Elsevier

Ophthalmology

Volume 116, Issue 4, April 2009, Pages 685-690
Ophthalmology

Original article
Diagnosis and Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis with Ocular Complications

https://doi.org/10.1016/j.ophtha.2008.12.048Get rights and content

Purpose

To present a detailed clarification of the symptoms at disease onset of Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN), with ocular complications and to clarify the relationship between topical steroid use and visual prognosis.

Design

Cross-sectional study.

Participants

Ninety-four patients with SJS and TEN with ocular complications.

Methods

A structured interview, examination of the patient medical records, or both addressing clinical manifestations at disease onset were conducted for 94 patients seen at Kyoto Prefectural University of Medicine. Any topical steroid use during the first week at the acute stage also was investigated.

Main Outcome Measures

The incidence and the details of prodromal symptoms and the mucosal involvements and the relationship between topical steroid use and visual outcomes.

Results

Common cold-like symptoms (general malaise, fever, sore throat, etc.) preceded skin eruptions in 75 cases, and extremely high fever accompanied disease onset in 86 cases. Acute conjunctivitis and oral and nail involvements were reported in all patients who remembered the details. Acute conjunctivitis occurred before the skin eruptions in 42 patients and simultaneously in 21 patients, whereas only 1 patient reported posteruption conjunctivitis. Visual outcomes were significantly better in the group receiving topical steroids compared with those of the no-treatment group (P<0.00001).

Conclusions

Acute conjunctivitis occurring before or simultaneously with skin eruptions accompanied by extremely high fever and oral and nail involvement indicate the initiation of SJS or TEN. Topical steroid treatment from disease onset seems to be important for the improvement of visual prognosis.

Financial Disclosure(s)

The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Section snippets

Patients and Methods

From November 2005 through May 2008, extensive interviews were conducted with 94 patients (45 males and 49 females) with SJS or TEN with ocular complications seen at the SJS outpatient service at Kyoto Prefectural University Hospital. Of those patients, 88 cases were referral patients from the greater Japan area who had come to the SJS service at the acute stage (n = 14) or at the chronic stage (n = 74). Their ages ranged from 1 to 83 years (mean age±standard deviation, 41.6±18.5 years). At

Results

Of the 94 patients, drugs were the most commonly associated etiologic factor in 84 patients (89.4%). The causative drugs were cold remedies in 30 patients, antibiotics in 23 patients, nonsteroidal anti-inflammatory drugs in 19 patients, anticonvulsants in 5 patients, and others (anticancer agents, antirheumatic drugs, antimalarial, Chinese medicine, etc.).

Best-corrected visual acuity obtained at the chronic stage was 20/20 or better in 34 eyes (18.3%; Fig 1A), worse than 20/20 and up to and

Discussion

Stevens-Johnson syndrome and TEN are rare but potentially fatal skin disorders. Ocular involvement is common and often results in long-term complications such as serious visual impairment with ocular discomforts.13, 28 Although much has been learned over the past 50 years about the management of SJS and TEN, the following 3 important problems still remain: (1) the difficulty of obtaining a prompt and accurate diagnosis of SJS or TEN at disease onset, (2) ocular involvement often is overlooked

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  • Cited by (0)

    Manuscript no. 2008-1063.

    Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

    Supported in part by Health and Labor Sciences Research Grants (Research on Intractable Diseases) from the Ministry of Health, Labour and Welfare of Japan, Tokyo, Japan; the Japanese Ministry of Education, Culture, Sports, Science and Technology, Tokyo, Japan; the Kyoto Foundation for the Promotion of Medical Science, Kyoto, Japan; and the Intramural Research Fund of Kyoto Prefectural University of Medicine, Kyoto, Japan.

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