Sjs stevens johnson syndorme Eye Ophthalmology

By: Dr. Sayed Aminullah Ehsas

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2. Thursday, August 2, 2018 2

3. Ministry of Public Health of
Afghanistan

NOOR Eye Care Training Center
(NETC(
Thursday, August 2, 2018 3
Instructor: Dr. Abdul Wodood (Wahab)
Presenter: Dr. Sayed Aminullah
Issue: (SJS/TEN or Lyell Syndrome)

4. Outlines
SJS/TEN or Lyell Syndrome
1.Introduction
2.Ocular Features
3.Systemic Features
4.Systemic Treatment
5.Ocular Treatment
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5. Introduction
•The terms ‘Stevens–Johnson syndrome (SJS)’
and ‘erythema multiforme major’ have
historically been used synonymously.
•However, it is now believed that erythema
multiforme (without the ‘major’) is a distinct
disease, milder and recurrent, with somewhat
dissimilar clinical features.
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6. Introduction…
•Toxic epidermal necrolysis (TEN – Lyell
syndrome) is a severe variant of SJS.
•SJS/TEN patients tend to be young adults,
though other groups may be affected.
•The condition involves a cell-mediated
delayed hypersensitivity reaction, usually
related to drug exposure.
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7. Introduction…
•A wide range of medications have been
incriminated, including antibiotics (especially
sulfonamides and trimethoprim), analgesics
including paracetamol (acetaminophen), cold
remedies and anticonvulsants.
•Infections due to microorganisms such as
Mycoplasma pneumoniae and herpes simplex
virus, and some cancers have also been
implicated.
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8. Introduction…
•Because symptoms often take weeks to
develop, in many cases the precipitant cannot
be identified.
•Mortality overall is around 5% in SJS (death is
commonly due to infection), but is
considerably higher in TEN.
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9. Ocular features
•In the acute stage there are often practical
obstacles to standard slit lamp examination;
• the patient may be bedridden and
undergoing barrier nursing; a portable slit
lamp may be helpful.
Symptoms:
•Acute ocular symptoms may include redness,
mild–severe grittiness, photophobia, watering
and blurring.
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10. Ocular features…
Acute signs:
•Haemorrhagic crusting of the lid margins is
characteristic;
•skin lesions may be confluent and it is often
difficult for an examiner to open the eyes
without causing marked discomfort.
•Papillary conjunctivitis, which can range from
mild, transient and self-limiting to severe.
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Hemorrhagic lid Crusting

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Sever Acute Conjunctivitis

13. Ocular features…
•Conjunctival membranes and
pseudomembranes , severe hyperaemia,
haemorrhages, blisters and patchy infarction.
•Keratopathy: a spectrum of lesions from
punctate erosions to large epithelial defects,
secondary bacterial keratitis and occasionally
perforation.
•Iritis is not infrequent, and panophthalmitis
has been reported.
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Pseudomembrane

15. Ocular features…
Late signs:
•Conjunctival cicatrization with forniceal
shortening and symblepharon formation.
•Keratinization of the conjunctiva and lid margin,
sometimes with abrasive plaque formation.
•Eyelid complications include cicatricial entropion
and ectropion, trichiasis, metaplastic lashes and
ankyloblepharon.
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conjunctival scarring

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keratinization with severe lid margin involvement;

18. Ocular features…
•Keratopathy including scarring, vascularization
and keratinization as a result of the primary
inflammation and/or infection, as well as
cicatricial entropion and aberrant lashes.
•Watery eyes due to fibrosis of the lacrimal
puncta.
•Dry eyes may also occur as a result of fibrosis
of lacrimal gland ductules and conjunctival
metaplasia with loss of goblet cells.
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Corneal Keratinization

20. Systemic features
Skin biopsy may help to establish the diagnosis
but is rarely necessary.
Symptoms:
•Flu-like symptoms, which can be severe, may last
up to 14 days before the appearance of lesions.
•In many cases the patient is very ill and
hospitalization is required.
•Symptoms of systemic mucosal involvement
include nasal pain and discharge, pain on
micturition, diarrhoea, cough, shortness of
breath, and pain on eating and drinking.
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21. Systemic features…
Signs
•Mucosal involvement is characterized by
blistering and haemorrhagic crusting of the lips.
•The blisters may also involve the tongue,
oropharynx, nasal mucosa and occasionally the
genitalia.
•Small purpuric, vesicular, haemorrhagic or
necrotic skin lesions involving the extremities,
face and trunk.
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Extensive purpuric Lesions Shoulder and Neck area

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Haemorrhagic lip crusting

24. Systemic features…
•These are usually transient but may be
widespread.
•Healing usually occurs within 1–4 weeks, leaving
a pigmented scar.
•Widespread sloughing of the epidermis is
uncommon.
•Target’ lesions showing the classic three zones
are now viewed as characteristic of erythema
multiforme rather than SJS/TEN.
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25. Systemic treatment
•Removal of the precipitant if possible, such
as discontinuation of drugs and treatment of
suspected infection.
•General supportive measures such as
maintenance of adequate hydration,
electrolyte balance and nutrition (especially
protein replacement) are critical.
•Management in a specialist burns unit should
reduce the chance of infection when the
extent of skin involvement is substantial.
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26. Systemic treatment…
•Systemic steroids remain controversial.
•There are reports of increased mortality in older
papers, but later research has raised the possibility
that early short-term high-dose intravenous
treatment may improve outcomes.
•Other immunosuppressants including ciclosporin,
azathioprine, cyclophosphamide and intravenous
immunoglobulin may be considered in selected
cases, but are controversial and controlled trials
are lacking.
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27. Systemic treatment…
•Systemic antibiotics may be given as
prophylaxis against skin or other systemic
infection, avoiding those known to be
•at higher risk of precipitating SJS/TEN.
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28. Ocular treatment
•Acute disease:
•Daily review is advisable initially in most
patients to check the corneas and exclude
symblepharon formation.
•Topical lubricants are used as frequently as
necessary, e.g. hypromellose 0.3%
preservative-free up to hourly, high-viscosity
ointment during sleep.
•Prevention of corneal exposure, e.g. moisture
chambers, gel pads if mechanically ventilated.
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29. Ocular treatment…
•Topical steroids may be used for iritis and for
•conjunctival inflammation, though a benefit for
the latter has not been demonstrated
conclusively.
•Topical cycloplegia (e.g. atropine 1% once or twice
daily) may improve comfort.
•Lysis of developing symblephara with a sterile
glass rod or damp cotton bud.
•A scleral ring, consisting of a large haptic lens, may
help to prevent symblepharon formation.
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31. Ocular treatment…
•Pseudomembrane/membrane peeling can be
considered, although the benefit is unproven.
•Treatment of acute corneal problems such as
bacterial keratitis; the use of prophylactic
topical antibiotics is common, but as there
may be a propensity to adverse drug reactions
a decision should be made on a case basis.
•Conjunctival swabs should be considered for
prophylactic culture. IOP monitoring may be
prudent, using portable tonometry if
necessary.
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32. Ocular treatment…
Chronic disease
•Adequate lubrication, including punctal occlusion
if required.
•Topical transretinoic acid 0.01% or 0.025% may
reverse keratinization.
•Treatment of aberrant lashes.
•Bandage contact lenses (typically gas permeable
scleral lenses) to maintain surface moisture,
protect the cornea from aberrant lashes and
address irregular astigmatism.
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33. Ocular treatment…
•Mucous membrane grafting (e.g. buccal
mucosa autograft) for forniceal reconstruction.
•Corneal rehabilitation may involve superficial
keratectomy for keratinization, lamellar
corneal grafting for superficial scarring
(preferred to penetrating keratoplasty),
amniotic membrane grafting, limbal stem cell
transplantation, and keratoprosthesis
implantation in end-stage disease.
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34. Refernces:
•Kanski’s Clinical Ophthalmology A SYSTEMATIC
APPROACH EIGHTH EDI T ION
•www.mediscape.com
•www.google.com/image/search
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