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Case Report
Open Access Peer-reviewed

Acute Infectious Purpura Fulminans Complicated with Multiple Erosions

Saya Ikegami, Kei Jitsuiki, Ken-ichi Muramatsu, Hiroki Nagasawa, Ikuto Takeuchi, Youichi Yanagawa
American Journal of Medical Case Reports. 2020, 8(11), 421-423. DOI: 10.12691/ajmcr-8-11-11
Received June 19, 2020; Revised July 20, 2020; Accepted July 30, 2020

Abstract

An 80-year-old man was extremely skinny and developed a left leg phlegmon. He was admitted and underwent an infusion of cefazolin. On the third day, he suddenly collapsed, and advanced cardiac life support resulted in a return of his circulation. He showed vasopressor-resistant hypotension and therefore received an infusion of steroids and multiple supportive therapies. A blood culture later showed Pseudomonas aeruginosa. While his blood pressure increased, he showed peripheral and labile cyanotic changes, even though his peripheral arteries were palpable. On the third day after the collapse, he showed blisters at purpura sites and labile, resulting in the formation of erosions. On the fourth day, massive effusion was drained from the erosive lesions. He temporarily showed a return of consciousness, but his extremities became necrotic, and he ultimately died due to a secondary infection at the erosive lesions. The present fatal case of acute infectious purpura fulminans might have been induced by a P. aeruginosa infection due to complications with multiple blisters, similar to Stevens-Johnson syndrome/toxic epidermal necrolysis. Early aggressive surgical amputation or debridement might be required to obtain a survival outcome when encountering such cases.

1. Introduction

Acute infectious purpura fulminans (AIPF) is a rare syndrome of hemorrhagic infarction of the skin, manifesting as a symmetric peripheral gangrene resulting in extremity loss, disseminated intravascular coagulation and multiple organ failure induced by infection. 1 The two predominantly identified microorganisms are Neisseria meningitidis and Streptococcus pneumoniae. 2, 3 AIPF has a high mortality rate and miserable functional outcome due to amputation, even when patients obtain a survival outcome. 2

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by detachment of the epidermis and mucous membrane, occurring mainly due to drugs. SJS/TEN are considered on the same spectrum of diseases, only demonstrating different severities. 4 AIPF may be complicated with extensive blisters, similar to those seen in cases of SJS/TEN. 5, 6

We herein report a rare case of AIPF with blisters resembling SJS/TEN induced by Pseudomonas (P)aeruginosa.

2. Case Presentation

An 80-year-old man who was extremely skinny due to post-operative esophageal gastrointestinal stromal tumor and had leg edema due to deep venous thrombosis presented with left leg phlegmone. He was admitted to the Dermatology Ward and received infusion of cefazolin. However, his systolic blood pressure decreased to 60 mmHg which thus made it necessary to place the patient in the shock position. The next morning, he suddenly collapsed, and a code blue alert was sounded. Tracheal intubation, infusion of 1 mg of adrenaline and 1 cycle of chest compression resulted in a return of circulation being obtained. He underwent a blood examination and enhanced whole-body computed tomography (CT) and was transferred to the intensive-care unit. The results of blood examinations showed anemia, thrombocytopenia, hypoproteinemia, hyperglycemia, rhabdomyolysis, renal failure and coagulopathy (Table 1). CT showed only bilateral pleural effusion and atelectasis. An electrocardiogram showed sinus tachycardia without ST changes. He showed vasopressor-resistant hypotension and therefore received infusion of steroid in addition to noradrenaline and vasopressin. He also received infusion of meropenem, vancomycin and gamma globulin instead of cefazolin and was transfused with red blood cells, platelets and fresh-frozen plasma. Culture of blood later showed P. aeruginosa. His blood pressure increased under these treatments. However, he showed peripheral and labile cyanotic changes, even though his peripheral arteries were palpable. On the third day after the collapse, he showed blisters at purpura sites and labile, resulting in the formation of erosions. He also showed black gastric fluid from the nasogastric tube and black diarrhea, suggestion hemorrhaging from the bowels. On the fourth day, massive effusion (over 3 L) was drained from the erosive lesions. He temporarily showed a return of consciousness, but his extremities became necrotic (Figure 1), and he ultimately died on day 21 due to secondary infection at the erosive lesions. Permission to perform an autopsy was not obtained from his family.

3. Discussion

We performed a Medline search to identify any related articles using the key words “purpura fulminans” and “P. aeruginosa”. As a result, we found two reports: one by Lerolle et al. and one by Aroor et al. 7, 8 Lerolle et al. reported the results of fluorescence confocal microscopy, which showed P. aeruginosa in the dermal vessels of a deceased patient with purpura fulminans. 7 Microorganisms were typically identified inside the capillaries in foci with multiple bacteria in the purpuric skin. 7 In their report, the clinical course was not described except for the patient’s ultimately death. Aroor at al. reported an eight-year-old boy who presented with a fever and purpuric rash involving all four limbs. 8 Their patient eventually developed gangrene of all digits and the pinna, and the condition deteriorated progressively until he was discharged against medical advice in a moribund state. In that case, the blood culture grew P., but whether or not it was P. aeruginosa was unknown. Accordingly, the present case might be the second or third case of AIPF induced by P. aeruginosa.

Risk factors for AIPF in the present case might have included malnutrition, old age and a history of skin disease. 9, 10 As the patient had vasopressor-resistant hypotension requiring steroids, he might have had adrenal insufficiency in a critical state or Waterhouse-Friderichsen syndrome. 11, 12 As he had received infusion of cefazolin before his collapse, the potential involvement of some undetected causative bacteria cannot be ruled out.

The present case developed multiple erosions at purpura lesions, resulting in massive effusion resembling an extended burn, and secondary infection at these lesions led to his death. Patients with atopic dermatitis whose skin barrier is weak to bacterial infection might become complicated with staphylococcal scalded skin syndrome (SSSS)-like blister lesions induced by P. aeruginosa infection. 13 As the present case had a history of skin lesions and complication with P. aeruginosa infection, his multiple blister lesions might have been complicated with a P. aeruginosa infection.

Mazzone et al. reported that the survival in cases of AIPF was not dependent on surgery, suggesting that surgery does not play a key role in the early phase of the disease and that debridement should be postponed until clear demarcation has been established. 14 However, all patients with AIPF complicated with multiple erosions resembling SJS/TEN who underwent supportive therapy without early surgical operation died, similar to the present case. 5, 6, 15 Accordingly, early aggressive surgical amputation or debridement may be required to obtain a survival outcome, even in patients with serious sequelae.

4. Conclusion

The present fatal case of AIPF might have been induced by P. aeruginosa infection and complicated with SJS/TEN. Early aggressive surgical amputation or debridement may be required to obtain a survival outcome in such combination cases.

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.

Statement of Competing Interests

We do not have no competing interests.

List of Abbreviations

AIPF: Acute infectious purpura fulminans

CT: computed tomography

SJS: Stevens-Johnson syndrome

SSSS: staphylococcal scalded skin syndrome

TEN: toxic epidermal necrolysis

P: pseudomonas

References

[1]  Childers, B.J. and Cobanov, B. “Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases”. Am Surg, 69(1), 86-90, Jan 2003.
In article      
 
[2]  Contou, D., Sonneville, R., Canoui-Poitrine, F., Colin, G., Coudroy, R., Pène, F., Tadié, J.M., Cour, M., Béduneau, G., Marchalot, A., Guérin, L., Jochmans, S., Ehrmann, S., Terzi, N., Préau, S., Barbier, F., Schnell, G., Roux, D., Leroy, O., Pichereau, C., Gélisse, E., Zafrani, L., Layese, R., Brun-Buisson, C., Mekontso Dessap, A. and de Prost, N. “Hopeful Study Group. Clinical spectrum and short-term outcome of adult patients with purpura fulminans: a French multicenter retrospective cohort study”. Intensive Care Med, 44(9), 1502-1511, Sep 2018.
In article      View Article  PubMed
 
[3]  Childers, B.J. and Cobanov, B. “Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases”. Am Surg, 69(1), 86-90, Jan 2003.
In article      
 
[4]  Hasegawa, A. and Abe, R. “Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis.” F1000Res, 9, F1000 Faculty Rev-612, Jun 2020.
In article      View Article  PubMed
 
[5]  Dautzenberg, K.H.W., Polderman, F.N., van Suylen, R.J. and Moviat, M.A.M. “Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy”. Neth J Med, 75(4), 165-168, May 2017.
In article      
 
[6]  Jones, E., Stair-Buchmann, M., Kotliar, S. and Haith, L. “Purpura fulminans in toxic epidermal necrolysis: case report and review”. J Burn Care Res, 36(6), e274-82, 2015.
In article      View Article  PubMed
 
[7]  Lerolle, N., Carlotti, A., Melican, K., Aubey, F., Pierrot, M., Diehl, J.L., Caille, V., Hékimian, G., Gandrille, S., Mandet, C., Bruneval, P., Dumenil, G. and Borgel, D. “Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans”. Am J Respir Crit Care Med, 188(6), 684-92, Sep 2013.
In article      View Article  PubMed
 
[8]  Aroor, S., Varma, C. and Mundkur, S.C. “Purpura fulminans in a child: a case report”. J Clin Diagn Res, 6(10), 1812-3, Dec 2012.
In article      View Article  PubMed
 
[9]  Chakhtoura, N.G.E., Bonomo, R.A. and Jump, R.L.P. “Influence of Aging and environment on presentation of infection in older adults”. Infect Dis Clin North Am, 31(4), 593-608, Dec 2017.
In article      View Article  PubMed
 
[10]  Winter-Jensen, M., Afzal, S., Jess, T., Nordestgaard, B.G. and Allin, K.H. “Body mass index and risk of infections: a Mendelian randomization study of 101,447 individuals”. Eur J Epidemiol, 35(4), 347-354, Apr 2020.
In article      View Article  PubMed
 
[11]  Cooper, M.S. and Stewart, P.M. “Adrenal Insufficiency in Critical Illness”. J Intensive Care Med, 22(6), 348-62, Nov-Dec 2007
In article      View Article  PubMed
 
[12]  Tormos, L.M. and Schandl, C.A. “The significance of adrenal hemorrhage: undiagnosed Waterhouse-Friderichsen syndrome, a case series”. J Forensic Sci, 58(4), 1071-4, Jul 2013.
In article      View Article  PubMed
 
[13]  Chan, J.C.Y., Chiu,. ML.S., Yeung, C.K., Yuen, K.Y. and Chan, H.H.L. “A unique pattern of Staphylococcal scalded skin syndrome-like erosions in patients with atopic dermatitis: dermatitis flammeus”. Skinmed. 16(5), 309-313, Nov 2018.
In article      
 
[14]  Mazzone, L. and Schiestl, C. “Management of septic skin necroses”. Eur J Pediatr Surg, 23(5), 349-58, Oct 2013.
In article      View Article  PubMed
 
[15]  Gunawardane, N.D., Menon, K., Guitart, J. and Cotliar, J.A. “Purpura fulminans from Meningococcemia mimicking Stevens-Johnson syndrome in an adult patient taking etanercept”. Arch Dermatol. 148(12), 1429-31, Dec 2012.
In article      View Article  PubMed
 

Published with license by Science and Education Publishing, Copyright © 2020 Saya Ikegami, Kei Jitsuiki, Ken-ichi Muramatsu, Hiroki Nagasawa, Ikuto Takeuchi and Youichi Yanagawa

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Cite this article:

Normal Style
Saya Ikegami, Kei Jitsuiki, Ken-ichi Muramatsu, Hiroki Nagasawa, Ikuto Takeuchi, Youichi Yanagawa. Acute Infectious Purpura Fulminans Complicated with Multiple Erosions. American Journal of Medical Case Reports. Vol. 8, No. 11, 2020, pp 421-423. http://pubs.sciepub.com/ajmcr/8/11/11
MLA Style
Ikegami, Saya, et al. "Acute Infectious Purpura Fulminans Complicated with Multiple Erosions." American Journal of Medical Case Reports 8.11 (2020): 421-423.
APA Style
Ikegami, S. , Jitsuiki, K. , Muramatsu, K. , Nagasawa, H. , Takeuchi, I. , & Yanagawa, Y. (2020). Acute Infectious Purpura Fulminans Complicated with Multiple Erosions. American Journal of Medical Case Reports, 8(11), 421-423.
Chicago Style
Ikegami, Saya, Kei Jitsuiki, Ken-ichi Muramatsu, Hiroki Nagasawa, Ikuto Takeuchi, and Youichi Yanagawa. "Acute Infectious Purpura Fulminans Complicated with Multiple Erosions." American Journal of Medical Case Reports 8, no. 11 (2020): 421-423.
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[1]  Childers, B.J. and Cobanov, B. “Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases”. Am Surg, 69(1), 86-90, Jan 2003.
In article      
 
[2]  Contou, D., Sonneville, R., Canoui-Poitrine, F., Colin, G., Coudroy, R., Pène, F., Tadié, J.M., Cour, M., Béduneau, G., Marchalot, A., Guérin, L., Jochmans, S., Ehrmann, S., Terzi, N., Préau, S., Barbier, F., Schnell, G., Roux, D., Leroy, O., Pichereau, C., Gélisse, E., Zafrani, L., Layese, R., Brun-Buisson, C., Mekontso Dessap, A. and de Prost, N. “Hopeful Study Group. Clinical spectrum and short-term outcome of adult patients with purpura fulminans: a French multicenter retrospective cohort study”. Intensive Care Med, 44(9), 1502-1511, Sep 2018.
In article      View Article  PubMed
 
[3]  Childers, B.J. and Cobanov, B. “Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases”. Am Surg, 69(1), 86-90, Jan 2003.
In article      
 
[4]  Hasegawa, A. and Abe, R. “Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis.” F1000Res, 9, F1000 Faculty Rev-612, Jun 2020.
In article      View Article  PubMed
 
[5]  Dautzenberg, K.H.W., Polderman, F.N., van Suylen, R.J. and Moviat, M.A.M. “Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy”. Neth J Med, 75(4), 165-168, May 2017.
In article      
 
[6]  Jones, E., Stair-Buchmann, M., Kotliar, S. and Haith, L. “Purpura fulminans in toxic epidermal necrolysis: case report and review”. J Burn Care Res, 36(6), e274-82, 2015.
In article      View Article  PubMed
 
[7]  Lerolle, N., Carlotti, A., Melican, K., Aubey, F., Pierrot, M., Diehl, J.L., Caille, V., Hékimian, G., Gandrille, S., Mandet, C., Bruneval, P., Dumenil, G. and Borgel, D. “Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans”. Am J Respir Crit Care Med, 188(6), 684-92, Sep 2013.
In article      View Article  PubMed
 
[8]  Aroor, S., Varma, C. and Mundkur, S.C. “Purpura fulminans in a child: a case report”. J Clin Diagn Res, 6(10), 1812-3, Dec 2012.
In article      View Article  PubMed
 
[9]  Chakhtoura, N.G.E., Bonomo, R.A. and Jump, R.L.P. “Influence of Aging and environment on presentation of infection in older adults”. Infect Dis Clin North Am, 31(4), 593-608, Dec 2017.
In article      View Article  PubMed
 
[10]  Winter-Jensen, M., Afzal, S., Jess, T., Nordestgaard, B.G. and Allin, K.H. “Body mass index and risk of infections: a Mendelian randomization study of 101,447 individuals”. Eur J Epidemiol, 35(4), 347-354, Apr 2020.
In article      View Article  PubMed
 
[11]  Cooper, M.S. and Stewart, P.M. “Adrenal Insufficiency in Critical Illness”. J Intensive Care Med, 22(6), 348-62, Nov-Dec 2007
In article      View Article  PubMed
 
[12]  Tormos, L.M. and Schandl, C.A. “The significance of adrenal hemorrhage: undiagnosed Waterhouse-Friderichsen syndrome, a case series”. J Forensic Sci, 58(4), 1071-4, Jul 2013.
In article      View Article  PubMed
 
[13]  Chan, J.C.Y., Chiu,. ML.S., Yeung, C.K., Yuen, K.Y. and Chan, H.H.L. “A unique pattern of Staphylococcal scalded skin syndrome-like erosions in patients with atopic dermatitis: dermatitis flammeus”. Skinmed. 16(5), 309-313, Nov 2018.
In article      
 
[14]  Mazzone, L. and Schiestl, C. “Management of septic skin necroses”. Eur J Pediatr Surg, 23(5), 349-58, Oct 2013.
In article      View Article  PubMed
 
[15]  Gunawardane, N.D., Menon, K., Guitart, J. and Cotliar, J.A. “Purpura fulminans from Meningococcemia mimicking Stevens-Johnson syndrome in an adult patient taking etanercept”. Arch Dermatol. 148(12), 1429-31, Dec 2012.
In article      View Article  PubMed