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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 33-35

A case report of ocular Stevens–Johnson syndrome: An important condition for the internist to see


Department of Internal Medicine, HealthOne/Rocky Vista University, Parker, CO, USA

Date of Submission05-Jun-2019
Date of Acceptance29-Sep-2019
Date of Web Publication27-Mar-2020

Correspondence Address:
Dr. Patrick Jenkins
10099 Ridgegate Parkway, Suite 200 Lone Tree, CO, 80124
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJAM.IJAM_33_19

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  Abstract 

A patient was admitted with Stevens–Johnson syndrome (SJS) and found to have ocular manifestations including eyelid edema, conjunctival hyperemia, and worsening pseudomembranes which threatened his vision. He was referred to a corneal specialist and successfully treated with topical therapy and amniotic membrane grafts. This case is important because while ocular manifestations of SJS are common, there is a lack of awareness of its prevalence and available treatment modalities. Rapid diagnosis and initiation of treatment is crucial to avoid devastating and permanent ocular complications.
The following core competencies are addressed in this article: Patient care, Medical knowledge, and System-based practice.

Keywords: Amniotic membrane graft, corneal epithelial defect, ocular Stevens–Johnson syndrome


How to cite this article:
Jenkins P, Enurah A, Scherbak D. A case report of ocular Stevens–Johnson syndrome: An important condition for the internist to see. Int J Acad Med 2020;6:33-5

How to cite this URL:
Jenkins P, Enurah A, Scherbak D. A case report of ocular Stevens–Johnson syndrome: An important condition for the internist to see. Int J Acad Med [serial online] 2020 [cited 2020 May 25];6:33-5. Available from: http://www.ijam-web.org/text.asp?2020/6/1/33/281456


  Introduction Top


We present a case of Stevens–Johnson syndrome (SJS) with ocular involvement which progressed to the point of vision loss which was successfully treated with amniotic membrane graft. SJS and toxic epidermal necrolysis (TEN) are conditions within a spectrum characterized by severe mucocutaneous reactions. The most common causes include medications, although SJS has been associated with infection.[1],[2] TEN is defined as involvement of greater than 30% body surface area; SJS–TEN overlap is 10%–30% and SJS is less than 10%. One study showed ocular manifestations occurred in up to 81% in SJS and 66.7% in TEN.[3] Chronic ocular sequelae from SJS/TEN include persistent ulceration of the ocular surface, chronic dry eyes, and scarring.[2] Involvement of ophthalmology and initiation of topical medications is indicated at any level of disease.[4] Surgical options, including amniotic membrane graft, are available and have been shown to improve the visual outcomes, especially when used early.[5],[6],[7]

Despite the prevalence of ocular involvement in cases of SJS and the potentially devastating ocular complications, there is a lack of awareness among many providers about this aspect of the disease and treatments available for it. This likely occurs for two reasons: first, while ocular involvement is common when SJS occurs, SJS remains a rare disease with a small number of patients treated at a center in a given year. This is particularly true outside of large referral centers.[8] Second, there may be a tendency to focus on life-threatening cutaneous manifestations rather than signs and symptoms of ocular involvement;[9] this is problematic given that initially, mild ocular involvement does not ensure a mild course.[8]


  Case Report Top


A 54-year-old African American male was admitted to our hospital. He had developed perioral swelling and vesicular lesions over the week proceeding hospitalization and was diagnosed with mucocutaneous SJS on admission [Figure 1]. Thirty days before presentation, he suffered a generalized tonic–clonic seizure for the first time and was started on phenytoin. Phenytoin was thought to be the cause of his SJS. On admission, he was admitted to the intensive care unit (ICU) and evaluated by ophthalmology. He was found to have eyelid edema, conjunctival hyperemia, and mild pseudomembranes indicating conjunctival involvement of his SJS. His vision was 20/25 in the right eye and 20/30 in his left, and he was treated with erythromycin ointment, prednisolone eye drops, and artificial tears. The SJS involvement of his oral mucosa did not progress. He did not develop lesions anywhere else on his body. However, 10 days after admission, he reported total vision loss.
Figure 1: Stevens–Johnson syndrome involving the oral mucosa

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The patient was evaluated under slit lamp with fluorescein staining and was found to have worsening of pseudomembranes with bilateral epithelial defects [Figure 2]. Pseudomembranes of the palpebral conjunctiva were also appreciated with eversion of the upper eyelids and fluorescein staining. His vision at this time was 20/400 in both eyes. He was transferred to another hospital to see a corneal specialist and treated with topical cyclosporine, dexamethasone, ciprofloxacin, and tobramycin. Two days after transfer (12 days after the initial presentation) under the care of the corneal specialist, the patient received bilateral human amniotic membrane grafts to the upper and lower eyelid margins under general anesthesia. Amniotic membranes clipped between symblepharon rings were placed in both eyes at that time and removed along with his grafts 19 days after the initial presentation. After the removal of grafts, he had significant improvement in epithelial defects, and his vision was now 20/30 on the right and 20/40 on the left eye. He was discharged home with scheduled outpatient ophthalmology and primary care follow-up.
Figure 2: Ocular Stevens–Johnson syndrome involving the lid margin and conjunctiva with pseudomembranes and epithelial defect

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  Discussion Top


SJS is an acute and potentially life-threatening cutaneous drug reaction that affects the skin and mucous membranes. It is commonly associated with anti-infective sulfonamides, allopurinol, carbamazepine, phenobarbital, phenytoin, and oxicam - nonsteroidal anti-inflammatory drugs and less commonly with viral and mycobacterial infection.[1],[2] Ocular involvement in the acute stage is common with 15%–75% of patients developing bilateral conjunctivitis and 25% of hospitalized patients developing conjunctival or corneal ulcerations.[2] Up to 35% of patients have chronic ocular sequelae from the disease process including persistent ulceration of the ocular surface, chronic dry eyes, and scarring.[2] Medical management of acute ocular Stevens–Johnson includes topical antibiotics, steroids, calcineurin inhibitors, and lubricant.[4] Surgical management includes amniotic membrane transplantation. There are three methods of amniotic membrane transplant: cryopreserved amniotic membrane, a biologic device made by clipping a piece of amniotic membrane between two symblepharon rings, and human amniotic membrane graft.[2] A few small studies have been done to evaluate amniotic membrane transplant in ocular SJS and seem to demonstrate a significant benefit.[5],[6] This benefit appears to be greatest when done early in the disease with increased risk of long-term ocular sequelae after 6 days.[7] In one study, rather than suturing grafts in the operating room, a sterile bedside procedure was performed using fibrin glue.[6] While the majority of these patients had mild ocular disease and none were severe, this could provide a way to intervene early in the disease process for patients with SJS who are not able to be transported out of the ICU.


  Conclusions Top


Due to the high prevalence of ocular involvement in SJS, the availability of treatment, and the devastating complications of delayed treatment, it is important for clinicians to be aware of this condition. A treatment regimen including topical dexamethasone, moxifloxacin, cyclosporine, tobramycin, and preservative-free tears can be initiated as soon as ocular surface involvement is suspected.[4] Surgical options involving the use of amniotic membrane grafts are available and are effective in preventing long-term sequelae of the disease when used early.

Declaration of patient consent

The authors certify that they have obtained required appropriate patient consent form. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Disclaimer: This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.

Acknowledgments

We would like to thank Echalier, Lacey MD, University of Colorado, Department of Oculofacial Plastic and Orbital Surgery, for reviewing the work.

Research quality and ethics statement

The authors of this manuscript declare that this scientific work complies with reporting quality, formatting, and reproducibility guidelines set forth by the EQUATOR Network. The authors also attest that this clinical investigation was determined to not require institutional review board/ethics committee review, and the corresponding protocol/approval number is not applicable.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mockenhaupt M, Viboud C, Dunant A, Naldi L, Halevy S, Bouwes Bavinck JN, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol 2008;128:35-44.  Back to cited text no. 1
    
2.
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;61:369-99.  Back to cited text no. 2
    
3.
Chang YS, Huang FC, Tseng SH, Hsu CK, Ho CL, Sheu HM, et al. Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: Acute ocular manifestations, causes, and management. Cornea 2007;26:123-9.  Back to cited text no. 3
    
4.
Gregory DG. New grading system and treatment guidelines for the acute ocular manifestations of Stevens-Johnson syndrome. Ophthalmology 2016;123:1653-8.  Back to cited text no. 4
    
5.
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.  Back to cited text no. 5
    
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.  Back to cited text no. 6
    
7.
Cherof AM. Acute Stevens-Johnson Syndrome. The Effect the Timing of Amniotic Membrane Trans Plantation has on the Occurrence of Significant Ocular Sequelae. Las Vegas: AAO Annual Meeting; 2015.  Back to cited text no. 7
    
8.
Gueudry J, Roujeau JC, Binaghi M, Soubrane G, Muraine M. Risk factors for the development of ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2009;145:157-62.  Back to cited text no. 8
    
9.
Miliszewski MA, Kirchhof MG, Sikora S, Papp A, Dutz JP. Stevens–Johnson syndrome and toxic epidermal necrolysis: An analysis of triggers and implications for improving prevention. Am J Med 2016;129:1221-5.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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