scholarly journals The Role of IL-13, IL-15 and Granulysin in the Pathogenesis of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

2021 ◽  
Vol 27 ◽  
pp. 107602962095083
Author(s):  
Michael Sadek ◽  
Omer Iqbal ◽  
Fakiha Siddiqui ◽  
Sean Till ◽  
Melissa Mazariegos ◽  
...  
Keyword(s):  
Skin Biopsy ◽  
Toxic Epidermal Necrolysis ◽  
Lichen Planus ◽  
Stevens Johnson Syndrome ◽  
Corneal Epithelial ◽  
Johnson Syndrome ◽  
Tnf Α ◽  
Normal Human ◽  
Human Corneal Epithelial Cells

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are Severe Cutaneous Adverse Reactions (SCARS) characterized by fever and mucocutaneous lesions leading to necrosis and sloughing of the epidermis. Conjunctival lesions are reported in 85% of patients. The pathogenesis of SJS/TEN/SCARS is not completely understood. It is hypothesized that IL-13, IL-15 and Granulysin expressed in plasma and skin may play a role. We measured the circulating levels of these cytokines in the plasma using ELISA and their expression in the skin using immunofluorescence microscopy. A total of 12 SJS/TEN skin biopsy samples (8 SJS, 2 SJS/TEN overlap and 2 TEN) were analyzed. Biopsy samples from patients with Lichen Planus (an inflammatory condition of the skin and mucous membranes) served as controls. Studies were also performed in human corneal epithelial cells where expression of these cytokines were measured following a challenge with TNF-α (0, 1, 10 and 100 ng/ml). The intensity of immunofluorescence was measured Using Imaris® software. The results showed significantly increased expression of these cytokines in the skin biopsy samples as measured by the average intensities of IL-13 (6.1 x 133.0 ± 4.231 x 10^8), and Granulysin (4.2 x 123.0 ± 4.231 x 10^8) compared to Lichen planus control (3.0 x 123.0 ±1.62 x 10^5). Increased expression of IL-13 and IL-15 were noted in cell culture studies and in the plasma samples when compared to Normal Human Plasma as controls. It is concluded that IL-13, IL-15 and Granulysin play a role in the pathogenesis of SJS/TEN.

scholarly journals Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 612
Author(s):  
Akito Hasegawa ◽  
Riichiro Abe
Keyword(s):  
Cell Death ◽  
Toxic Epidermal Necrolysis ◽  
Epidermal Cell ◽  
Therapeutic Options ◽  
Formyl Peptide Receptor ◽  
Stevens Johnson Syndrome ◽  
Recent Advances ◽  
Johnson Syndrome ◽  
Tnf Α ◽  
Severity Prediction

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by detachment of the epidermis and mucous membrane. SJS/TEN are considered to be on the same spectrum of diseases with different severities. They are classified by the percentage of skin detachment area. SJS/TEN can also cause several complications in the liver, kidneys, and respiratory tract. The pathogenesis of SJS/TEN is still unclear. Although it is difficult to diagnose early stage SJS/TEN, biomarkers for diagnosis or severity prediction have not been well established. Furthermore, optimal therapeutic options for SJS/TEN are still controversial. Several drugs, such as carbamazepine and allopurinol, are reported to have a strong relationship with a specific human leukocyte antigen (HLA) type. This relationship differs between different ethnicities. Recently, the usefulness of HLA screening before administering specific drugs to decrease the incidence of SJS/TEN has been investigated. Skin detachment in SJS/TEN skin lesions is caused by extensive epidermal cell death, which has been considered to be apoptosis via the Fas-FasL pathway or perforin/granzyme pathway. We reported that necroptosis, i.e. programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, and its interaction with the formyl peptide receptor 1 induce necroptosis. Several diagnostic or prognostic biomarkers for SJS/TEN have been reported, such as CCL-27, IL-15, galectin-7, and RIP3. Supportive care is recommended for the treatment of SJS/TEN. However, optimal therapeutic options such as systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and TNF-α antagonists are still controversial. Recently, the beneficial effects of cyclosporine and TNF-α antagonists have been explored. In this review, we discuss recent advances in the pathophysiology and management of SJS/TEN.

scholarly journals Association of the HLA-B alleles with carbamazepine-induced Stevens–Johnson syndrome/toxic epidermal necrolysis in the Javanese and Sundanese population of Indonesia: the important role of the HLA-B75 serotype

2017 ◽  
Vol 18 (18) ◽  
pp. 1643-1648 ◽  
Author(s):  
Rika Yuliwulandari ◽  
Erna Kristin ◽  
Kinasih Prayuni ◽  
Qomariyah Sachrowardi ◽  
Franciscus D Suyatna ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Stevens Johnson Syndrome ◽  
Johnson Syndrome

TOXIC EPIDERMOLYSIS CAUSED BY ANTIEPILEPTIC DRUGS: CLINICAL OBSERVATIONS

2021 ◽  
Vol 100 (1) ◽  
pp. 282-287
Author(s):  
M.A. Ufimtseva ◽  
◽  
M.A. Zakharov ◽  
O.Yu. Averyanov ◽  
O.V. Kozhevnikova ◽  
...  
Keyword(s):  
Adverse Drug Reaction ◽  
Drug Reaction ◽  
Toxic Epidermal Necrolysis ◽  
Antiepileptic Drugs ◽  
Stevens Johnson Syndrome ◽  
International Literature ◽  
Emergency Medical Care ◽  
Clinical Observations ◽  
Johnson Syndrome

Toxic epidermal necrolysis (Lyell's syndrome) and Stevens–Johnson syndrome are severe types of toxicodermia and require emergency medical care. The pathophysiology of toxicoderma is associated with an adverse drug reaction. The article provides data from Russian and international literature on the role of antiepileptic drugs in these diseases occurrence. It also presents clinical cases of toxic epidermal necrolysis in children caused by anticonvulsants intake.

501 Burn Center Referral of Patients with Suspected Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN)

2020 ◽  
Vol 41 (Supplement_1) ◽  
pp. S87-S87
Author(s):  
Julie A Rizzo ◽  
David S Lidwell ◽  
Leopoldo C Cancio
Keyword(s):  
Skin Biopsy ◽  
Toxic Epidermal Necrolysis ◽  
Disease Process ◽  
Stevens Johnson Syndrome ◽  
Burn Center ◽  
Johnson Syndrome ◽  
Number Of Patients ◽  
Wide Range ◽  
Burn Unit ◽  
Life Threatening

Abstract Introduction Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but potentially life-threatening, as such referral of these patients to a burn center is appropriate. However, patients with suspected SJS/TEN are often referred to the Burn Center and are found to not actually be suffering from the disease process in question. This inefficient referral process warrants further examination to identify pre-arrival those patients who are appropriate for transfer. Methods As an approved PI project we examined the records of all patients referred to our Burn Center for suspicion of SJS/TEN for the time period 2016–2018. We analyzed the corresponding data to in an attempt to more effectively identify patients with SJS/TEN and prevent unnecessary Burn Center transfers. Results Of 84 patients referred for suspected SJS/TEN 32 received confirmatory diagnosis with skin biopsy after transfer (38%). The average length of stay was 8 ICU days and 14 hospital days versus 3.6 and 9.6 days, respectively, for patients with a negative diagnosis. The mortality rate of SJS/TEN patients was 12.5% (4/32). In addition to SJS/TEN, a wide range of skin conditions were identified among referred patients, many of whom also required hospitalization, including BICU care. The various diagnoses included: Drug eruptions (14%), psoriasis (6%), dermatitis (6%), erythema multiforme (2%), lupus erythematosus (2%) and generalized exanthematous pustulosis (2%). The remainder of patients had miscellaneous or nonspecific conditions (28%). Conclusions SJS/TEN is a potentially life threatening disease often requiring hospitalization in a Burn Intensive Care Unit (BICU). However, many other disease processes have similar presentations and may also be appropriate for Burn Unit care. Without a confirmatory skin biopsy prior to referral a large number of patients are transferred to the BICU unnecessarily. Applicability of Research to Practice In the absence of skin biopsy capabilities at the referring facility an algorithm using common characteristics of actual SJS/TEN patients may improve the accuracy of pre-referral diagnosis. Additionally, this data underscores the importance of dermatology support to the Burn Unit in diagnosing and treating desquamating skin disorders.

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