Long-term Progression of Ocular Surface Disease in Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is an acute inflammatory vesiculobullous reaction of the mucosa of the ocular surface, oral cavity, and genitals, and of the skin. Severe ocular complications (SOC) are observed in about half of SJS/TEN patients diagnosed by dermatologists and in burn units. Ophthalmologists treat SOC, and they tend to encounter the patients not only in the acute stage, but also in the chronic stage. Our investigation of the pathogenesis of SJS/TEN with SOC led us to suspect that abnormal innate mucosal immunity contributes to the ocular surface inflammation seen in SJS/TEN with SOC. We confirmed that cold medicines such as NSAIDs and multi-ingredient cold medications are the main causative drugs for SJS/TEN with SOC. Single nucleotide polymorphism (SNP) association analysis of cold medicine-related SJS/TEN with SOC showed that the Toll-like receptor 3 (TLR3)-, the prostaglandin-E receptor 3 (PTGER3)-, and the IKZF1 gene were significantly associated with SNPs and that these genes could regulate mucocutaneous inflammation including that of the ocular surface. We also examined the tear cytokines of SJS/TEN with SOC in the chronic stage and found that IL-8, IL-6, IFN-γ, RANTES, eotaxin, and MIP-1β were significantly upregulated in SJS/TEN with SOC in the chronic stage. Only IP-10 was significantly downregulated in SJS/TEN with SOC in the chronic stage. This mini-review summarizes the pathological mechanisms that we identified as underlying the development of SJS/TEN with SOC.

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HLA association with antipyretic analgesics-induced Stevens-Johnson Syndrome/toxic epidermal necrolysis with severe ocular surface complications in japanese patients

Clinical and Translational Allergy ◽

10.1186/2045-7022-4-s3-p89 ◽

2014 ◽

Vol 4 (S3) ◽

Author(s):

Ryosuke Nakamura ◽

Nahoko Kaniwa ◽

Mayumi Ueta ◽

Chie Sotozono ◽

Emiko Sugiyama ◽

...

Keyword(s):

Toxic Epidermal Necrolysis ◽

Ocular Surface ◽

Japanese Patients ◽

Stevens Johnson Syndrome ◽

Hla Association ◽

Johnson Syndrome

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Stevens-Johnson Syndrome and toxic epidermal necrolysis in children: a retrospective study at Srinagarind Hospital, Khon Kaen, Thailand 1992–2012

Asian Biomedicine ◽

10.5372/1905-7415.0902.386 ◽

2017 ◽

Vol 9 (2) ◽

pp. 193-196

Author(s):

Watuhatai Paipool ◽

Leelawadee Sriboonnark

Keyword(s):

Toxic Epidermal Necrolysis ◽

Drug Exposure ◽

Length Of Hospital Stay ◽

Retrospective Chart Review ◽

Stevens Johnson Syndrome ◽

Ocular Complications ◽

Common Mean ◽

Johnson Syndrome ◽

Acute Complications

Abstract Background Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening skin conditions with an etiology of drug exposure or infections. Objectives To determine the cause, treatments, complications, and outcomes of SJS/TEN in children admitted to Srinagrind Hospital during 1992–2012. Methods Retrospective chart review. A diagnosis of SJS and TEN was confirmed by pediatric dermatologists. Results A total of 38 patients was recorded. They consisted 31 (82%) SJS patients and 7 (18%) TEN patients. Mean age 6.6 years (range 1 to 14 years). Male to female was 1.1:1. Most cases (30 or 79%) were caused by drug exposure. Three cases (8%) by infection, and 5 cases (13%) were of unknown cause. The antiepileptic drug group was the most common cause. Systemic corticosteroids were used in 33 cases (87%). Intravenous immunoglobulin was used in one TEN patient (3%). There were 18 cases (47%) with acute complications. Ocular complications (7 cases, 39%), septicemia (4 cases, 22%), and secondary skin infections (3 cases, 17%) were the most common. Mean difference in length of hospital stay between those with and without acute complications was 12.3 days (P < 0.01, 95% CI 5.9–18.6). Ocular complications were the only long-term complications at 1-year follow up, and included symblepharon, corneal pannus, and dry eyes. Two patients (5%), both having cases of TEN, died. Conclusions Antiepileptic drugs were the most common causes of SJS/TEN in our study. Good ophthalmologic care of the prevalent acute eye complications in these patients is needed to prevent long-term ophthalmic complications.

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