|Year : 2018 | Volume
| Issue : 1 | Page : 1-2
Cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis: Current scenario
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
|Date of Web Publication||19-Jun-2018|
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
Source of Support: None, Conflict of Interest: None
|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
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.” 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. 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.,,,,, 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.,
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.
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