Dear Label_Name,

Greetings! I hope you are well.

I am writing on behalf of an international publishing house, Science and Education Publishing, USA (http://www.sciepub.com).

Science and Education Publishing (SciEP) is a multidisciplinary open access publisher of Journals covering the fields of Science, Technology and Medicine. All SciEP publications are subject to high-quality peer review, editorial and production processes and are freely available online.

Welcome to the April, 2013 edition of SciEP newsletter, providing monthly summaries of articles on science & education related topics of interest to scientist and professionals. The mission of our journals is to provide an open access forum for publishing new findings on issues pertinent to scientific and academic field. We also invite you to submit original research papers, review articles, short reports, or case studies are considered for publication in our Multidisciplinary Journals.

American Journal of Public Health Research
DOI: 10.12691/ajphr-1-1-3

The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

American Journal of Medical and Biological Research
DOI: 10.12691/ajmbr-1-1-2

The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
Lelevich Sergey Vladimirovich, Khrustalev Vladislav Victorovich, Barkovsky Eugene Victorovich, Shedogubova Tatyana Aleksandrovna

American Journal of Medical Sciences and Medicine
DOI: 10.12691/ajmsm-1-1-1

Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
DOI: 10.12691/ajidm-1-1-2

The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

We have released issues of some journals on February 2013.
American Journal of Public Health Research
American Journal of Medical Sciences and Medicine
American Journal of Medical and Biological Research
American Journal of Pharmacological Sciences
American Journal of Clinical Medicine Research
American Journal of Nursing Research
American Journal of Cancer Prevention
American Journal of Medicine Studies
American Journal of Infectious Diseases and Microbiology
American Journal of Cardiovascular Disease Research
American Journal of Biomedical Research
American Journal of Mechanical Engineering
American Journal of Microbiological Research
Journal of Preventive Medicine
American Journal of Food and Nutrition
American Journal of Hypertension Research
American Journal of Sports Science and Medicine
Journal of Cancer Research and Treatment
American Journal of Zoological Research
Journal of Biomedical Engineering and Technology
American Journal of Microbiological Research
Biomedical Science and Engineering

We are pleased to announce that submissions are now being accepted for peer review for all the journals across all our subject areas in SciEP.

Please submit your manuscript via our online submission system:
http://www.sciepub.com/author/signin

To unsubscribe from our mailing list, please click on the following link: unsubscribe

I am looking forward to hearing from you.

Kind regards,

David Rain
Email:join@sciepub.com
Science and Education Publishing (http://www.sciepub.com)

Acute Infectious Purpura Fulminans with Anticentromere Antibodies

Ikuto Takeuchi, Ken-ichi Muramatsu, Soichiro Ota, Youichi Yanagawa*

Department of Acute Critical Care Medicine, Shizuoka Hospital, Juntendo University

Abstract: Purpura fulminans (PF) is a rare, life-threatening syndrome characterized by disseminated intravascular coagulation (DIC) and endovascular thrombosis resulting in a characteristic pattern of cutaneous purpura. There have been no reports of the combination of PF and anti-centromere antibody (ACA). A 70-year-old woman with a 2-day history of lumbago was transported from a local medical facility to our hospital due to right hydronephrosis due to ureterolithiasis and urosepsis. She had systemic lupus erythematosus (SLE) and schizophrenia. On arrival, she had consciousness disturbance and was in a shock state with cyanotic extremities. She was additionally diagnosed with renal failure, DIC and ACA positivity after examinations. She was treated with vasopressor, antibiotics and ureter stent placement. However, she developed unstable circulation, which necessitated tracheal intubation and continuous hemodiafiltration. Urine and blood cultures showed Escherichia coli. However, the cyanosis of all of her extremities changed to necrosis and she died on day 8 due to multiple organ failure. This is the first case of acute infectious PF with ACA positivity. As ACA is known to be a risk factor for digital necrosis, the present case suggests that in addition to a decrease of protein C or S, ACA may contribute to the development of PF.

Keywords: purpura fulminans, anticentromere antibody, pyelonephritis

Received January 19, 2023; Revised February 25, 2023; Accepted March 07, 2023

Citation: Ikuto Takeuchi, Ken-ichi Muramatsu, Soichiro Ota, Youichi Yanagawa, Acute Infectious Purpura Fulminans with Anticentromere Antibodies. American Journal of Medical Case Reports, 11 (3). 52-54. January 2023. doi:10.12691/ajmcr-11-3-3

1. Introduction

Purpura fulminans (PF) is a rare, life-threatening syndrome characterized by disseminated intravascular coagulation (DIC) and endovascular thrombosis resulting in a characteristic pattern of cutaneous purpura. [1,2] PF is thought to result from dysfunction of the body's natural anticoagulant mechanisms, particularly abnormalities in the function of protein C (PC) and associated proteins as protein S (PS), and antithrombin (AT). PC is a vitamin K-dependent serine protease that negatively regulates coagulation and has anti-inflammatory and cytoprotective properties. The plasma levels of PC, PS, and AT antigen, which works as an anticoagulant, were significantly lower in affected patients. [3] In addition, among elderly individuals, asplenic or immunosuppressed patients are more likely to develop acute infectious PF in response to infection. PF tends to occur as a result of infection by endotoxin-producing Gram-negative bacteria but can also occur secondary to infection with Gram-positive and anaerobic organisms or viruses in adults.

While, anti-centromere antibody (ACA) is a type of anti-nuclear antibody (ANA) detected by indirect immunofluorescence displaying a centromeric pattern. ACA has been considered to be specific for limited cutaneous systemic sclerosis. [4] However, ACA and digital necrosis without sclerodactyly and sclerosis of the internal organs has been reported as a distinct entity from scleroderma with sclerosis. [5,6] Digital necrosis also occurs in PF. However, there has been no reports of the combination of PF and ACA. We herein report a case of acute infectious PF with ACA positivity.

2. Case Report

A 70-year-old woman with a 2-day history of right lumbago was transported from a local medical facility to our hospital by physician-staffed helicopter due to right hydronephrosis due ureterolithiasis, uroseptic shock. She had systemic lupus erythematosus (SLE) and schizophrenia, and was treated with methylprednisolone (5 mg), brexpiprazole (2 mg), aripiprazole (6 mg), and haloperidol (1 mg). Tests for antinuclear antibodies had been negative for 10 years. On arrival, her vital signs were as follows: Glasgow Coma Scale, E3V4M6; blood pressure, 90/50 mmHg under 0.1 μg/kg/min of noradrenalin; heart rate, 88 beats per minute; respiratory rate, 17 breaths per minute; body temperature, 36.6°C. A physical examination revealed cyanotic change at all extremities, with palpable radial and dorsal pedis arteries. There were no other remarkable findings due to consciousness disturbance. The patient’s blood test results are shown in Table 1. She was positive for antinuclear antibodies and ACA. Whole body computed tomography revealed calcification of the bilateral kidneys with right hydronephrosis and the dirty fat sign. She was diagnosed with right hydronephrosis due to ureterolithiasis, calculous pyelonephritis with septic shock, renal failure, and DIC, and was treated with vasopressor, antibiotics (linezolid and meropenem), an increased steroid dose and indwelling ureteral stent placement. However, her unstable circulation deteriorated and tracheal intubation was performed with mechanical ventilation and continuous hemodiafiltration on day 2. Urine and blood cultures grew Escherichia (E) coli. However, the cyanosis of all extremities progressed to necrosis (Figure 1) on day 6. She died due to multiple organ failure on day 8.

Figure 1. All extremities of the patient on day 6

All extremities had purpura and digital necrosis was present.

Table 1.

3. Discussion

This patient had DIC with necrosis of all extremities after developing calculous pyelonephritis due to E. coli infection and died of multiple organ failure. These findings are compatible with PF, as reported in a previous case. [7] While the present case was also positive for ACA. To our best knowledge, this is the first report of a patient with acute infectious PF with positive ACA.

In the present case, autoimmune disease was risk factor for the development of PF. [8,9,10] Most reported cases were associated with antiphospholipid antibody positivity, which can induce venous and/or arterial thrombosis by platelet activation and the production of procoagulants, such as von Willebrand factor. [11] The skin appears to be an important target organ in antiphospholipid antibody syndrome, including livedo reticularis, digital gangrene, or extensive cutaneous necrosis resembling PF. Unfortunately, we did not measure the patient’s antiphospholipid antibody level. Digital necrosis can occur in patients with ACA positivity. [5,6] ACA might be related to the factors that are thought to be responsible for arteriole occlusion, such as enhanced platelet activation, endothelial damage, increased soluble mediators of vasoconstriction, or a hypercoagulation state. [5,12] Although these are hypotheses and further investigation is required for validation, the present case suggests that, in addition to a decrease in protein C or S, ACA may contribute to the development of PF.

4. Conclusion

This is the first case of a case of acute infectious PF with ACA positivity. The present case suggests that in addition to a decrease of protein C or S, ACA may contribute to the development of PF.

Acknowledgements

This work was supported in part by a Grant-in-Aid for Special Research in Subsidies for ordinary expenses of private schools from The Promotion and Mutual Aid Corporation for Private Schools of Japan.

References

[1]   | Colling ME, Bendapudi PK. Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis. Transfus Med Rev. 2018 Apr; 32(2): 69-76.
[2]   | Perera TB, Murphy-Lavoie HM. Purpura Fulminans. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. 2022 Jul 18. PMID: 30422460.
[3]   | High KA. Antithrombin III, protein C, and protein S. Naturally occurring anticoagulant proteins. Arch Pathol Lab Med. 1988. PMID: 2962557 Review.

[4]  
Kuramoto N, Ohmura K, Ikari K, Yano K, Furu M, Yamakawa N, Hashimoto M, Ito H, Fujii T, Murakami K, Nakashima R, Imura Y, Yukawa N, Yoshifuji H, Taniguchi A, Momohara S, Yamanaka H, Matsuda F, Mimori T, Terao C. Anti-centromere antibody exhibits specific distribution levels among anti-nuclear antibodies and may characterize a distinct subset in rheumatoid arthritis. Sci Rep. 2017 Jul 31; 7(1): 6911.

[5]   | Sachsenberg-Studer EM, Prins C, Saurat JH, Salomon D. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma? J Am Acad Dermatol. 2000 Oct; 43(4): 631-4.
[6]   | Bolster L, Taylor-Gjevre RM, Nair B, Gjevre JA. Digital gangrene associated with anticentromere antibodies: a case report. J Med Case Rep. 2010 Jun 22; 4: 189.
[7]   | Lowry J, Noel E. A Rare Cause of a Rare Disorder: E. coli-Induced Purpura Fulminans Secondary to Urinary Tract Infection. Case Rep Crit Care. 2022 Apr 6; 2022: 9291424.
[8]   | Gamba G, Montani N, Montecucco CM, Caporali R, Ascari E. Purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report. Haematologica. 1991 Sep-Oct; 76(5): 426-8.
[9]   | Chatterjee S, Pauling JD. Anti-phospholipid syndrome leading to digital ischaemia and rare organ complications in systemic sclerosis and related disorders. Clin Rheumatol. 2021 Jun; 40(6): 2457-2465.
[10]   | Demirkaya E, Cakmakli HF, Güçer S, Aktay-Ayaz N, Gürgey A, Ozen S. Purpura fulminans as the presenting manifestation in a patient with juvenile SLE. Turk J Pediatr. 2009 Jul-Aug; 51(4): 378-80.
[11]   | Sole K. Mechanisms of thrombosis in antiphospholipid syndrome. Nat Clin Pract Rheumatol. 2006; 2: 176.
[12]   | Takahashi M, Okada J, Kondo H. Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities. Br J Rheumatol. 1997 Aug; 36(8): 889-93.