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Cytokine storm arising on the ocular surface in a patient with Stevens–Johnson syndrome
  1. Tomohito Yagi1,
  2. Chie Sotozono2,
  3. Masami Tanaka1,
  4. Masahiro Fuwa1,
  5. Eiichi Sekiyama2,
  6. Mayumi Ueta2,
  7. Kei Tashiro1,
  8. Shigeru Kinoshita2
  1. 1Department of Genomic Medical Sciences, Kyoto Prefectural University of Medicine, Kyoto, Japan
  2. 2Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan
  1. Correspondence to Dr Chie Sotozono, Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kajii-cho465, Hirokoji-agaru, Kawaramachi-dori, Kamigyo-ku, Kyoto 602-0841, Japan; csotozon{at}

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Stevens–Johnson syndrome (SJS) and its more extreme variant, toxic epidermal necrolysis (TEN), are acute, adverse systemic reactions that can affect anyone who takes medications. SJS/TEN predominantly affects the skin and mucosal membranes and predisposes patients to life-threatening complications such as sepsis, respiratory dysfunction and multi-organ failure. Even when a patient does survive this disease, serious ocular discomfort and morbidity often persists life long.1 2

In May 2008, a 59-year-old female inpatient had a case of red eyes, and 2 days later she presented with a sudden onset of high fever and eruption and erosion in the mucocutaneous regions including the mouth, paronychia and bilateral conjunctivitis. Slit-lamp examination revealed a large epithelial defect of the conjunctiva with severe hyperaemia in both eyes (figure 1A,B). There was no viral or bacterial infection, and skin biopsy specimens of the erythematous macules revealed necrotic keratinocytes and liquefaction, compatible with the diagnosis of SJS. Steroid pulse therapy and intensive topical betamethasone (0.1%, 10 times daily) were …

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  • Funding Health and Labor Sciences Research Grants (Research on Intractable Diseases) from the Ministry of Health, Labour and Welfare of Japan, and the Japanese Ministry of Education, Culture, Sports, Science and Technology.

  • Competing interests None.

  • Patient consent This study was performed in accordance with the tenets set forth in the Declaration of Helsinki and written informed consent was obtained from the patient prior to involvement in the study.

  • Ethics approval Ethics approval was provided by the Institute Review Board (IRB) of Kyoto Prefectural University of Medicine, Kyoto, Japan.

  • Provenance and peer review Not commissioned; externally peer reviewed.