Stevens-Johnson Syndrome (Lyell's Syndrome)

Rare cutaneous eruption characterized by extensive KERATINOCYTE apoptosis resulting in skin detachment with mucosal involvement. It is often provoked by the use of drugs (e.g., antibiotics and anticonvulsants) or associated with PNEUMONIA, MYCOPLASMA. It is considered a continuum of Toxic Epidermal Necrolysis.

Also Known As:

Lyell's Syndrome; Drug-Induced Stevens Johnson Syndrome; Drug-Induced Stevens-Johnson Syndrome; Epidermal Necrolysis, Toxic; Mycoplasma-Induced Stevens Johnson Syndrome; Mycoplasma-Induced Stevens-Johnson Syndrome; Nonstaphylococcal Scalded Skin Syndrome; Scalded Skin Syndrome, Nonstaphylococcal; Stevens Johnson Syndrome Toxic Epidermal Necrolysis; Stevens Johnson Syndrome Toxic Epidermal Necrolysis Spectrum; Stevens-Johnson Syndrome Toxic Epidermal Necrolysis; Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Spectrum; Toxic Epidermal Necrolysis; Toxic Epidermal Necrolysis Stevens Johnson Syndrome; Toxic Epidermal Necrolysis Stevens Johnson Syndrome Spectrum; Toxic Epidermal Necrolysis Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis Stevens-Johnson Syndrome Spectrum; Drug Induced Stevens Johnson Syndrome; Drug-Induced Stevens-Johnson Syndromes; Epidermal Necrolyses, Toxic; Lyell Syndrome; Lyell's Syndromes; Mycoplasma Induced Stevens Johnson Syndrome; Necrolyses, Toxic Epidermal; Necrolysis, Toxic Epidermal; Stevens Johnson Syndrome; Stevens-Johnson Syndrome, Drug-Induced; Stevens-Johnson Syndrome, Mycoplasma-Induced; Stevens-Johnson Syndromes, Drug-Induced; Syndrome, Lyell's; Syndrome, Mycoplasma-Induced Stevens-Johnson; Syndromes, Lyell's; Toxic Epidermal Necrolyses

Networked: 3043 relevant articles (87 outcomes, 146 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Stevens-Johnson Syndrome (Lyell's Syndrome)
2.	Graft vs Host Disease (Graft-Versus-Host Disease)
3.	Dry Eye Syndromes (Dry Eye Syndrome)
4.	Multiple Myeloma
5.	Atopic Dermatitis (Atopic Eczema)

Experts

1.	Chung, Wen-Hung: 46 articles (01/2023 - 04/2004)
2.	Kinoshita, Shigeru: 44 articles (01/2022 - 10/2002)
3.	Ueta, Mayumi: 42 articles (01/2022 - 02/2007)
4.	Sotozono, Chie: 35 articles (11/2021 - 10/2002)
5.	Hung, Shuen-Iu: 33 articles (01/2021 - 04/2004)
6.	Aihara, Michiko: 23 articles (09/2021 - 10/2007)
7.	Mockenhaupt, Maja: 22 articles (01/2022 - 08/2002)
8.	Roujeau, Jean-Claude: 21 articles (01/2019 - 01/2002)
9.	Sukasem, Chonlaphat: 19 articles (01/2022 - 01/2016)
10.	Saito, Yoshiro: 19 articles (01/2021 - 11/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Stevens-Johnson Syndrome:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 08/01/2013 - "Intravenous immune globulin (IVIG) is generally thought to be of relatively low risk for adverse events and some experts consider this to be the best treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis. " 01/01/2021 - "We describe the case of an 8-week-old male with toxic epidermal necrolysis treated successfully with intravenous immunoglobulin and amniotic membrane transplant. " 10/01/2016 - "The efficacy of intravenous immunoglobulin (IVIg) for toxic epidermal necrolysis (TEN) remains controversial, particularly for high-dose IVIg. " 02/01/2000 - "[Response to the comment on the contribution "Successful therapy of Lyell syndrome with intravenous immunoglobulins"]." 05/01/2012 - "Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising."
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 03/01/1992 - "To confirm that corticosteroids are beneficial in the treatment of Stevens-Johnson syndrome (SJS), 15 patients with the syndrome were evaluated by the same group of physicians over 2 years. " 02/01/1995 - "Although the use of corticosteroids for Stevens-Johnson syndrome has recently been controversial, we thought that administration of corticosteroids was an effective treatment for some selective cases of Stevens-Johnson syndrome. " 12/01/1994 - "Corticosteroids have been shown to be helpful in the management of Stevens-Johnson syndrome. " 11/01/1986 - "Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids." 01/01/2023 - "Positive experience with TNF-α inhibitor in toxic epidermal necrolysis resistant to high-dose systemic corticosteroids."
3.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/2018 - "Cyclosporine has shown promising results for mortality in patients with Stevens-Johnson syndrome/toxic epidermal necrolysis. " 05/01/2017 - "Effectiveness, safety and tolerability of cyclosporine versus supportive treatment in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A record-based study." 12/01/2010 - "A French phase II study gives a glimpse of a trend toward efficacy in terms of survival in the treatment of toxic epidermal necrolysis with cyclosporine. " 06/01/2022 - "Combined use of cyclosporine in the treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis." 08/01/2021 - "Use of cyclosporine for the treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis."
4.	SteroidsIBA 10/01/2013 - "Question 2: Would systemic steroids be useful in the management of Stevens-Johnson syndrome?" 09/09/2009 - "We describe two cases of drug-induced toxic epidermal necrolysis, a male and a female, both caucasoids of Pakistani origin, one treated without any steroids and the other with them, who made complete recovery without any major complications or sequelae. " 01/01/2005 - "Controversy in the use of high-dose systemic steroids in the acute care of patients with Stevens-Johnson syndrome." 11/01/1997 - "Toxic epidermal necrolysis: an analysis of referral patterns and steroid usage." 06/01/2009 - "Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset."
5.	Carbamazepine (Tegretol)FDA LinkGeneric 01/01/2020 - "Thus, with the help of paramedics staff in a community health center and mobile teledermatology, the patient was diagnosed as carbamazepine-induced toxic epidermal necrolysis and treated successfully with good outcome." 09/01/2021 - "The patient responded well to carbamazepine but had to withdraw the treatment due to carbamazepine-induced Stevens-Johnson syndrome after 3 weeks of medication. " 09/01/2009 - "In contrast to the study of carbamazepine-induced Stevens-Johnson syndrome in Han Chinese patients, none of the cases carried B*1502. " 09/01/2022 - "Moreover, carbamazepine is one of the most common causes of Stevens-Johnson syndrome (SJS), a severe skin condition with a high mortality rate. " 02/01/2022 - "Eventually, the formal diagnosis of carbamazepine-induced Stevens-Johnson syndrome (SJS) was made based on history, clinical presentation, and skin biopsy. "
6.	Infliximab (Remicade)FDA Link 04/01/2023 - "Complete Remission in a Child With Multisystem Inflammatory Syndrome and Stevens-Johnson Syndrome Treated With Infliximab." 05/01/2019 - "Pediatric toxic epidermal necrolysis treated successfully with infliximab." 01/01/2009 - "To report the case of a patient with Stevens-Johnson Syndrome (SJS) with Boston Keratoprosthesis (KPro) who may have benefited from infliximab infusions. " 06/01/2017 - "Infliximab after Boston Keratoprosthesis in Stevens-Johnson Syndrome: An Update." 08/01/2015 - "The authors reported their experience on three patients affected by Lyell syndrome treated with infliximab. "
7.	Immunoglobulins (Immunoglobulin)IBA 07/01/2020 - "Case of toxic epidermal necrolysis successfully treated with repeated i.v. immunoglobulin." 04/01/2015 - "Non-drug-induced Stevens-Johnson syndrome successfully treated with high-dose i.v. immunoglobulin." 01/01/2009 - "Toxic Epidermal Necrolysis Managed with Immunoglobulin: Reply." 07/01/2008 - "Toxic Epidermal Necrolysis Managed with Immunoglobulin." 03/31/2006 - "In view of the pathogenic mechanisms of Lyell's syndrome, we consider support-only treatment to be insufficient and believe it is necessary to administer i.v. human immunoglobulin. "
8.	Ophthalmic Solutions (Eye Drops)IBA 05/01/2013 - "Toxic epidermal necrolysis caused by over the counter eyedrops." 09/01/2011 - "To compare the concentrations of epitheliotrophic factors in autologous serum eye drops (ASE) prepared from sera of chronic Stevens-Johnson syndrome (SJS) patients with dry eyes to those prepared from non-autoimmune dry eye controls and to study the stability of the epitheliotrophic factors in different storage conditions. " 01/01/2022 - "To evaluate the efficacy and safety of umbilical cord serum eye drops for dry eyes in ocular Stevens-Johnson Syndrome (SJS). " 01/01/2022 - "Treatment of Severe Dry Eye in Stevens-Johnson Syndrome with Umbilical Cord Serum Eye Drops." 09/01/2011 - "Stability of epitheliotrophic factors in autologous serum eye drops from chronic Stevens-Johnson syndrome dry eye compared to non-autoimmune dry eye."
9.	Acetylcysteine (Siran)FDA LinkGeneric 10/01/2014 - "Toxic epidermal necrolysis treated with N-acetylcysteine." 03/01/2002 - "Toxic epidermal necrolysis treated with N-acetylcysteine." 01/01/2020 - "Three cases of toxic epidermal necrolysis or Stevens-Johnson Syndrome-associated mucocutaneous blisters are presented in this study where intravenous N-acetylcysteine (600 mg, every 8 h) was given in early hospitalization hours for the treatment of mucocutaneous fluid-filled blisters. " 05/01/2019 - "Results: Efficacy of N-acetylcysteine was shown in excoriation disorder, onychophagia disorder, trichotillomania, acne vulgaris, Type I lamellar ichthyosis, bullous morphea, systemic sclerosis, toxic epidermal necrolysis, atopic dermatitis, xeroderma pigmentosum, and pseudoporphyria. " 12/01/2014 - "Effect of N-acetylcysteine combined with infliximab on toxic epidermal necrolysis. "
10.	Etanercept (Enbrel)FDA Link 01/01/2021 - "Treatment of Toxic Epidermal Necrolysis with Etanercept in a Pediatric Patient." 01/01/2021 - "A Cautionary Tale of Criteria Based on Anecdotal Evidence With Etanercept in Toxic Epidermal Necrolysis." 01/01/2021 - "A Cautionary Tale of Etanercept Use in Patients With Toxic Epidermal Necrolysis." 10/01/2020 - "A new case series on etanercept treatment for toxic epidermal necrolysis." 01/01/2020 - "Use of Etanercept in a Series of Pediatric Patients With Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis Spectrum Disease."

Therapies and Procedures

1.	Therapeutics 05/01/2011 - "We were able to continue high-dose PB therapy in six patients because their adverse effects were transient and improved after a decrease in PB concentration, but we discontinued this therapy in the patient who developed Stevens-Johnson syndrome. " 11/01/2016 - "The ideal therapy of Stevens-Johnson syndrome/toxic epidermal necrolysis still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments. " 01/01/2023 - "Stevens-Johnson syndrome (SJS) is a rare but potentially fatal complication of TKI and anti-PD-1 therapy. " 12/01/2022 - "What are the effects of systemic therapies for Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)? " 04/16/2022 - "However, we report a case of toxic epidermal necrolysis (TEN), a grade 4 toxicity, after this combination therapy. "
2.	Plasmapheresis 10/01/2008 - "This study describes the efficacy of plasmapheresis for the treatment of toxic epidermal necrolysis (TEN), as reported in Japan. " 05/01/1998 - "A study of the efficacy of plasmapheresis for the treatment of drug induced toxic epidermal necrolysis." 01/01/2012 - "Although plasmapheresis is expensive and requires easy venous access, it could be included in the first-line treatment of toxic epidermal necrolysis. " 05/01/1998 - "The efficacy of plasmapheresis for the treatment of toxic epidermal necrolysis (TEN) in our patient and related reports in the literature were examined. " 10/01/1996 - "On the basis of the clinical results, the pathogenetic considerations, and in view of the lack of specific treatment modalities, clinical studies concerning plasmapheresis for the treatment of toxic epidermal necrolysis are recommended, particularly since this procedure is widely available and generally well tolerated."
3.	Transplantation 01/01/2006 - "To report successful management of acute stage toxic epidermal necrolysis (TEN) by amniotic membrane transplantation. " 05/01/2022 - "The purpose of this study was to describe the chronic ocular sequelae and subsequent surgical interventions after amniotic membrane transplantation (AMT) in a patient with Stevens-Johnson syndrome (SJS). " 01/01/2021 - "To study the outcomes of cultivated oral mucosal epithelial transplantation (COMET) in eyes with chronic Stevens-Johnson syndrome (SJS) sequelae. " 11/01/2016 - "Re: Sharma et al.: Adjuvant role of amniotic membrane transplantation in acute ocular Stevens-Johnson syndrome: a randomized control trial (Ophthalmology 2016;123:484-91)." 03/01/2016 - "Adjuvant Role of Amniotic Membrane Transplantation in Acute Ocular Stevens-Johnson Syndrome: A Randomized Control Trial."
4.	Plasma Exchange 12/01/1985 - "Since there is no effective treatment for toxic epidermal necrolysis, a controlled clinical trial to evaluate the effectiveness of plasma exchange would seem worthwhile." 12/01/1985 - "Five patients with severe drug-induced toxic epidermal necrolysis improved rapidly after one to two plasma exchanges. " 03/01/2021 - "Toxic epidermal necrolysis after therapeutic plasma exchange in pediatric lupus patients and associated risk factors analysis." 01/01/2012 - "Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: a series of four cases." 06/01/2002 - "Plasma exchange in patients with toxic epidermal necrolysis."
5.	Contact Lenses 09/01/2010 - "To evaluate the efficacy of scleral contact lenses use on the management of ocular sequelae from Stevens-Johnson syndrome patients. " 03/01/2021 - "Management of Stevens-Johnson syndrome using a mini-scleral contact lens." 12/01/2020 - "Clinical trial to evaluate the therapeutic benefits of limbal-supported contact lens wear for ocular sequelae due to Stevens-Johnson syndrome/toxic epidermal necrolysis." 09/01/2010 - "Scleral contact lenses represent an important and accessible alternative to reduce the limitations inferred by the damages from Stevens-Johnson syndrome." 09/01/2010 - "In a retrospective study, patients who suffered sequelae of Stevens-Johnson syndrome and started the use of scleral contact lenses were followed. "