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 Table of Contents  
EDITORIAL
Year : 2018  |  Volume : 3  |  Issue : 1  |  Page : 1-2

Cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis: Current scenario


Department of Dermatology, Venereology and Leprosy, Shri B M Patil Medical College, Hospital and Research Center, BLDE (Deemed to be University), Vijayapur - 586 103, Karnataka, India

Date of Web Publication19-Jun-2018

Correspondence Address:
Dr. Keshavmurthy A Adya
Department of Dermatology, Venereology and Leprosy, Shri B M Patil Medical College, Hospital and Research Center, BLDE (Deemed to be University), Vijayapur - 586 103, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/bjhs.bjhs_15_18

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How to cite this article:
Adya KA. Cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis: Current scenario. BLDE Univ J Health Sci 2018;3:1-2

How to cite this URL:
Adya KA. Cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis: Current scenario. BLDE Univ J Health Sci [serial online] 2018 [cited 2018 Nov 17];3:1-2. Available from: http://www.bldeujournalhs.in/text.asp?2018/3/1/1/234639



Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent the acute, severe, and life-threatening forms of adverse cutaneous drug reactions clinically presenting as “acute skin failure.”[1] The cornerstone in the management of these conditions is the supportive treatment promptly addressing the various cutaneous and systemic consequences of acute skin failure by a multidisciplinary approach. In addition, specific treatment modalities also need to be considered to halt the disease progression. Many specific treatment modalities have been employed in SJS/TEN including systemic steroids, intravenous immunoglobulins (IVIG), thalidomide, and cyclosporine. The main issue is the lack of objective validation for the use of any of these modalities due to the lack of blinded randomized controlled trials for various practical and ethical reasons. Hence, currently, the use of any specific treatment modality in SJS/TEN is at the discretion of the treating physician.

 Stevens-Johnson syndrome More Details and TEN are T-cell-mediated disorders characterized by widespread cutaneous necrosis and inflammation. As the mechanism of action of cyclosporine is mainly centered on the suppression of T lymphocyte proliferation and the cytokines released by them, it appears to be a better option theoretically in SJS/TEN. This is particularly of significance when compared to the commonly used systemic corticosteroids which have a rather nonspecific, broad-spectrum immunosuppressive function which may increase the susceptibility to secondary infections in patients with SJS/TEN who have an already compromised cutaneous barrier function and systemic derangements.

The possible role of cyclosporine in SJS/TEN was proposed way back in 1986 based on the role of T-cells in its pathogenesis and its striking resemblance with graft versus host disease.[2] The drug has been used by many authors either as monotherapy, concurrently with systemic steroids, or sequentially following initial systemic steroids. Based on the available evidence, cyclosporine seems to produce an early halt in the progression of the disease and promotes early reepithelialization when compared to other modalities (systemic steroids and IVIG). The drug also appears to be well tolerated even by acutely ill patients and is associated with low septic as well as drug-related complications. When used concurrently with steroids, it allows early tapering of the latter and prevents the complications associated with it. Overall, the mortality appears to be lower with cyclosporine compared to other modalities.[3],[4],[5],[6],[7],[8] In author's unpublished experience as well, the drug appears to be more effective compared to systemic corticosteroids, even in pediatric age group.

Unfortunately, the evidence is scarce both in terms of quantity, as well as in terms of “level of evidence.” Most of the available evidence predominantly comprises short case series and open-label trials. This is understandable as practical and ethical issues preclude carrying out randomized controlled trials of such acute, severe, and life-threatening illnesses. Furthermore, the rarity of the disorder makes it difficult in achieving an adequate sample size for the results of such studies, if carried out, to be statistically relevant. Hence, objective validation of the use of cyclosporine is lacking.

One must also be mindful of the fact that the role of any specific treatment modality in SJS/TEN is only second to adequate and prompt supportive measures which are actually the lifesaving measures in these patients. Hence, the latest guidelines also advocate prompt and meticulous conservative management as the cornerstone in the management of SJS/TEN and the call on the administration of a specific treatment modality to be taken by the treating multispecialty team.[9],[10]

To conclude, the mainstay of management of SJS/TEN is meticulous supportive therapy. As a specific therapeutic modality, although cyclosporine appears to have an upper hand in many aspects over other modalities based on the available albeit little and hierarchically inferior levels of evidence, it cannot be concluded if it is objectively superior to others. Large case series and randomized controlled trial are necessary to objectively validate the role and utility of the drug in SJS/TEN.



 
  References Top

1.
Inamadar AC, Palit A. Acute skin failure: Concept, causes, consequences and care. Indian J Dermatol Venereol Leprol 2005;71:379-85.  Back to cited text no. 1
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2.
Biren CA, Barr RJ. Dermatologic applications of cyclosporine. Arch Dermatol 1986;122:1028-32.  Back to cited text no. 2
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3.
Singh GK, Chatterjee M, Verma R. Cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis and retrospective comparison with systemic corticosteroid. Indian J Dermatol Venereol Leprol 2013;79:686-92.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Conner CD, McKenzie E, Owen CE, Callen JP. The use of cyclosporine for Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum at the university of Louisville: A case series and literature review. Dermatol Online J 2018;24. pii: 13030/qt6d56n4j8.  Back to cited text no. 4
    
5.
Kirchhof MG, Miliszewski MA, Sikora S, Papp A, Dutz JP. Retrospective review of Stevens-Johnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol 2014;71:941-7.  Back to cited text no. 5
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6.
Aihara Y, Ito R, Ito S, Aihara M, Yokota S. Toxic epidermal necrolysis in a child successfully treated with cyclosporin A and methylprednisolone. Pediatr Int 2007;49:659-62.  Back to cited text no. 6
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7.
Valeyrie-Allanore L, Wolkenstein P, Brochard L, Ortonne N, Maître B, Revuz J, et al. Open trial of ciclosporin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol 2010;163:847-53.  Back to cited text no. 7
    
8.
Ng QX, De Deyn ML, Venkatanarayanan N, Ho CY, Yeo WS. A meta-analysis of cyclosporine treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis. J Inflamm Res 2018;11:135-42.  Back to cited text no. 8
    
9.
Gupta LK, Martin AM, Agarwal N, D'Souza P, Das S, Kumar R, et al. Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: An Indian perspective. Indian J Dermatol Venereol Leprol 2016;82:603-25.  Back to cited text no. 9
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10.
Creamer D, Walsh SA, Dziewulski P, Exton LS, Lee HY, Dart JK, et al. U.K. Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol 2016;174:1194-227.  Back to cited text no. 10
    




 

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