Ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis among patients admitted to a medical college hospital: A case series

Shadin Khadeeja; Rashmi Shambhu; Anupama Bappal; Shama Sharief

doi:10.4103/ijo.IJO_1294_20

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 1 | Page : 103-105

Ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis among patients admitted to a medical college hospital: A case series

Shadin Khadeeja, Rashmi Shambhu, Anupama Bappal, Shama Sharief
Department of Ophthalmology, Yenepoya Medical College, Yenepoya, Mangalore, Karnataka, India

Date of Submission	03-May-2020
Date of Acceptance	29-Jul-2020
Date of Web Publication	31-Dec-2020

Correspondence Address:
Dr. Rashmi Shambhu
Flat No. 302, Gokul Residency, Kadri Kambala, Near Gopal Krishna Mutt, Mangalore - 575 004, Karnataka
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1294_20

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare blistering diseases of the skin and mucous membranes with ocular involvement. This is a retrospective analysis of patients with SJS and TEN admitted in a medical college hospital from June 2016 to May 2019. Six among 21 patients had ocular involvement. Five patients with mild grade of ocular involvement were managed with conservative treatment. One patient with TEN and bilateral severe grade involvement underwent bedside application of amniotic membrane. Performing this technique can become challenging. Hence, we look forward towards the inventions of easier variations of the technique.

Keywords: Amniotic membrane dressing, ocular manifestation, Stevens-Johnson syndrome, toxic epidermal necrolysis

How to cite this article:
Khadeeja S, Shambhu R, Bappal A, Sharief S. Ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis among patients admitted to a medical college hospital: A case series. Indian J Ophthalmol Case Rep 2021;1:103-5

How to cite this URL:
Khadeeja S, Shambhu R, Bappal A, Sharief S. Ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis among patients admitted to a medical college hospital: A case series. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Feb 26];1:103-5. Available from: https://www.ijoreports.in/text.asp?2021/1/1/103/305481

Stevens-Johnson syndrome ^{More Details} (SJS) and toxic epidermal necrolysis (TEN) are rare acute immune-mediated blistering diseases of the skin and mucous membranes mostly triggered by certain drugs and less commonly by systemic infections. The disease can be fatal in acute phase, with mortality rates that range from 1 to 5% in SJS and 25 to 35% in TEN.^[1]

Ocular involvement in SJS/TEN during acute phase is very common and varies from mild to severe grade. There can be potentially vision threatening complications and chronic disabling sequel in some patients if the acute phase is not managed appropriately.^[2] Because of the rarity of SJS and TEN, most ophthalmologists do not encounter acute-phase patients on a regular basis.^[3] Hence, it becomes imperative on part of the treating team and ophthalmologists in particular not to miss this window of opportunity and be aware of the current methods of management.

Case Reports

The case records of the patients admitted with SJS and TEN in a medical college hospital from June 2016 to May 2019 were retrospectively analyzed. Among the 21 patients, 6 patients had ocular involvement that included one child. The profile of patients with ocular involvement and the findings on ocular examination are detailed in [Table 1] and [Table 2],

Table 1: Demographic and clinical profile of the patients with ocular involvement in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)

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Table 2: Findings on ocular examination of the patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)

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To grade ocular involvement, grading system as suggested by Gregory was followed.^[3] Of the six patients, five were considered to have mild grade of ocular involvement. They were managed with conservative treatment. Patient number 6 had bilateral severe grade of ocular grade involvement [Figure 1]a. In addition to conservative treatment, amniotic membrane transplantation (AMT) was planned. Her systemic condition was very poor due to secondary sepsis and multiorgan involvement. As it was not possible to shift her to operation theatre, a bedside procedure was planned in the intensive care unit.

Figure 1: (a) At presentation, peeling of lid skin and eyes are closed with crusting. On evaluation membranous conjunctivitis and corneal epithelial defects were noted (patient number 6). (b) At the time of discharge, showing Meibomian gland ^{More Details} dysfunction and severe dry eye. (c) At 8 months follow up, showing mild dry eye; rest normal

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Dry amniotic membrane (Amnio-care, Biocover Laboratories, India) was just placed on the ocular surface spread evenly as much as possible and tucked in the fornices and patched for 24 h (no use of any sutures, glue, symblepharon rings as in conventional method). There was notable improvement in the ocular surface after the amniotic membrane dressing. There was no need to take her up for the repeat amniotic membrane transplant procedure as her ocular surface inflammation had drastically reduced. The retention of amniotic membrane lasted for 48 h. Topical medications were continued. The patient did not have any active disease at the time of discharge. She had normal vision with clear cornea in both eyes; however, there was meibomian gland dysfunction and severe dry eye [Figure 1]b. She was regularly followed up at monthly intervals. Her condition improved over months and at 8 months follow up; she had mild dry eye only [Figure 1]c. Best corrected vision of 20/20 was present.

Discussion

Ocular involvement is common (50–88% of patients) in the acute phase of SJS/TEN, with signs ranging from conjunctival hyperemia to complete sloughing of the ocular surface and eyelid skin. Moderate-to-severe ocular involvement may be seen in up to 41% of patients.^[2] In severe cases, pseudomembranous or membranous conjunctivitis, symblephron, ulcerations of lid margin, conjuctival/corneal ulcerations, and even perforations can occur.

During the acute phase of the disease, the ocular changes may go unnoticed as patient may be under intensive care, and eyes are usually closed with crust. It is important that the case is referred to an ophthalmologist at this stage as early interventions improve long-term outcomes. Once the disease progresses, the treatment options become more limited, difficult, invasive, and high risk.^[2],[4],[5]

During acute stage, medical treatment and close observation have been advised for mild and moderate cases. Urgent AMT is required for severe and very severe cases.^[1],[3] Conventional medical management comprises of administration of topical steroids to control inflammation, antibiotics to prevent infection, lubricants, cyclosporine, and periodic lysis of symblepharon with a glass rod.^[3],[6] Many studies have proved that the early use of AMT in addition to conventional therapy is beneficial during the acute phase to prevent vision threatening scarring sequel.^{[1],[3],[6],[7],[8]} Most commonly, cryopreserved amniotic membrane is used and the bedside procedure is performed.^[6],[7] The technique of AMT includes covering the entire ocular surface and securing with sutures^[7],[8] or fibrin glue.^[6],[9] Additional procedures like the use of Prokera device^[2],[5] and symblepharon ring^[6],[9] have been described.

In this patient, the conventional technique of AMT (secured with sutures/glue) could not be followed. This was due to technical difficulties in the intensive care unit (critically ill patient, with multiple tubings, poor cooperation) and also nonavailability of device like symblepharon ring at that time. Fortunately, the early use of amniotic membrane even as a bedside dressing (adjunct to medical treatment) proved to be efficient in the significant control of ocular surface inflammation.

We followed all the patients for a minimum of 3 months, which was felt inadequate to conclude on long-term sequel.

Conclusion

Performing AMT at bedside can become very challenging. Considering it to be the most crucial in cases with severe grade ocular involvement, inventions of easier variations would be appreciated.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Jain R, Sharma N, Basu S, Iyer G, Ueta M, Sotozono C, et al. Stevens-Johnson syndrome: The role of an ophthalmologist. Surv Ophthalmol 2016;61:361-99.
2.	Shanbhag SS, Rashad R, Chodosh J, Saeed HN. Long-term effect of a treatment protocol for acute ocular involvement in Stevens-Johnson syndrome/toxic epidermal necrolysis. Am J Ophthalmol 2019;208:331-41.
3.	Gregory DG. New grading system and treatment guidelines for the acute ocular manifestations of Stevens-Johnson syndrome. Ophthalmology 2016;123:1653-8.
4.	Honavar SG, Bansal AK, Sangwan VS, Rao GN. Amniotic membrane transplantation for ocular surface reconstruction in Stevens-Johnson syndrome. Ophthalmology 2000;107:975-9.
5.	Saeed HN, Chodosh J. Ocular manifestations of Stevens-Johnson syndrome and their management. Curr Opin Ophthalmol 2016;27:522-9.
6.	Sharma N, Thenarasun SA, Kaur M, Pushker N, Khanna N, Agarwal T, et al. Adjuvant role of amniotic membrane transplantation in acute ocular Stevens-Johnson syndrome: A randomized control trial. Ophthalmology 2016;123:484-91.
7.	Ciralsky JB, Sippel KC, Gregory DG. Current ophthalmologic treatment strategies for acute and chronic Stevens-Johnson syndrome and toxic epidermal necrolysis. Curr Opin Ophthalmol 2013;24:321-8.
8.	Hsu M, Jayaram A, Verner R, Lin A, Bouchard C. Indications and outcomes of amniotic membrane transplantation in the management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis: A case-control study. Cornea 2012;31:1394-402.
9.	Shanbhag SS, Chodosh J, Saeed HN. Sutureless amniotic membrane transplantation with cyanoacrylate glue for acute Stevens-Johnson syndrome/toxic epidermal necrolysis. Ocul Surf 2019;17:560-4.