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E-IJD CASE REPORT
Year : 2016  |  Volume : 61  |  Issue : 1  |  Page : 120
A fatal case of "bullous erysipelas-like" pseudomonas vasculitis


1 Department of General Medicine, University Medicine Cluster, National University of Singapore, Singapore, Asia
2 Department of Pathology, Yong Loo Lin School of Medicine, National University Health System, National University of Singapore, Singapore, Asia

Date of Web Publication15-Jan-2016

Correspondence Address:
Sam Shiyao Yang
Department of General Medicine, University Medicine Cluster, National University Health System, Singapore
Asia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.174095

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   Abstract 

Erysipelas is a generally benign superficial bacterial skin infection, and its bullous form constitutes a rare and more severe variant. We describe the first and fatal case of "bullous erysipelas-like" septic vasculitis due to Pseudomonas bacteremia. A 69-year-old Chinese man presenting with diarrhea and septic shock initially began to rapidly develop sharply defined erythematous plaques with non-hemorrhagic bullae over his lower limbs. Culture of the aspirate from the bullae was positive for Pseudomonas aeruginosa. This was also consistent with his blood cultures showing Pseudomonas bacteremia. Histology of the skin lesion showed microthrombi and neutrophilic infiltrates in blood vessels with Gram-negative bacilli extruding from the vessel walls, characteristic of septic vasculitis. The bullous erysipelas-like lesions seen in this patient represents a rare manifestation of both septic vasculitis and Pseudomonas infection.


Keywords: Bullous erysipelas, leukocytoclastic vasculitis, septic vasculitis


How to cite this article:
Yang SS, Chandran NS, Huang JX, Tan KB, Aw DC. A fatal case of "bullous erysipelas-like" pseudomonas vasculitis. Indian J Dermatol 2016;61:120

How to cite this URL:
Yang SS, Chandran NS, Huang JX, Tan KB, Aw DC. A fatal case of "bullous erysipelas-like" pseudomonas vasculitis. Indian J Dermatol [serial online] 2016 [cited 2020 Sep 26];61:120. Available from: http://www.e-ijd.org/text.asp?2016/61/1/120/174095

What was known?
Erysipelas is a generally benign superficial bacterial skin infection, and its bullous form constitutes a rare and more severe variant that may represent a synergistic superinfection with Staphylococcus aureus.



   Introduction Top


Erysipelas or St. Anthony's fire, as it was formerly known, is defined as a febrile skin infection with a sudden onset of a red indurated expanding plaque with a distinct border. [1]

It is a superficial skin infection involving the upper level of the dermis. Its morphological presentation is reflective of this level of skin involvement as opposed to other superficial infections such as crusting seen in impetigo and superficial ulceration in ecthyma, or deeper infections like cellulitis where the lesional borders becomes indistinct. [2]

Streptococcus is the most common pathogen involved in erysipelas. It is a generally benign condition; only severely ill patients require inpatient treatment, where they receive intravenous antibiotics and have a median stay of 6 to 7 days. [1] These patients are usually immunocompromised with co-morbidities such as diabetes mellitus, liver cirrhosis, malignancies and alcohol abuse. Complications are generally uncommon, occurring at a rate of 15%, [1] where these patients are at risk of septic shock, abscesses, and localized gangrene.

The bullous form of erysipelas is rarely reported in the medical literature. A retrospective study conducted in Jerusalem between 1992 and 1996 reported an incidence of 5.2% (26 out of 498 admissions) of patients with erysipelas and cellulitis having a bullous morphology. As for the underlying etiology, other bacterium was found previously, such as Vibrio vulnificus[3] and Streptococcus pneumonia.[4] Bullous disease may also represent a synergistic superinfection with Staphylococcus aureus.[5] In addition, these cases of erysipelas tend to have a protracted course requiring 3 or more weeks of hospitalization and report significant discomfort and pain as a result of the bullae and subsequent erosions. [2]

We describe a fatal case of septic vasculitis due to Pseudomonas bacteremia with a previously unreported bullous erysipelas-like clinical morphology.


   Case Report Top


The patient is a 69-year-old Chinese man with a medical history of hypertension, coronary artery disease and stage 4 chronic kidney disease.

He presented with a history of a diarrheal illness for over 2 months, unassociated with fever, nausea, abdominal pain or jaundice. Within 12 hours of admission, he developed septic shock and was transferred to the Medical Intensive Care Unit. He was initially treated with intravenous ceftriaxone, which was later escalated to meropenem when blood cultures returned positive for P. aeruginosa.

On the second day of admission, sharply defined erythematous plaques developed on the left anterior leg and spread to involve the right anterior leg and lateral aspects of both thighs with patchy involvement over the posterior aspects of both legs. Tense bullae containing serous fluid were formed over the plaques in the subsequent hours [Figure 1]a and b.
Figure 1: (a) Initial sharply defined areas of erythema with early bullae formation (b) Progression of erythema with extension of bullae

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The clinical differential diagnoses were bullous drug reaction, bullous erysipelas, and septic vasculitis. A biopsy was taken from the right thigh. Histological examination revealed partial necrosis of the epidermis with sub-epidermal blistering [Figure 2]a. Microthrombi were present in the dermal blood vessels [Figure 2]b with deeper vessels showing a neutrophilic infiltrate with nuclear dust [Figure 2]c. Of note, Gram-negative bacilli were seen extruding from the vessel wall [Figure 2]d.
Figure 2: (a) There is subepidermal blistering with partial necrosis of the epidermis (H and E stain, Magnification: x200) (b) The underlying superficial dermis reveals blood vessels occluded by fibrin microthrombi (H and E stain; Magnification: x100) (c) Septic vasculitis -A blood vessel in the deep dermis shows neutrophilic infiltrate and fibrinoid necrosis of vessel wall (H and E, Magnification: x400) (d) Gram-negative bacilli can be seen extruding from the blood vessel wall (arrows) (H and E stain; Magnification x600)

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Fluid culture of a needle aspirate taken from a bulla grew P. aeruginosa.

In spite of aggressive fluid resuscitation, high vasopressor support and appropriate antibiotic therapy, he continued to succumb to severe sepsis. His stay in the intensive care unit was complicated by an evolving ST elevation myocardial infarction and he eventually passed away from severe disseminated intravascular coagulopathy with intracranial bleeding.


   Discussion Top


Normally, erysipelas and localized skin infections are easily treated with antibiotics. Bulla formation usually predicts a more aggressive course and often represents a synergistic superinfection with Staphylococcus. [5] This was exemplified in a retrospective study conducted in Jerusalem where their cases of bullous erysipelas required a median of 3 or more weeks of hospitalization and also reported significant discomfort, pain and subsequent erosions as opposed to the typical 1 week of hospitalization in garden-variety erysipelas. [2]

In this patient, his cutaneous morphology was rapidly evolving, with erythema and edema first emerging on the legs in a sharply demarcated fashion that was initially consistent with erysipelas. It was only on the second day of admission that blistering emerged. At that point in time, the patient had received multiple medications, casting suspicion as to whether his cutaneous manifestations were a part of his underlying primary illness or a complication of treatment such as a bullous drug eruption. The progression of septicemia led us to deduce that the tense bullae were possibly due to severe dermal edema caused by a primarily infective process.

The histology was characteristic of septic vasculitis, a variant of small-vessel neutrophilic vasculitis that is typically immune-complex negative and is a direct result of septicemia. [6] This is indeed an unusual finding as the histology of erysipelas classically reveals a neutrophilic infiltrate of the interstitium within a markedly edematous dermis, in the presence of dilated capillaries and lymphatics. [7] Vasculitis is not typically a feature. This patient had cultures positive for P. aeruginosa both from the blood and the blister fluid. This case of septic vasculitis has a highly unusual bullous erysipelas-like clinical morphology. Septic vasculitis normally presents with typical clinical lesions consistent with leukocytoclastic vasculitis (LCV) that are commonly characterized by petechiae, purpura, and vesiculo-pustules. The presence of bulla formation is rare and would normally be hemorrhagic in nature. [6] This non-hemorrhagic bullous variant of septic vasculitis featuring bacterial extrusion from the small blood vessels has not been previously reported. The bullous phenomenon is manifested as a result of the partially-necrotic epidermis being separated from the underlying edematous dermis.

Infective causes of LCV include streptococcal infections and viral hepatitis; an uncommon association with pulmonary tuberculosis has also been previously reported. [8] There have been only two previous reports linking erysipelas with LCV: A patient with erysipelas who had C3-mediated LCV in the lower limbs, [9] and a patient with facial erysipelas who also developed LCV. [10] In both these cases, there were no positive microbial isolates, and the presence of LCV was distant to the area of erysipelas, which was postulated to be secondary to hypersensitivity or an exotoxin-mediated reaction.

Cutaneous manifestations due to pseudomonas infections are known to occur in two ways: Primary cutaneous infections manifesting as folliculitis, balanitis, external otitis, digital intertrigo, and wound infections; and secondary cutaneous infections as a diffuse, maculopapular eruption, clusters of painful vesicles, ecthyma gangrenosum and subcutaneous nodules. [11] Ecthyma gangrenosum has been described to begin as erythematous macules that become indurated and form bullae before sloughing off to reveal a gray-black eschar with surrounding erythema. [12] In this case, the extensive involvement of the indurated plaques over the patient's lower body is inconsistent with the discrete macules and ulcerations of ecthyma gangrenosum. The bullae in this case also sloughed off to reveal exposed dermis and not necrotic ulcerations.



 
   References Top

1.
Jorup-Rönström C. Epidemiological, bacteriological and complicating features of erysipelas. Scand J Infect Dis 1986;18:519-24.  Back to cited text no. 1
    
2.
Guberman D, Gilead LT, Zlotogorski A, Schamroth J. Bullous erysipelas: A retrospective study of 26 patients. J Am Acad Dermatol 1999;41:733-7.  Back to cited text no. 2
    
3.
Penman AD, Lanier DC Jr, Avara WT 3 rd , Canant KE, DeGroote JW, Brackin BT, et al. Vibrio vulnificus wound infections from the Mississippi Gulf coastal waters: June to August 1993. South Med J 1995;88:531-3.  Back to cited text no. 3
    
4.
Corredoira JM, Ariza J, Pallarés R, Carratalá J, Viladrich PF, Rufí G, et al. Gram-negative bacillary cellulitis in patients with hepatic cirrhosis. Eur J Clin Microbiol Infect Dis 1994;13:19-24.  Back to cited text no. 4
    
5.
Krasagakis K, Samonis G, Maniatakis P, Georgala S, Tosca A. Bullous erysipelas: Clinical presentation, staphylococcal involvement and methicillin resistance. Dermatology 2006;212:31-5.  Back to cited text no. 5
    
6.
Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol 2008;9:71-92.  Back to cited text no. 6
    
7.
Celestin R, Brown J, Kihiczak G, Schwartz RA. Erysipelas: A common potentially dangerous infection. Acta Dermatovenerol Alp Pannonica Adriat 2007;16:123-7.  Back to cited text no. 7
    
8.
Mendiratta V, Gaur N, Sud R, Agarwal S, Chander R. Cutaneous leucocytoclastic vasculitis and pulmonary tuberculosis: An uncommon association. Indian J Dermatol 2014;59:614-5.  Back to cited text no. 8
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9.
Margolin L, Feinmesser M, Hodak E. Leukocytoclastic vasculitis associated with bullous erysipelas. Int J Dermatol 2003;42:300-1.  Back to cited text no. 9
    
10.
Goel G, Fomberstein B. Leukocytoclastic vasculitis secondary to erysipelas: An unusual case of noncryoglobulinemic vasculitis in a hepatitis C patient. J Clin Rheumatol 2013;19:282-3.  Back to cited text no. 10
    
11.
Silvestre JF, Betlloch MI. Cutaneous manifestations due to Pseudomonas infection. Int J Dermatol 1999;38:419-31.  Back to cited text no. 11
    
12.
Greene SL, Su WP, Muller SA. Ecthyma gangrenosum: Report of clinical, histopathologic, and bacteriologic aspects of eight cases. J Am Acad Dermatol 1984;11:781-7.  Back to cited text no. 12
    

What is new?
The bullous erysipelas-like lesions seen in this patient represents a rare, previously unreported manifestation of septic vasculitis due to Pseudomonas infection.


    Figures

  [Figure 1], [Figure 2]



 

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