Skip to main content
Top
Published in: European Journal of Pediatrics 7/2010

01-07-2010 | Short Report

Purpura fulminans in a newborn infant with galactosemia

Authors: Aysegul Zenciroglu, Mehmet Sah Ipek, Mustafa Aydin, Abdurrahman Kara, Nurullah Okumus, Mustafa Kilic

Published in: European Journal of Pediatrics | Issue 7/2010

Login to get access

Abstract

An 11-day-old neonate presented with purpura fulminans and was subsequently diagnosed with galactosemia. Neonatal purpura fulminans occurs predominantly in patients suffering from inherited protein C deficiency or disseminated intravascular coagulation associated with septicemia. Hemostatic changes in patients with liver disease may result in bleeding or, rarely, thrombosis. We suppose that in the present patient, deficiency of protein C, secondary to liver disease, was responsible for the development of purpura fulminans. Treatment consisted of blood and blood products and galactose-free formula. The patient recovered with residual mild psychomotor retardation and the lesions with minimal scarring. In conclusion, galactosemia also should be kept in mind as an uncommon cause of purpura fulminans in newborn infants.
Literature
1.
go back to reference Adcock DM, Brozna J, Marlar RA (1990) Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 16:333–340CrossRefPubMed Adcock DM, Brozna J, Marlar RA (1990) Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 16:333–340CrossRefPubMed
2.
go back to reference Adcock DM, Hicks MJ (1990) Dermatopathology of skin necrosis associated with purpura fulminans. Semin Thromb Hemost 16:283–292CrossRefPubMed Adcock DM, Hicks MJ (1990) Dermatopathology of skin necrosis associated with purpura fulminans. Semin Thromb Hemost 16:283–292CrossRefPubMed
3.
go back to reference Amitrano L, Guardascione MA, Brancaccio V, Balzano A (2002) Coagulation disorders in liver disease. Semin Liver Dis 22:83–96CrossRefPubMed Amitrano L, Guardascione MA, Brancaccio V, Balzano A (2002) Coagulation disorders in liver disease. Semin Liver Dis 22:83–96CrossRefPubMed
5.
go back to reference Darmstadt GL (1998) Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol 15:169–183CrossRefPubMed Darmstadt GL (1998) Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol 15:169–183CrossRefPubMed
7.
go back to reference Gurgey A, Aytac S, Kanra G et al (2005) Outcome in children with purpura fulminans: report on 16 patients. Am J Hematol 80:20–25CrossRefPubMed Gurgey A, Aytac S, Kanra G et al (2005) Outcome in children with purpura fulminans: report on 16 patients. Am J Hematol 80:20–25CrossRefPubMed
8.
go back to reference Levi M, Toh CH, Thachil J, Watson HG (2009) Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Hematology. Br J Hematol 145:24–33CrossRef Levi M, Toh CH, Thachil J, Watson HG (2009) Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Hematology. Br J Hematol 145:24–33CrossRef
9.
go back to reference Lisman T, Leebeek FW (2007) Hemostatic alterations in liver disease: a review on pathophysiology, clinical consequences, and treatment. Dig Surg 24:250–258CrossRefPubMed Lisman T, Leebeek FW (2007) Hemostatic alterations in liver disease: a review on pathophysiology, clinical consequences, and treatment. Dig Surg 24:250–258CrossRefPubMed
10.
go back to reference Minutillo C, Pemberton PJ, Willoughby ML et al (1990) Neonatal purpura fulminans and transient protein C deficiency. Arch Dis Child 65:561–562CrossRefPubMed Minutillo C, Pemberton PJ, Willoughby ML et al (1990) Neonatal purpura fulminans and transient protein C deficiency. Arch Dis Child 65:561–562CrossRefPubMed
11.
go back to reference Nolan J, Sinclair R (2001) Review of management of purpura fulminans and two case reports. Br J Anaesth 86:581–586CrossRefPubMed Nolan J, Sinclair R (2001) Review of management of purpura fulminans and two case reports. Br J Anaesth 86:581–586CrossRefPubMed
12.
go back to reference Northup PG, Sundaram V, Fallon MB, Coagulation in Liver Disease Group et al (2008) Hypercoagulation and thrombophilia in liver disease. J Thromb Haemost 6:2–9PubMed Northup PG, Sundaram V, Fallon MB, Coagulation in Liver Disease Group et al (2008) Hypercoagulation and thrombophilia in liver disease. J Thromb Haemost 6:2–9PubMed
13.
go back to reference Ray JG, Shmorgun D, Chan WS (2002) Common C677T polymorphism of the methylenetetrahydrofolate reductase gene and the risk of venous thromboembolism: meta-analysis of 31 studies. Pathophysiol Haemost Thromb 32:51–58CrossRefPubMed Ray JG, Shmorgun D, Chan WS (2002) Common C677T polymorphism of the methylenetetrahydrofolate reductase gene and the risk of venous thromboembolism: meta-analysis of 31 studies. Pathophysiol Haemost Thromb 32:51–58CrossRefPubMed
14.
go back to reference Reverdiau-Moalic P, Delahousse B, Body G et al (1996) Evolution of blood coagulation activators and inhibitors in the healthy human fetus. Blood 88:900–906PubMed Reverdiau-Moalic P, Delahousse B, Body G et al (1996) Evolution of blood coagulation activators and inhibitors in the healthy human fetus. Blood 88:900–906PubMed
15.
go back to reference Veldman A, Nold MF, Michel-Behnke I (2008) Thrombosis in the critically ill neonate: incidence, diagnosis, and management. Vasc Health Risk Manag 4:1337–1348PubMed Veldman A, Nold MF, Michel-Behnke I (2008) Thrombosis in the critically ill neonate: incidence, diagnosis, and management. Vasc Health Risk Manag 4:1337–1348PubMed
16.
go back to reference Walter JH, Collins JE, Leonard JV (1999) Recommendations for the management of galactosemia. UK Galactosemia Steering Group. Arch Dis Child 80:93–96CrossRef Walter JH, Collins JE, Leonard JV (1999) Recommendations for the management of galactosemia. UK Galactosemia Steering Group. Arch Dis Child 80:93–96CrossRef
17.
go back to reference Welton MJ (1972) Local ‘Schwartzman equivalent’ reaction in active chronic hepatitis. Gut 13:43–46CrossRef Welton MJ (1972) Local ‘Schwartzman equivalent’ reaction in active chronic hepatitis. Gut 13:43–46CrossRef
Metadata
Title
Purpura fulminans in a newborn infant with galactosemia
Authors
Aysegul Zenciroglu
Mehmet Sah Ipek
Mustafa Aydin
Abdurrahman Kara
Nurullah Okumus
Mustafa Kilic
Publication date
01-07-2010
Publisher
Springer-Verlag
Published in
European Journal of Pediatrics / Issue 7/2010
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-009-1121-y

Other articles of this Issue 7/2010

European Journal of Pediatrics 7/2010 Go to the issue