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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 7  |  Issue : 2  |  Page : 254-257

Ophthalmological perspectives of toxic epidermal necrolysis


Department of Ophthalmology, Kalinga Institute of Medical Sciences and Pradyumna Bal Memorial Hospital, Bhubaneswar, Odisha, India

Date of Submission10-Jul-2019
Date of Decision28-Oct-2019
Date of Acceptance11-Nov-2019
Date of Web Publication16-Dec-2019

Correspondence Address:
Dr. Shovna Dash
Plot No. 57/353, Jaydev Vihar, Bhubaneswar - 751 013, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/amhs.amhs_98_19

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  Abstract 


Toxic epidermal necrolysis (TEN) is as rare as two cases per million per year, but the ocular manifestations and sequelae are very severe. We present a case series of seven patients admitted to the intensive care unit in a tertiary hospital with a diagnosis of TEN describing the extent of ocular involvement, its response to treatment, and appearance of sequelae. All seven were drug-induced with moderate-to-severe skin, mucosal, ophthalmic involvement, and systemic involvement. Ocular findings on presentation were eyelid skin detachment, adherent lid margins, bilateral mucopurulent conjunctivitis with inflammatory membranes, and punctate epithelial keratitis in all patients. Large persistent corneal epithelial defect was seen in four patients. On follow-up, five had severe keratoconjunctivitis sicca and entropion with trichiasis. Photophobia was the major debilitating sequelae. Early management was the key to reduce severity, cicatrization, and sequelae. Corticosteroids topically and systemically, topical cyclosporine, and lubricants were the mainstay of management.

Keywords: Body surface area, membranous conjunctivitis, ocular sequelae, toxic epidermal necrolysis


How to cite this article:
Mohanty G, Dash S, Kumar M, Desai D. Ophthalmological perspectives of toxic epidermal necrolysis. Arch Med Health Sci 2019;7:254-7

How to cite this URL:
Mohanty G, Dash S, Kumar M, Desai D. Ophthalmological perspectives of toxic epidermal necrolysis. Arch Med Health Sci [serial online] 2019 [cited 2020 Mar 30];7:254-7. Available from: http://www.amhsjournal.org/text.asp?2019/7/2/254/273080




  Introduction Top


Steven–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are as rare as two cases per million per year, but the ocular manifestations and sequelae are very severe and difficult to manage because of concurrent systemic morbidity. Our study describes briefly the extent of ocular involvement, its response to treatment and appearance of sequelae in seven cases of TEN.


  Case Series Top


Seven patients with a diagnosis of TEN admitted to intensive care unit in a tertiary hospital were included in our case series as summarized in [Table 1] and [Table 2]. There were three males and four females. The age ranged from 6 to 41 years.
Table 1: Demographics and ocular findings of 7 cases of toxic epidermal necrolysis

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Table 2: Body surface area involvement and visual outcome at 4 weeks

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All seven patients had an evident drug history. Three patients had a history of Mefenamic acid intake and four of phenytoin intake. All of the them had more than 30% total body surface area involvement and severe mucosal involvement and were categorized as TEN. At presentation, all of them had moderate-to-severe skin, mucosal, ophthalmic, and systemic involvement.

Ocular findings at presentation in all the patients: eyelid skin detachment, adherent lid margins, bilateral mucopurulent conjunctivitis with inflammatory membranes and corneal punctate epithelial keratitis. Large persistent corneal epithelial defect was seen in four patients associated with severe pain and photophobia. The best-corrected vision ranged from hand movement to 6/60 (Snellen's chart). Two brief case reports (Case 5 and 6) are illustrated here.

Case 5

A 17-year-old girl with adherent lid margins with Nicolsky's sign on lid skin, conjunctival membranes [Figure 1], and more than 90% skin surface area involvement [Figure 2] after intake of phenytoin for grand mal seizure. The extensive lesions involving the total cornea could not be documented because of severe photophobia.
Figure 1: Case 5– Lid skin detachment and adherent lid margins

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Figure 2: Case 5 – Before and after treatment

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Case 6

A 33-year-old man with thick fibrotic membranes with forniceal shortening despite daily manual separation of the membranes by swiping glass rod, symblepharon, xerotic cornea, and epithelial defect more than half the cornea after phenytoin injection as a postoperative medication postfrontal tumor surgery [Figure 3] and [Figure 4].
Figure 3: Case 6 – Inflammatory membranes with corneal haze

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Figure 4: Case 6 – Fibrous conjunctival membranes and forniceal shortening

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They were all treated with pulse therapy of methyl prednisolone and broad-spectrum antibiotics systemically. Topically treated with Chloramphenicol-Dexamethasone combination eye ointment, cyclosporine 0.05% eye drops 4 times a day, carboxymethylcellulose 1% eye drops 6–8 times a day, and hypromellose 0.3% Gel form 3–4 times a day along with swiping of the conjunctival membranes every alternate day. They were discharged after the resolution of the acute phase.

On follow-up, the best-corrected vision ranged from counting fingers 3 meters to 6/6, depending on the corneal involvement. Five of them had keratoconjunctiva sicca with persistent corneal epithelial erosions. Two of them had persistent corneal epithelial defects. Three patients with severe persistent corneal erosions and epithelial defects (including Case 5) required rigorous treatment with gel form of hypromellose 0.3%, topical cyclosporine 0.05% eye drops with amniotic membrane graft transplantation (AMT) to enhance healing. Trichiatic lashes seen in five of them were epilated periodically. Entropion and symblepharon were seen in five, and two of the patients, respectively, were managed conservatively. The first case had lacrimal punctual atresia, which was managed conservatively. The second case has conjunctival retention cyst which resolved with continued application of cyclosporine 0.05% eye drops. Forniceal reformation with AMT was done in fornecial shortening to inhibit further fibrotic reaction in Case 6. Photophobia due to persistent epithelial erosions was the major debilitating complaint at follow-up.

The skin lesions healed with supportive treatment after 3 weeks [Figure 2].


  Discussion Top


Epidermal necrolysis encompasses SJS (<10% of the skin affected), Lyell syndrome (TEN, with ≥30% of the skin affected) and an overlap syndrome (10%–29% of the skin affected). These rare diseases are caused, in 85% of cases, by pharmacological treatments, with symptoms occurring 4–28 days after treatment initiation. The diagnosis is clinical. Mortality is 20%–25% during the acute phase, and almost all patients display disabling sequelae (mostly ocular impairment and psychological distress).[1],[2],[3]

The triggering factors in our patients were mefenamic acid and phenytoin, which are established trigger agents. Two patients of the four TEN patients triggered by phenytoin were found to be HLA B*1502 positive suggesting genetic predisposition and altered drug metabolism. Others could not undergo HLA typing because of financial limitations.[4],[5],[6]

All our patients showed moderate-severe lid and conjunctival inflammation with membrane formation and corneal erosions. They have been classified as described by Power et al. depending on their degree of lid, conjunctival and corneal involvement and graded as proposed by Sotozono et al. according to the degree of ocular surface inflammation and epithelial necrosis or apoptosis.[7],[8] Four patients were graded as severe because of painful nonhealing persistent epithelial defects.

All responded to the topical regimen. However, the three patients with severe keratoconjunctivitis sicca and persistent corneal lesions required AMT and had a vision of counting fingers postsurgery but were relieved of pain. Forniceal reformation with AMT was done for forniceal shortening in Case 6 to relieve painful eye movements and corneal erosions. The other patients with symblepharon and entropion have been managed conservatively on follow-ups. The final visual outcome ranged from counting fingers three meters to 6/6. The skin lesions in all the patients healed irrespective of the status of ocular lesions [Table 1].

Photophobia was observed to be more severe in patients with total body surface area involvement more than 80% and severe systemic involvement, which inspired us to start our study but, because of the rare occurrence of the disease and very small subject size, we were unable to establish any causal-effect hypothesis [Table 2].


  Conclusion Top


TEN is more than just a hypersensitivity reaction and dermatological entity. The ocular involvement with cicatrizing sequelae jeopardizes the quality of life grievously. The ocular involvement can occur at any stage of the disease, so require close follow-up and prolonged maintenance treatment to reduce the degree of keratoconjunctiva sicca and photophobia. All TEN patients require a team effort for recovery and have to be counseled for frequent follow-ups to maintain a satisfactory quality of life in future.

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 Top

1.
Morales ME, Purdue GF, Verity SM, Arnoldo BD, Blomquist PH. Ophthalmic manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis and relation to SCORTEN. Am J Ophthalmol 2010;150:505-10.e1.  Back to cited text no. 1
    
2.
Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129:92-6.  Back to cited text no. 2
    
3.
Harris V, Jackson C, Cooper A. Review of toxic epidermal necrolysis. Int J Mol Sci 2016;17. pii: E2135.  Back to cited text no. 3
    
4.
Roujeau JC, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;333:1600-7.  Back to cited text no. 4
    
5.
Léauté-Labrèze C, Lamireau T, Chawki D, Maleville J, Taïeb A. Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome. Arch Dis Child 2000;83:347-52.  Back to cited text no. 5
    
6.
Kohanim S, Palioura S, Saeed HN, Akpek EK, Amescua G, Basu S, et al. Stevens-Johnson syndrome/Toxic epidermal necrolysis – A comprehensive review and guide to therapy. I. Systemic disease. Ocul Surf 2016;14:2-19.  Back to cited text no. 6
    
7.
Power WJ, Ghoraishi M, Merayo-Lloves J, Neves RA, Foster CS. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology 1995;102:1669-76.  Back to cited text no. 7
    
8.
Sotozono C, Ueta M, Nakatani E, Kitami A, Watanabe H, Sueki H, et al. Predictive factors associated with acute ocular involvement in Stevens-Johnson syndrome and toxic epidermal necrolysis. Am J Ophthalmol 2015;160:228-37.e2.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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