scholarly journals Stevens-Johnson syndrome and toxic epidermal necrolysis: a review

2019 ◽  
Vol 7 (6) ◽  
pp. 2470
Author(s):  
Rodrigo Banegas Ruiz ◽  
Alan I. Valderrama Treviño ◽  
Gómez Mendoza F. F. ◽  
Baca Domínguez C. R. ◽  
Campos Angulo G. ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
B Lymphocytes ◽  
Body Surface ◽  
Cytotoxic T Cells ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Johnson Syndrome ◽  
Diffuse Erythema ◽  
Cutaneous Reactions ◽  
Therapeutic Alternatives

Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are considered a single entity with variability in the extent of the lesions, characterized by erythema multiforme that may involve mucosa. Severe cutaneous reactions secondary to medications are classified according to the area of epidermal detachment. The activation of cytotoxic T cells and macrophages is mediated mainly by IL-2 and interferon gamma secreted by Th1 lymphocytes, and the activation of eosinophils and B lymphocytes in IgE is mediated by secreted IL-4, IL-5, IL-10 and IL13 by B lymphocytes. The topography of SJS is predominantly central, affecting the trunk and sometimes a generalized dissemination is shown that affects a body surface area of less than 10%, characterized by irregular violaceous erythematous macules of target shooting, which can form confluent blisters. TEN is characterized by a skin detachment greater than 30% of the body surface, whose predominant lesion is diffuse erythema with individual macules, which give rise to detachment surfaces greater than 5 cm. The treatment is symptomatic, nonspecific, and aimed at avoiding complications, carried out in specialized intensive care units, due to ignorance of the pathogenesis. Integral management with different therapeutic alternatives can represent a crucial part in the multisystemic management of SJS and TEN.

scholarly journals Serum Granulysin in Differentiation of Stevens-Johnson Syndrome/toxic Epidermal Necrolysis and Erythema Multiforme

2020 ◽  
Vol 8 (B) ◽  
pp. 381-388
Author(s):  
Tran Thi Huyen ◽  
Pham Dinh Hoa ◽  
Trinh Minh Trang ◽  
Riichiro Abe ◽  
Nguyen Van Thuong ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Body Surface ◽  
Erythema Multiforme ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Enzyme Linked Immunosorbent Assay ◽  
Drug Reactions ◽  
Skin Manifestation ◽  
Johnson Syndrome ◽  
Life Threatening

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening drug reactions, which lead to massive epidermal necrolysis. Granulysin plays an important role as a key mediator for keratinocyte apoptosis in these conditions. Erythema multiforme (EM) may have skin manifestation similar to SJS/TEN. AIMS: The aim of the study was to compare serum granulysin levels in patients with SJS/TEN and EM as well as to investigate a possible association between serum granulysin levels and the severity of SJS/TEN. METHODS: In total, 48 patients with SJS/TEN, 43 patients with EM, and 20 health controls (HCs) were enrolled. We measured serum granulysin levels using enzyme-linked immunosorbent assay. RESULTS: The average level of serum granulysin in the SJS/TEN patients was 23.0 ng/ml (range 1.2–144.6 ng/ml), significantly higher than that of EM group (20.1 ng/ml; range 8.5–121 ng/ml, p < 0.05) and HCs group (20.8 ng/ml; range 10.1–46.7 ng/ml, p < 0.05). Of 48 SJS/TEN patients, the 25 samples collected <6 days after onset showed higher level of serum granulysin (27.7 ng/ml; range 2.5–144.6 ng/ml) than those collected ≥6 days after onset (17.9 ng/ml; range 1.2–59 ng/ml; p > 0.05). No significant correlation was found between serum granulysin levels and the body surface area affected and the modified-SCORTEN. At the day of re-epithelialization, serum granulysin levels were not different compared with those at the day of hospitalization. CONCLUSIONS: Serum granulysin levels are significantly higher in SJS/TEN group than in EM group. After the onset, serum granulysin levels in patients with SJS/TEN are not a good biomarker to evaluate the severity of the diseases.

scholarly journals Phenytoin induced toxic epidermal necrolysis: a case report

2019 ◽  
Vol 8 (6) ◽  
pp. 1448
Author(s):  
Sandipan Barkakaty ◽  
Girish K.
Keyword(s):  
Oral Cavity ◽  
Toxic Epidermal Necrolysis ◽  
Past History ◽  
Clinical History ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Medicine Department ◽  
Johnson Syndrome ◽  
History Of ◽  
Emergency Medicine Department

Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), are severe idiosyncratic reactions characterized by fever and mucocutaneous lesions leading to necrosis and sloughing of the epidermis. The usage of anticonvulsants like carbamazepine, phenytoin, lamotrigine, phenobarbital are associated with high risk for occurrence of TEN. We present a case of toxic epidermal necrolysis in a 30 year old female probably induced by phenytoin. A 30 year old female was admitted to the emergency medicine department of KIMS hospital, Bengaluru. Lesions over the lips and oral cavity, multiple fluid filled blisters were present diffusely all over the body. Patient had a past history of oral cavity lesions with injection phenytoin. Patient is a known epileptic of over 12 years and was on treatment. Patient had a seizure attack 3 days back and visited nearby hospital and did not inform the doctor of her allergy to phenytoin. Patient was given inj phenytoin after which she developed oral lesions and also presented with fluid filled bullae all over the body. A diagnosis of toxic epidermal necrolysis was made based on clinical history and Scoreten score and was treated with betadine wash, fluconazole and antibiotics .The lesions improved significantly with the above management and patient recovered enough to be discharged from the hospital after 5 days. Severe and serious reactions such as toxic epidermal necrolysis can be caused by commonly used drugs like phenytoin.

Neutrophils initiate and exacerbate Stevens-Johnson syndrome and toxic epidermal necrolysis

2021 ◽  
Vol 13 (600) ◽  
pp. eaax2398
Author(s):  
Manao Kinoshita ◽  
Youichi Ogawa ◽  
Natsumi Hama ◽  
Inkin Ujiie ◽  
Akito Hasegawa ◽  
...  
Keyword(s):  
T Cells ◽  
Immune Responses ◽  
Toxic Epidermal Necrolysis ◽  
Adverse Drug Reactions ◽  
Cytotoxic T Cells ◽  
Formyl Peptide Receptor ◽  
Stevens Johnson Syndrome ◽  
Lipocalin 2 ◽  
Drug Reactions ◽  
Johnson Syndrome

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous adverse drug reactions characterized by massive epidermal detachment. Cytotoxic T cells and associated effector molecules are known to drive SJS/TEN pathophysiology, but the contribution of innate immune responses is not well understood. We describe a mechanism by which neutrophils triggered inflammation during early phases of SJS/TEN. Skin-infiltrating CD8+ T cells produced lipocalin-2 in a drug-specific manner, which triggered the formation of neutrophil extracellular traps (NETs) in early lesional skin. Neutrophils undergoing NETosis released LL-37, an antimicrobial peptide, which induced formyl peptide receptor 1 (FPR1) expression by keratinocytes. FPR1 expression caused keratinocytes to be vulnerable to necroptosis that caused further release of LL-37 by necroptotic keratinocytes and induced FPR1 expression on surrounding keratinocytes, which likely amplified the necroptotic response. The NETs-necroptosis axis was not observed in less severe cutaneous adverse drug reactions, autoimmune diseases, or neutrophil-associated disorders, suggesting that this was a process specific to SJS/TEN. Initiation and progression of SJS/TEN keratinocyte necroptosis appear to involve a cascade of events mediated by innate and adaptive immune responses, and understanding these responses may contribute to the identification of diagnostic markers or therapeutic targets for these adverse drug reactions.

scholarly journals Toxic epidermal necrolysis (Lyell's syndrome) in a patient with HIV infection.

2021 ◽  
Author(s):  
Darya Popova ◽  
S L Voznesenskiy
Keyword(s):  
Hiv Infection ◽  
Toxic Epidermal Necrolysis ◽  
Clinical Manifestations ◽  
Staphylococcal Infection ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Johnson Syndrome ◽  
Immunodeficiency Virus ◽  
Lyell’S Syndrome ◽  
Lyell's Syndrome

Lyell's syndrome or toxic epidermal necrolysis (TEN) is more common with human immunodeficiency virus (HIV) infection than in the general population. The article describes a clinical case of Lyell's syndrome in an HIV-infected patient who was first prescribed ART in combination with valganciclovir. The diagnosis was made on the basis of characteristic clinical manifestations and the exclusion of another similar pathology. On the background of the therapy, the rash regressed, the areas of damaged skin became epithelialized, the body temperature returned to normal. A differential diagnosis was made with measles, Stevens-Johnson syndrome, staphylococcal infection. Against the background of the therapy, the patient's condition was positive.

scholarly journals IVIG and under Burn Unit Care Yield Favorable Outcomes in Pediatric Patients with Toxic Epidermal Necrolysis: A Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Tareq Z. Alzughayyar ◽  
Wasim Noureddin Ibrahim Hamad ◽  
Eman A. S. Abuqweider ◽  
Bilal Nabeel Mohammad Alqam ◽  
Sadi A. Abukhalaf ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Severe Form ◽  
Stevens Johnson Syndrome ◽  
Care Level ◽  
Johnson Syndrome ◽  
Management Protocol ◽  
Burn Unit ◽  
Threatening Condition ◽  
Life Threatening ◽  
Cutaneous Reactions

Body reactions to drugs can manifest as Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). TEN is the most severe form of cutaneous reactions with an incidence rate of 1-2 per million cases per year. Despite TEN being a critical and life-threatening condition, there is little to no evidence of clear management protocol. We reported a 5-year-old male child who presented with lamotrigine-induced TEN and was successfully treated with intravenous immune globulin (IVIG) with a burn unit care level, while TEN treatment with IVIG is an appropriate approach with predictable good outcomes, burn unit care is also effective in creating highly favorable effects. Upon reviewing the literature, several studies indicate that TEN patients treated with the combination of IVIG and burn unit care lead to decreased levels of morbidity and mortality than when treated with IVIG or burn unit care alone. Therefore, treatment involving both IVIG and burn unit care should be considered for TEN patients.

Toxic epidermal necrolysis associated with pembrolizumab

2019 ◽  
Vol 26 (5) ◽  
pp. 1259-1265 ◽  
Author(s):  
Zhuo Ran Cai ◽  
Julie Lecours ◽  
Jean-Philippe Adam ◽  
Isabelle Marcil ◽  
Normand Blais ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Surface Area ◽  
Body Surface Area ◽  
Body Surface ◽  
Stevens Johnson Syndrome ◽  
First Case ◽  
Johnson Syndrome ◽  
Programmed Death ◽  
Mucosal Involvement ◽  
Programmed Death 1

Introduction Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous drug eruptions characterized by epidermal detachment. Pembrolizumab is a monoclonal antibody that binds to the programmed death-1 receptor, and it has been associated with numerous cutaneous adverse side-effects, including Stevens-Johnson syndrome. Case report We describe a 63-year-old male with metastatic lung adenocarcinoma who developed a rapidly progressing maculopapular rash three days after a first dose of pembrolizumab. On day 16, the rash affected more than 80% of body surface area with detachment of large sheets of necrolytic epidermis in 30–40% of body surface area. However, the patient only presented with mild mucosal involvement. Histopathologic examination of a skin biopsy showed a subepidermal blister with overlying prominent full thickness epidermal keratinocytic necrosis and a superficial perivascular infiltrate of lymphocytes. A toxic epidermal necrolysis secondary to pembrolizumab was then diagnosed. Management and outcome: In addition to supportive cares, the patient received corticosteroids and cyclosporine. The patient responded rapidly to the immunosuppressant therapy, and nearly complete re-epithelialization was achieved 24 days after the start of the reaction. Discussion In our review of the literature, 15 other cases of Stevens-Johnson syndrome/toxic epidermal necrolysis were reported with programmed death-1/programmed cell death ligand-1 inhibitors. To our knowledge, this is the first case of toxic epidermal necrolysis secondary to pembrolizumab published in the literature. The American Society of Clinical Oncology guidelines suggest that cyclosporine, in addition to corticosteroids, be initiated when toxic epidermal necrolysis is suspected. Clinicians should be aware of this rare dermatological emergency with the increasing use of pembrolizumab in oncology.

Levetiracetam Treatment Induced Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

2017 ◽  
Vol 06 (04) ◽  
pp. 182-185 ◽  
Author(s):  
Fazıl Orhan ◽  
Mehtap Abul ◽  
Mehmet Mutlu ◽  
Sevim Şahin ◽  
Ali Cansu ◽  
...  
Keyword(s):  
Risk Factor ◽  
Toxic Epidermal Necrolysis ◽  
Clinical Picture ◽  
Stevens Johnson Syndrome ◽  
Cutaneous Eruption ◽  
Mucosal Surfaces ◽  
Johnson Syndrome ◽  
Carbamazepine Therapy ◽  
Initial Improvement ◽  
Cutaneous Reactions

AbstractStevens–Johnson syndrome and toxic epidermal necrolysis are severe mucocutaneous reactions involving at least two mucosal surfaces and resulting in cutaneous eruption. They are frequently associated with infection and drug use. The best-known infectious cause is Mycoplasma pneumoniae, while antiepileptics are often among the drugs giving rise to these conditions. We describe two patients with suspected Stevens–Johnson syndrome and cutaneous eruption, which were primarily attributed to carbamazepine therapy. Another important shared feature in these two cases is that following an initial improvement in lesions in both patients, an increase in lesions and worsening of clinical picture after initiation of levetiracetam therapy was noted. One of the patients was found to be HLA-B 1502 positive, which is a known risk factor for carbamazepine-induced Stevens–Johnson syndrome. Levetiracetam therapy, which is regarded as safe in terms of cutaneous reactions, if chosen following such reactions due to another antiepilepsy medication, may be capable of reactivating Stevens–Johnson syndrome and toxic epidermal necrolysis as was seen in our patients.

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