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Year : 2016  |  Volume : 6  |  Issue : 3  |  Page : 108-118

Cold medicine-related Stevens-Johnson syndrome/toxic epidermal necrolysis with severe ocular complications–phenotypes and genetic predispositions

Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, Kamigyoku, Kyoto, Japan

Correspondence Address:
Mayumi Ueta
Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, 465 Kajiicho, Hirokoji, Kawaramachi, Kamigyoku, Kyoto 602-0841
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Source of Support: None, Conflict of Interest: None

DOI: 10.1016/j.tjo.2016.06.001

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Stevens–Johnson syndrome (SJS) is an acute inflammatory vesiculobullous reaction of the skin and mucosa, such as the ocular surface, oral cavity, and genitals. In patients with extensive skin detachment and a poor prognosis, the condition is called toxic epidermal necrolysis (TEN). Severe ocular complications (SOCs) appear in some–but not all–SJS/TEN patients who are diagnosed by dermatologists, and cold medicines including multi-ingredient cold medications and nonsteroidal anti-inflammatory drugs are the main causative drugs particularly for SJS/TEN with SOCs and all SJS and TEN. In this review, we focus on the genetic predisposition of cold medicine-related SJS/TEN (CM-SJS/TEN) with SOCs. CM-SJS/ TEN with SOCs was strongly associated with HLA-A*02:06 and significantly associated with HLA-B*44:03 in Japanese individuals, significantly associated with HLA-B*44:03 in Indian and Brazilian individuals, and associated with HLA-A*02:06 in Korean individuals. In the first genome-wide association study (GWAS), we found an association between the prostaglandin E receptor 3 (PTGER3) gene and SJS/ TEN with SOCs. In this study, we focused on CM-SJS/TEN with SOCs and found that the association of CM-SJS/TEN with SOCs became stronger than all SJS/TEN with SOCs. In the second GWAS, we found an association between the IKZFi gene and CM-SJS/TEN with SOCs not only in Japanese, but also in Korean and Indian populations. Moreover, we found that TSHZ2 gene single nucleotide polymorphisms (SNPs) also showed especially low p values in the Japanese population; however, this association was not found in the Korean population. Furthermore, we investigated the interaction between susceptibility genes, and found multiplicative interactions of HLA-A*02:06 and TLR3 SNPs and additive interactions of HLA-A*02:06 and PTGER3 SNPs.

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