Lee Hyo Seok, Ueta Mayumi, Kim Mee Kum, Seo Kyoung Yul, Sotozono Chie, Kinoshita Shigeru, Yoon Kyung Chul
*Department of Ophthalmology, Chonnam National University Medical School and Hospital, Gwangju, South Korea; Departments of †Frontier Medical Science and Technology for Ophthalmology, and ‡Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan; §Department of Ophthalmology, Seoul National University College of Medicine, Seoul, South Korea; and ¶Department of Ophthalmology, Institute of Vision Research, Yonsei University College of Medicine, Seoul, South Korea.
Cornea. 2016 Feb;35(2):199-204. doi: 10.1097/ICO.0000000000000708.
To describe the clinical characteristics and genetic background of allopurinol-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in South Korea.
This is a prospective, noncomparative case series. Visual acuity, detailed medical history, ocular findings, and systemic manifestations of 5 patients (10 eyes) with allopurinol-induced SJS/TEN were recorded. The acute ocular involvement score and the chronic ocular manifestation score were graded on scales of 0-3 and 0-39, respectively, based on severity. Human leukocyte antigen (HLA) genotyping was also performed during the hospitalization.
Three patients were diagnosed with SJS, and 2 with TEN. Mild ocular involvement with only conjunctival hyperemia (acute ocular involvement score ≤ 1) was present in all 10 eyes during the acute stage. Patients were treated with systemic steroids and topical antibiotics, steroids, and preservative-free artificial tears, with rinsing of the ocular surface, in the acute stages of SJS/TEN. In the final follow-up, none of the patients had developed severe chronic ocular complications (chronic ocular manifestation score ≤ 8), including keratinization, corneal conjunctivalization, mucocutaneous junction involvement, or symblepharon. One patient developed bilateral persistent epithelial defects 3 months after the disease onset, which healed after conservative treatment, leaving a bilateral central corneal haze. HLA genotyping showed that 4 of the 5 patients (80%) were positive for HLA-B*58:01.
Allopurinol-induced SJS/TEN might not cause serious acute or chronic complications of the ocular surface. In addition, our HLA genotyping results are consistent with previous studies reporting a strong association between HLA-B*58:01 and allopurinol-induced SJS/TEN among Koreans.
描述韩国别嘌醇诱发的史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）的临床特征及遗传背景。
这是一个前瞻性、非对照病例系列研究。记录了5例（10只眼）别嘌醇诱发的SJS/TEN患者的视力、详细病史、眼部检查结果及全身表现。根据严重程度，急性眼部受累评分和慢性眼部表现评分分别采用0 - 3分和0 - 39分的量表进行分级。住院期间还进行了人类白细胞抗原（HLA）基因分型。
3例诊断为SJS，2例诊断为TEN。急性期所有10只眼均有轻度眼部受累，仅表现为结膜充血（急性眼部受累评分≤1）。在SJS/TEN急性期，患者接受全身类固醇及局部抗生素、类固醇和无防腐剂人工泪液治疗，并进行眼表冲洗。在最终随访中，所有患者均未出现严重的慢性眼部并发症（慢性眼部表现评分≤8），包括角化、角膜结膜化、黏膜皮肤交界处受累或睑球粘连。1例患者在发病3个月后出现双侧持续性上皮缺损，经保守治疗后愈合，遗留双侧中央角膜混浊。HLA基因分型显示，5例患者中有4例（80%）HLA - B*58:01阳性。
别嘌醇诱发的SJS/TEN可能不会导致严重的眼表急慢性并发症。此外，我们的HLA基因分型结果与之前报道韩国人HLA - B*58:01与别嘌醇诱发的SJS/TEN之间存在强关联的研究一致。
