Stevens–Johnson syndrome and toxic epidermal necrolysis: a 10-year experience in a burns unit

2021 ◽  
Vol 30 (6) ◽  
pp. 492-496
Author(s):  
Khosrow S Houschyar ◽  
Christian Tapking ◽  
Mimi R Borrelli ◽  
Ina Nietzschmann ◽  
Behrus Puladi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Toxic Epidermal Necrolysis ◽  
Retrospective Review ◽  
Mortality Rates ◽  
Intravenous Immunoglobulins ◽  
Stevens Johnson Syndrome ◽  
Control Group ◽  
High Dose ◽  
Johnson Syndrome ◽  
Patient Reported

Objective: Stevens-Johnson syndrome (SJS) and its more severe counterpart, toxic epidermal necrolysis (TEN), are skin hypersensitivity reactions defined by epidermal blistering and necrosis. The exact pathophysiology of SJS/TEN is yet to be deciphered, but a number of risk factors have been identified including adverse drug reactions. The diagnosis of SJS/TEN is made on a clinical basis, and treatment consists of supportive care and occasionally immunosuppressants, such as cyclosporin, high-dose intravenous immunoglobulins and/or corticosteroids. Mortality rates can reach 20–25% in adults but are reduced with early intervention. To identify optimal treatment regimens, to better understand the patient cohort affected, and to help identify key risk factors for mortality, we report our experience with the treatment and management of SJS/TEN patients. Methods: A retrospective review of consecutive patients with SJS and/or TEN admitted to a single burns centre in Germany, between 2008 and 2018, was conducted. The primary outcomes of demographics, clinical course, treatment and patient-reported outcomes were recorded and compared with a control group of patients with burns without a diagnosis of SJS/TEN. Results: A total of 23 patients with SJS/TEN met the inclusion criteria: 17 (74%) with TEN; four (17%) with SJS/TEN overlap; and two (9%) with SJS. Of the patients, 14 (61%) were female and nine (39%) were male. Patient age ranged from 32–78 years (mean: 52 years). A matched cohort of 23 patients with burns served as the control group. All patients received standard of care with a multidisciplinary team. Compared with the control group, SJS/TEN patients had higher mortality rates (n=6, 26% versus n=8, 35%, respectively). The average age of death was 69 years in SJS/TEN patients versus 63 years in control group patients. Age and SCORTEN scores were significant predictors of mortality. Conclusions: SJS and TEN are rare but extreme reactions of the skin and mucosa, associated with high disease mortality rates. This 10-year single-centre retrospective review contributes to the bank of information for reviews evaluating the management of SJS/TEN patients.

scholarly journals Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

2021 ◽  
Vol 8 ◽  
Author(s):  
Ming-Hsiu Hsieh ◽  
Tomoya Watanabe ◽  
Michiko Aihara
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Ivig Therapy ◽  
Japanese Patients ◽  
Mortality Rates ◽  
Pulse Therapy ◽  
Stevens Johnson Syndrome ◽  
High Dose ◽  
Methylprednisolone Pulse ◽  
Johnson Syndrome ◽  
Additional Therapy

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions characterized by necrosis of the skin and mucus membranes, and are mainly caused by medication and infections. Although the exact pathomechanism of SJS/TEN remains unclear, keratinocyte death is thought to be triggered by immune reactions to these antigens. While there is no established therapy for SJS/TEN, corticosteroids and intravenous immunoglobulin (IVIG) have been utilized as immunomodulator. We previously conducted a study to evaluate the efficacy of IVIG therapy in Japanese patients with SJS/TEN. IVIG was administered at a dosage of 400 mg/kg/day for 5 consecutive days as an additional therapy with systemic steroids. Prompt amelioration was observed in seven of the eight patients. All patients survived without sequelae. Recently, we retrospectively analyzed 132 cases of SJS/TEN treated in our two hospitals. The mortality rates in the patients treated with methylprednisolone pulse were 0% (0/31) for SJS and 7.0% (3/43) for TEN, and 0% (0/10) in the TEN patients treated with methylprednisolone pulse in combination with IVIG. These results suggest that early treatment with high-dose steroids, including methylprednisolone pulse therapy, and IVIG together with corticosteroids are possible therapeutic options to improve the prognosis of SJS/TEN.

scholarly journals Toxic Epidermal Necrolysis/Stevens-Johnson Syndrome at A University Hospital in Saudi: Causative Factors and Outcomes

2022 ◽  
Author(s):  
Amal A Kokandi
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Small Sample Size ◽  
Intravenous Immunoglobulins ◽  
Small Sample ◽  
University Hospital ◽  
Stevens Johnson Syndrome ◽  
Johnson Syndrome ◽  
Causative Agents ◽  
Life Threatening ◽  
King Abdulaziz University

Abstract Introduction:Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, life-threatening conditions caused mainly by drugs. Their management relies on the withdrawal of the culprit medication and supportive measures. Different pharmacotherapies have varied effects. However, data related to TEN and SJS in Saudi is limited. This study aimed to identify the causative agents, associated factors, and outcomes of TEN/SJS cases admitted to a teaching hospital (King Abdulaziz University) in Jeddah during the last 10 years.Methods: We retrospectively analyzed the data of TEN/SJS patients admitted to the hospital over the last 10 years.Results: We identified 12 patients with TEN/SJS. Of these, nine survived the condition and were discharged. The culprit medication was identified in eight of them, including antibiotics in six cases and Tegretol and allopurinol in one case each. Most of the patients received systemic steroids and intravenous immunoglobulins.Conclusion: TEN/SJS is mainly caused by medications of which antibiotics are the most implicated. Consistent with other studies, the mortality rate associated with TEN/SJS in Saudi is 25%. Limitations: restricted to a single center and small sample size.

Suspected Lamotrigine-Induced Toxic Epidermal Necrolysis

1997 ◽  
Vol 31 (6) ◽  
pp. 720-723 ◽  
Author(s):  
Julie J Chaffin ◽  
Steven M Davis
Keyword(s):  
Valproic Acid ◽  
Toxic Epidermal Necrolysis ◽  
Case Reports ◽  
Stevens Johnson Syndrome ◽  
Complex Partial Seizures ◽  
Skin Reactions ◽  
Johnson Syndrome ◽  
Patient Reported ◽  
History Of ◽  
Relationship Of

OBJECTIVE: To describe a patient who developed toxic epidermal necrolysis (TEN) possibly secondary to lamotrigine use. CASE SUMMARY: A 74-year-old white man with a history of probable complex partial seizures was admitted to the neurology service for a prolonged postictal state. His antiepileptic regimen was changed while he was in the hospital to include lamotrigine. After 19 days of hospitalization and 14 days of lamotrigine therapy, the patient became febrile. The next day he developed a rash which progressed within 4 days to TEN, diagnosed by skin biopsy. All suspected drugs were discontinued, including lamotrigine. The patient was treated with hydrotherapy in the burn unit. His symptoms improved and he was discharged from the hospital 26 days after the rash developed. DISCUSSION: During lamotrigine's premarketing clinical trials, the manufacturer reported several cases of Stevens-Johnson syndrome and TEN. There are several published case reports of lamotrigine-induced severe skin reactions. All of these reports included patients being treated with both valproic acid and lamotrigine. Our patient was exposed to phenytoin, carbamazepine, clindamycin, and lamotrigine, but not valproic acid. The patient reported prior use of phenytoin with no skin rash. Carbamazepine was the antiepileptic drug the patient was maintained on prior to his hospital admission, and the symptoms of TEN resolved while he was still receiving carbamazepine. The patient received only two doses of clindamycin, which makes this agent an unlikely cause of TEN. CONCLUSIONS: Because of the temporal relationship of the onset of the patient's rash and several drugs that are known to cause severe rashes, it is not certain which drug was the definite culprit. However, based on the evidence from the literature, lamotrigine appears to be the causative agent.

Medications as Risk Factors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children: A Pooled Analysis

PEDIATRICS ◽  
2009 ◽  
Vol 123 (2) ◽  
pp. e297-e304 ◽  
Author(s):  
N. Levi ◽  
S. Bastuji-Garin ◽  
M. Mockenhaupt ◽  
J.-C. Roujeau ◽  
A. Flahault ◽  
...  
Keyword(s):  
Risk Factors ◽  
Toxic Epidermal Necrolysis ◽  
Pooled Analysis ◽  
Stevens Johnson Syndrome ◽  
Johnson Syndrome

scholarly journals DRUG-INDUCED STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS

2016 ◽  
Vol 65 (4) ◽  
pp. 377-381
Author(s):  
Dalia Dop ◽  
◽  
Desdemona Stepan ◽  
Cristian Gheonea ◽  
Elena Carmen Niculescu ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Rare Diseases ◽  
Intravenous Immunoglobulins ◽  
Stevens Johnson Syndrome ◽  
Drug Induced ◽  
Johnson Syndrome ◽  
Medical Emergencies ◽  
Steven Johnson Syndrome ◽  
Restitutio Ad Integrum

Steven-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare diseases that appear following the administration of risk drugs. Both are severity grades of the same condition and are considered medical emergencies, because they are potentially lethal. They are characterized by mucocutaneous tenderness, erythema, necrosis and bullous detachment similar to extended burns. We report 3 cases of SJS/TEN in which the etiology was probably drug-related (Paracetamol, Atomoxetinum, Sulfamethoxazolum + trimethoprinum), with restitutio ad integrum following the administration of intravenous immunoglobulins.

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