Novel management of post varicella purpura fulminans owing to severe acquired protein S deficiency

Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis
Jennifer J ThomsonBeverley J Hunt

Abstract

Acquired protein S deficiency is a rare complication of varicella zoster infection often resulting in purpura fulminans, which is a potentially life-threatening syndrome of intravascular thrombosis and haemorrhagic infarction of the skin. Patients are presumed to develop crossreacting autoantibodies to the virus and protein S. There is no consensus on how best to manage these patients but previous recommendations focus on plasmapheresis and plasma exchange. We report the case of a 3-year-old girl with post varicella purpura fulminans owing to protein S deficiency, who was diagnosed promptly and made a full recovery following immediate treatment with anticoagulation and plasma support. This suggests that if anticoagulation is used, plasma exchange is not always necessary. As recovery occurred without plasmapheresis, we were able to track protein S levels. The timing of protein S recovery was consistent with the waning titre of an acquired immunoglobulin G autoantibody.

References

Dec 24, 1987·The New England Journal of Medicine·C A MitchellH H Salem
Sep 1, 1995·The Journal of Pediatrics·M LevinR S Heyderman
Mar 1, 1995·British Journal of Haematology·F BergmannA D'Angelo
Jun 17, 1993·The New England Journal of Medicine·A D'AngeloS Viganò D'Angelo
Feb 24, 2001·British Journal of Haematology·D P BusuttilR F Wynn
May 1, 2004·Dermatology : International Journal for Clinical and Investigative Dermatology·Alexandre CampanelliJean-Hilaire Saurat
Jun 11, 2005·Journal of Thrombosis and Haemostasis : JTH·V RegnaultP de Moerloose
Sep 3, 2005·American Journal of Hematology·Aytemiz GürgeyCigdem Altay
Sep 9, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Anis Skander LarakebVéronique Baudouin

❮ Previous
Next ❯

Citations

Apr 18, 2016·Journal of the American Academy of Dermatology·Ganary DabiriTania Phillips
Jun 21, 2016·Indian Journal of Dermatology, Venereology and Leprology·Keshavmurthy A AdyaAparna Palit
Dec 27, 2018·American Journal of Hematology·Lionel Kankeu FonkouaNatthapol Songdej
Apr 26, 2020·Current Opinion in Infectious Diseases·Maria A NagelAndrew N Bubak

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.