 s). Plasma protein C activity was detected to be markedly low (3%, reference range: 31%– 112%). His mother had a history of cortical vein thrombosis during the previous pregnancy when her protein C activity had also been detected to be low (28%) (figures 1–3).
Figure 3 left hip.
Clinical photograph, showing purpura fulminans lesion on
Purpura fulminans (PF) is a catastrophic disease of childhood characterised by the sudden appearance of symmetrical, tender, ecchymotic skin lesions usually involving the lower extremities.1 Inherited and acquired abnormalities of the protein C and S anticoagulant pathway are thought to be responsible for majority of cases. Protein C deficiency is predominantly inherited in an autosomal dominant manner with heterozygous carriers often remaining asymptomatic until later in life when they become highly susceptible to venous thromboembolism.2 The infant was given fresh frozen plasma transfusions and subsequently low molecular weight heparin injections. Within a few hours, the lesions that had been rapidly advancing ceased to progress. Unfortunately, the baby died of a large intraventricular haemorrhage on the tenth day of life, a likely complication of heparin therapy. Shreya Sharma, Jagadeesh Anbazhagan, Nishad Plakkal Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research ( JIPMER), Pondicherry, India
Figure 1 Clinical photograph, showing symmetric gangrenous lesions of purpura fulminans on both soles.
Correspondence to Dr Nishad Plakkal, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research ( JIPMER), Pondicherry605006, India; [email protected] Contributors SS, JA and NP: managed the patient. SS: reviewed the literature and drafted the initial version of the manuscript. NP and JA: contributed to literature review and critically revised the manuscript. All authors contributed to drafting of the manuscript and approved the final version of the manuscript. NP will act as guarantor of the paper. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; internally peer reviewed.
To cite Sharma S, Anbazhagan J, Plakkal N. Arch Dis Child Fetal Neonatal Ed 2015;100:F453. Received 14 January 2015 Accepted 19 February 2015 Published Online First 17 March 2015 Arch Dis Child Fetal Neonatal Ed 2015;100:F453. doi:10.1136/archdischild-2015-308238
REFERENCES 1 2
Figure 2 New gangrenous lesion on the fingers of the infant’s left hand after admission.
Dudgeon DL, Kellog DR, Gllchirst GS, et al. Purpura fulminans. Arch Surg 1971;103:351–8. Marlar RA, Montgomery RR, Broekmans AW. Diagnosis and treatment of homozygous protein C deficiency. Report of the Working Party on Homozygous Protein C Deficiency of the Subcommittee on Protein C and Protein S, International Committee on Thrombosis and Haemostasis. J Pediatr 1989;114:528–34.
Sharma S, et al. Arch Dis Child Fetal Neonatal Ed September 2015 Vol 100 No 5
F453
Downloaded from http://fn.bmj.com/ on August 19, 2015 - Published by group.bmj.com
Neonatal purpura fulminans due to protein C deficiency Shreya Sharma, Jagadeesh Anbazhagan and Nishad Plakkal Arch Dis Child Fetal Neonatal Ed 2015 100: F453 originally published online March 17, 2015
doi: 10.1136/archdischild-2015-308238 Updated information and services can be found at: http://fn.bmj.com/content/100/5/F453
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Images in neonatal medicine
Neonatal purpura fulminans due to protein C deficiency A 3-day-old term male neonate born of third-degree consanguineous marriage was admitted with purplish black, oval tender lesions with erythematous rim on both soles and left hip. He was normothermic, haemodynamically stable and feeding well. However, new gangrenous lesions appeared on his fingers soon after admission. C-reactive protein was negative. Complete blood count revealed a platelet count of 73 000/mm3 with normal haematocrit and leucocyte count. Prothrombin time and activated partial thromboplastin time were prolonged (PT: 26 s; INR: 2.03; aPTT>100 s). Plasma protein C activity was detected to be markedly low (3%, reference range: 31%– 112%). His mother had a history of cortical vein thrombosis during the previous pregnancy when her protein C activity had also been detected to be low (28%) (figures 1–3).
Figure 3 left hip.
Clinical photograph, showing purpura fulminans lesion on
Purpura fulminans (PF) is a catastrophic disease of childhood characterised by the sudden appearance of symmetrical, tender, ecchymotic skin lesions usually involving the lower extremities.1 Inherited and acquired abnormalities of the protein C and S anticoagulant pathway are thought to be responsible for majority of cases. Protein C deficiency is predominantly inherited in an autosomal dominant manner with heterozygous carriers often remaining asymptomatic until later in life when they become highly susceptible to venous thromboembolism.2 The infant was given fresh frozen plasma transfusions and subsequently low molecular weight heparin injections. Within a few hours, the lesions that had been rapidly advancing ceased to progress. Unfortunately, the baby died of a large intraventricular haemorrhage on the tenth day of life, a likely complication of heparin therapy. Shreya Sharma, Jagadeesh Anbazhagan, Nishad Plakkal Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research ( JIPMER), Pondicherry, India
Figure 1 Clinical photograph, showing symmetric gangrenous lesions of purpura fulminans on both soles.
Correspondence to Dr Nishad Plakkal, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research ( JIPMER), Pondicherry605006, India; [email protected] Contributors SS, JA and NP: managed the patient. SS: reviewed the literature and drafted the initial version of the manuscript. NP and JA: contributed to literature review and critically revised the manuscript. All authors contributed to drafting of the manuscript and approved the final version of the manuscript. NP will act as guarantor of the paper. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; internally peer reviewed.
To cite Sharma S, Anbazhagan J, Plakkal N. Arch Dis Child Fetal Neonatal Ed 2015;100:F453. Received 14 January 2015 Accepted 19 February 2015 Published Online First 17 March 2015 Arch Dis Child Fetal Neonatal Ed 2015;100:F453. doi:10.1136/archdischild-2015-308238
REFERENCES 1 2
Figure 2 New gangrenous lesion on the fingers of the infant’s left hand after admission.
Dudgeon DL, Kellog DR, Gllchirst GS, et al. Purpura fulminans. Arch Surg 1971;103:351–8. Marlar RA, Montgomery RR, Broekmans AW. Diagnosis and treatment of homozygous protein C deficiency. Report of the Working Party on Homozygous Protein C Deficiency of the Subcommittee on Protein C and Protein S, International Committee on Thrombosis and Haemostasis. J Pediatr 1989;114:528–34.
Sharma S, et al. Arch Dis Child Fetal Neonatal Ed September 2015 Vol 100 No 5
F453
Downloaded from http://fn.bmj.com/ on August 19, 2015 - Published by group.bmj.com
Neonatal purpura fulminans due to protein C deficiency Shreya Sharma, Jagadeesh Anbazhagan and Nishad Plakkal Arch Dis Child Fetal Neonatal Ed 2015 100: F453 originally published online March 17, 2015
doi: 10.1136/archdischild-2015-308238 Updated information and services can be found at: http://fn.bmj.com/content/100/5/F453
These include:
References Email alerting service
Topic Collections
This article cites 2 articles, 0 of which you can access for free at: http://fn.bmj.com/content/100/5/F453#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Articles on similar topics can be found in the following collections Immunology (including allergy) (385) Competing interests (ethics) (96) Drugs: cardiovascular system (262) Genetic screening / counselling (13) Journalology (104) Pregnancy (1486) Reproductive medicine (1399) Stroke (92)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
