CICATRIZING DISORDERS : OCULAR CICATRICIAL

PEMPHIGOID, STEVENS-JOHNSON SYNDROME,

TOXIC EPIDERMAL NECROLYSIS

CHAIRPERSON: DR. A.K. GUPTA

MODERATOR: DR. UMA SRIDHAR
PRESENTER : DR. GAURAV SHUKLA
OCULAR CICATRICIAL PEMPHIGOID
INTRODUCTION TO MMP & OCP
• Mucous membrane pemphigoid (MMP) : autoimmune chronic cicatrizing disease
characterized by cytotoxic (type II) reaction in which cell injury results from
autoantibodies directed against a cell surface antigen in the basement membrane zone
(BMZ)

• Bullous pemphigoid antigen II (BP180) and its soluble domains - as possible autoantigen

• Autoantibodies deposited along the basement membrane zone (BMZ) at the epithelial–
subepithelial junction of mucous membranes and occasionally skin

• Ocular mucous membrane pemphigoid, also known as ocular cicatricial pemphigoid

(OCP), is a subset of MMP which primarily affects the conjunctiva (and the mucosa,
including oral, nasal, and esophageal, in lesser frequency)
EPIDEMIOLOGY
• Rare disorder : incidence rates vary from 1 in 8000 to 1 in 50,000

• OCP can begin at least as early as the third decade of life, generally seen in 60-70 years age.

• OCP has a slight but definite preponderance for women at a rate of 2–3 : 1.

• Conjunctival involvement - occur as early as 10 years before other mucosal or skin lesions
develop, or it may occur as much as 20 years following the onset of other lesions
PATHOGENESIS

• Classically described as an autoimmune disease with a “genetic predisposition” & “second hit”
environmental requirement to trigger the onset of the disease.

• Second hit - microbial / chemical.

• Exhibits a type II immune reaction : deposition of immuno-reactants (IgG, IgA, IgM, and/or
complement C 3) along the epithelial BMZ.

• The target auto-antigen is the β4 peptide of α6β4 integrin & epiligrin (α3 subunit of laminin 5)
 The binding of the autoantibody to the autoantigen at the
epithelial basement membrane

CD4 T cells, Plasma Macrophage migration

cells, histiocytes, inhibitory factor, interferon-γ
mast cells in and TGF-β,
substantyia propria
relaese cytokines

Subepithelial fibrosis ,xerosis, meibomian gland dysfunction,

and trichiatic trauma

Ocular surface damage

CLINICAL FEATURES
• A chronic vesiculobullous disease primarily involving the conjunctiva, it frequently affects other
mucous membranes, including the mouth and oropharynx, genitalia, and anus

• Generally present with recurrent attacks of non specific unilateral conjunctivitis

• Early stages – fine white perivascular lines in inferior & superior tarsal conjunctiva

• Chronic papillary conjunctivitis

• Symptoms – burning, FB sensation, tearing, photophobia, itchiness, pain & mucoid discharge

American academy of ophthalmology Cornea/external disease 2016-17

CLINICAL FEATURES
• Cornea-
• Superficial punctate keratitis
• Epithelial defect
• Stromal ulcer
• Neovascularization
• Keratinization
• Limbitis
• Conjunctivalization of cornea
• Stromal opacity
Corneal neovascularization with ulceration
• Perforation
and stromal thinning after persistent
epithelial defect
American academy of ophthalmology Cornea/external disease 2016-17
CLINICAL FEATURES
• Sustained fibrosis – conjunctival shrinkage, fornix shortening

• Progressive fibrosis – Symblephara ( 1st inf fornix)

• Recurrent attacks of conjunctival inflammation causes loss of goblet cells – mucin

deficiency leads to severe dry eye

• Cicatrization results in obliteration of lacrimal ductules

• Cicatricial entropion, trichiasis, dystichiasis, lagophthalmos

• Ultimately Ankyloblepharon, severe keratopathy, neovascular pannus & scarring of

cornea – deterioration of vision

• Severe disease may even lead to perforation

American academy of ophthalmology Cornea/external disease 2016-17
 STAGING OF OCP
Two major staging classifications :-

Mondino and Brown (1981) Foster

Based on forniceal shortening Based on progression of the disease

Stage 1 : < 25% shortening Stage 1 : Subepithelial fibrosis

Stage 2 : 25-50% Stage 2 : Shortening of inf. fornix

Stage 3 : 75% Stage 3 : Symblepharon

Stage 4 : End stage OCP Stage 4 : Ankyloblepharon &

keratinization
 STAGE 1 PEMPHIGOID
Subepithelial fibrosis of tarsal
conjunctiva

STAGE 2 PEMPHIGOID
Inferior Forniceal shortening
 STAGE 3 PEMPHIGOID
Symblepharon formation

STAGE 4 PEMPHIGOID
Total obliteration of the inferior
fornix, the ankyloblepharon
formation, and keratinization of
the corneal surface.
MODIFIED FOSTER GRADING (1992 TAUBER
ET AL. )
• STAGE I : Chronic conjunctivitis, subconjunctival fibrosis
• STAGE II : Inferior forniceal shortening
• IIa : 0-25%
• IIb : 25-50%
• IIc : 50-75%
• IId : 75-100%
• STAGE III : Presence & extent of horizontal symblepharon
• IIIa : 0-25%
• IIIb : 25-50%
• IIIc : 50-75%
• IIId : >75%
• STAGE IV : Ankyloblepharon imobilize the globe
OTHER MUCOSAL TISSUES/ SKIN
INVOLVEMENT
• Scarring ( Brusting-Perry) dermatitis may occur in approximately 25% of cases

• Involvement of other mucosa may lead to scarring of the soft palate and oral
and nasal mucosa

• Esophageal, urethral, vaginal, and anal strictures may develop

• Laryngeal involvement - pain and hoarseness

• The esophageal scarring can lead to asphyxiation if a food bolus lodges during
attempted swallowing
 DIAGNOSIS

• Extremely important to prevent the inevitable blinding sequalae of OCP, Since the initial symptoms of
OCP are nonspecific and easily misdiagnosed

• Too often, patients present during the later stages of the disease

• A High level of clinical suspicion in cases of unexplained chronic, recurrent conjunctivitis, especially
when there is any evidence of subepithelial scarring.
DIAGNOSIS
DIRECT IMMUNOFLUORESCENCE
• Gold standard for diagnosis of OCP is a conjunctival MICROSCOPY
biopsy using immunofluorescent or immuno-
peroxidase techniques of fresh specimen

• If immediate testing cannot be performed, then the

specimen can be placed and maintained in Zeus or
Michel's fixative

• Homogeneous linear deposition of immuno-
reactants, such as IgG, IgA, IgM, and Complement 3
component (C3) along the BMZ of inflamed
conjunctiva, is diagnostic of OCP
Note the bright apple-green linear pattern of fluorescence
at the conjunctival epithelial basement membrane zone,
indicating the deposition of IgG, IgA, IgM at the
basement membrane, providing immuno-histopathologic
confirmation of the suspected clinical diagnosis.
 DIFFERENTIAL DIAGNOSIS :-

*Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment Panagiotis Georgoudis . Francesco
Sabatino . Nora Szentmary . Sotiria Palioura . Eszter Fodor . Samer Hamada . Hendrik P. N. Scholl . Zisis Gatzioufas (January 2019)
MANAGEMENT
MEDICAL MANAGEMENT –
A multidisciplinary approach is often required with the
involvement of ophthalmologists, dermatologists, oral
surgeons, primary care physicians and ENT surgeons

Stepladder regimen by Saw et al. 2008

A. Mild to moderate inflammation :

• Dapsone 1st line 25mg BD starting dose after excluding
sulpha allergy & G6PD deficiency.
• Methotrexate (15–25 mg once weekly) can be added to
dapsone
• Azathioprine or Mycophenolate mofetil (1–3 g/day) are
next agents
B. Severe inflammation & rapidly progressive disease : -

• Cyclophosphamide -oral / i.v. (1.5-2.0mg/kg per day) plus

short course of high dose systemic steroid

• Cyclophosphamide dose is adjusted according to therapeutic

response, bone marrow response, and drug tolerance

• Once control achieved steroids to be tapered within 12 weeks

preferably

C. Recalcitrant infection: -
• Multidrug treatment
• Intravenous immunoglobulin ( IVIG)
• Biologic agents such as Rituximab, infliximab, or Etanercept
THERAPEUTIC AGENTS
THERAPEUTIC AGENTS

Rituximab,
Infliximab,
Etanercept
 (23 January 2017)

Purpose :The purpose was to evaluate the effectiveness and safety of rituximab (RTX) for the treatment of
patients with aggressive ocular cicatricial pemphigoid (OCP)

Methods : Sixty-one eyes of 32 patients with symptomatic OCP who received treatment with RTX
monotherapy or RTX in combination with additional immunomodulatory treatment (IMT) were evaluated

32 Patients failed on different drugs

Conclusions:- These retrospective data
suggest that RTX is efficacious and well
tolerated when included for the treatment of
OCP.
 (January 2019)

In the case of failure of conventional immunosuppressive treatment, Anti-CD20 monoclonal

antibody rituximab , IVIg treatment, or a combination of the above has been described
 SURGICAL MANAGEMENT

EYELIDS/LASHES -
• Epilation, Cryoablation, Electrolysis
• Temporary or permanent punctal occlusion
• Marginal eyelid rotation
• Hard palate and buccal mucosal grafting can be useful in severe cases

CONJUNCTIVA –

• Fornix reconstruction with AMG & 0.04% MMC or autologous oral mucosa
• Limbal Stem Cell Transplant (LSCT)
 SURGICAL MANAGEMENT
CORNEA :
• Tarsorrhaphy – in decreased corneal sensations
• PK (with a very guarded prognosis )
• Keratoprosthesis can be used with some success

CATARACT :
• Should be operated for some improvement in VA after disease
control with immuno-suppressive agents
• Small clear corneal incision technique to avoid conjunctival
damage
SUPPORTIVE MANAGEMENT
• Treat the ocular surface disorder

• Preservative free artificial tear drops and ointments, AST ( autologous serum tears)

• Chronic blepharitis - lid hygiene, massage and oral doxycycline

• Punctal occlusion

• Fluid ventilated gas permeable lenses

• Newer therapies : TNFα inhibitors, Rituximab

PSEUDOPEMPHIGOID

• Drug-induced conjunctival cicatrization

• In most instances, appears to behave like mucous membrane pemphigoid

• Is self-limiting once the offending medication is stopped

• Long-term topical adrenaline, Idoxuridine, guanethidine, dipivefrin, pilocarpine, timolol, echothiophate

iodide (phospholine iodide) and demecarium bromide are commonly implicated
STEVENS-JOHNSON SYNDROME,
TOXIC EPIDERMAL NECROLYSIS
INTRODUCTION
• Erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN)
have traditionally been regarded as across-the-spectrum manifestations of the same clinical entity
affecting skin and mucous membrane

• All three conditions are precipitated by reactions to drugs or infectious diseases (eg, herpes
simplex virus, adenovirus) that result from the presentation of major histocompatibility complex
(MHC) class I–restricted antigens, leading to infiltration of the skin with cytotoxic T lymphocytes
and natural killer cells

• Approximately 80% of TEN and 50%–80% of SJS cases are thought to be drug induced
• Erythema multiforme minor primarily involves the
skin. It usually does not involve the eye

• Erythema multiforme major - characterized by both

skin lesions and involvement of two or more
mucosal surfaces; systemic symptoms are much
more common

• Toxic epidermal necrolysis - Most severe, potentially

life-threatening disease, involvement of over 30% of
their epidermis, and skin can be sloughed from the
body in sheets
HISTORY
• Ferdinand von Hebra, in 1866, first described erythema multiforme.

• Two American physicians, Stevens and Johnson, reported two classic cases in children &
named the disease eruptive fever with stomatitis and ophthalmia in 1922. Came to be known
as Stevens Johnson syndrome

• Thomas, in 1950, coined the terms erythema multiforme minor and major : EM minor to
what von Hebra described and EM major to SJS

• Lyell, in 1956, introduced the term toxic epidermal necrolysis

INCIDENCE AND PREVALENCE
• American study, reported an incidence of EM, SJS or toxic epidermal necrolysis resulting from all
causes at 4.2 to 5 per million per year

• Erythema multiforme may be more common in patients with acquired immunodeficiency

syndrome (AIDS), 1.0 per 1000 AIDS cases

• Males have a higher incidence of erythema multiforme 3 : 1. Peak incidence is in the 2nd & 3rd
decades of life
• Toxic epidermal necrolysis is slightly more common in women, with a ratio 2 : 1.

• The elderly have an increased incidence of toxic epidermal necrolysis and also have a higher rate
of morbidity and mortality

• American academy of ophthalmology Cornea/external disease ;2018-19

ETIOLOGY
• Drug-related cases of Erythema multiforme have a clinical spectrum that typically arises within 3 weeks
after initiation of drug therapy and within hours after re-exposure to an inciting agent

• From 50% to 60% of erythema multiforme cases are secondary to drugs

• Sulfonamides main agents causing erythema multiforme in healthy patients as well as in patients with
HIV or AIDS
• Other medications linked to erythema multiforme, including :
• Phenytoin
• Barbiturates
• Phenylbutazone
• Penicillin
• Salicylates
• Ophthalmic drops such as dorzolamide, or tropicamide
• Erythema multiforme can be due to
infections –
• Herpes simplex
• Mycoplasma pneumoniae
• Measles infection
• Mycobacterium
• Group A streptococci
• Epstein-Barr virus
• Yersinia
• Enterovirus

• Herpetic infection was found to be more prevalent

in cases of EM major, while drugs tended to be
linked more to SJS and TEN
PATHOGENESIS
• EM and TEN appear to be immune-mediated responses to certain drugs and infectious organisms

• Keratinocyte death occurs from extensive apoptosis, triggered by drug-specific cytotoxic T lymphocytes
via the perforin–granzyme pathway

• Apoptosis is induced by a suicidal interaction between Fas and Fas ligand (Fas L) which is either
membrane bound on keratinocytes

• Ocular lesions of Stevens-Johnson syndrome have a significantly increased incidence of HLA-B12, HLA-
Aw33, and DRw53

• Herpes simplex-induced erythema multiforme is related to HLA-DQw3

• An increased incidence of HLA-B12 was also seen in white patients with toxic epidermal necrolysis
CLINICAL FINDINGS
• INITIAL PRESENTATION-
• A systemic prodrome of malaise, fever, and headache or symptoms of an upper respiratory tract
infection may precede the ocular and dermatologic manifestations and can begin 1 to 3 weeks after
initial exposure and within hours after re-exposure to an inciting agent.

• Prodrome more severe in Stevens-Johnson syndrome and in toxic epidermal necrolysis –

• High fever
• Muscular pain
• Nausea & vomiting
• Diarrhoea
• Migratory arthralgias
• Pharyngitis
• The distinctive pathologic changes of SJS are
subepithelial bullae and subsequent scarring

• Skin eruption follows within a few days, with a classic

“target” lesion consisting of a red center surrounded
by a pale ring and then a red ring

• The mucous membranes of the eyes, mouth, and

genitalia may be affected by bullous lesions with
membrane or pseudo-membrane formation
 ACUTE EYE FINDINGS
• Initially, in SJS and TEN, a nonspecific conjunctivitis usually
occurs at the same time as lesions on the skin and other mucous
membranes

• The bilateral conjunctivitis may be catarrhal or

pseudomembranous & occurs in 15–75% of patients with
Stevens-Johnson syndrome

• Secondary purulent bacterial conjunctivitis can complicate the

initial ocular involvement.

• Conjunctival and corneal epithelial sloughing and necrosis with

severe inflammation and scarring may develop in severe cases

• Monocular involvement is unusual

CHRONIC EYE FINDINGS
• Scarring, symblepharon formation, and cicatrization of the conjunctiva
may result from the initial inflammatory process leading to entropion
formation, trichiasis, and instability of the tear film

• Breakdown of the ocular surface leads to corneal scarring,

neovascularization, and keratinization

• Eyelid margin keratinization and scarring is an important risk factor for

poor long-term outcomes

• Keratin accumulates along the posterior lid margin, characteristically

extending on to normally non-keratinized palpebral conjunctiva

• Subepithelial fibrosis of the conjunctiva

CHRONIC EYE FINDINGS
• SJS patients are at higher risk of infection due to loss of the epithelial barrier and hence may
develop severe ocular infection

• Cicatrization of the lacrimal ducts + destruction of the conjunctival goblet cells - severe dry eye

• Loss of goblet cells and their mucus secretion – unstable tear film, with poor wetting of the cornea

• Entropion, trichiasis, and lid margin keratinization result in chronic irritation of the cornea and
resultant persistent epithelial defects
OCULAR COMPLICATIONS
• In severe cases, acute ocular involvement may cause keratitis, corneal perforation, and
endophthalmitis

• Chronic complications :
• Conjunctival scarring
• Symblepharon
• Entropion
• Dry eye syndrome
MANAGEMENT - SYSTEMIC DISEASE
• Immediate discontinuation of the offending agent

• Careful monitoring of fluid balance, respiratory function, nutritional requirements, and wound care is
crucial - best managed in an intensive burn care unit

• More than half of all deaths occurring in toxic epidermal necrolysis are secondary to SEPSIS, so control of
infection is critical – Silver nitrate solution on the denuded skin

• The use of systemic corticosteroids (prednisolone 1mg/kg/ day for 3 days ) and i.v. immunoglobulins is
helpful in reducing mortality and morbidity
• High-dose corticosteroids may arrest the necrolysis and benefit the patient’s systemic recovery

• Disadvantages of employing systemic steroids - increased susceptibility to infection, masking of the early
signs of sepsis, gastrointestinal haemorrhage, impaired wound healing, and prolonged recovery
• Examples of fluorescein staining patterns in acute Stevens-Johnson
syndrome
• A, Mild: Only mild conjunctival injection with no epithelial sloughing.
Medical treatment recommended
•
• B, Moderate: Less than 1-cm of discrete epithelial sloughing involving
only the bulbar conjunctiva. Medical treatment recommended

• C, D, E, Severe: All have more than a 1-cm of palpebral conjunctival

sloughing with minimal bulbar conjunctival sloughing: (C) minimal
sloughing elsewhere, (D) sloughing of more than one-third of the lid
margin, and (E) extensive corneal sloughing.

• For all 3 cases, sutured amniotic membrane transplantation (AMT) is

recommended for the lid margins and palpebral conjunctiva, with
ProKera application to the surface of the globe.

• F, G, H, Extremely severe: Extensive sloughing of all the ocular

mucosal surfaces. Sutured AMT is recommended for the lid margins,
palpebral conjunctiva, and entire surface of the globe. Repeat AMT
may be needed 7 to 10 days after the initial AMT in such cases
 IMPORTANT POINTS-

• The parameters of 1 cm of conjunctival staining and one-third of the lid margin are relatively easy to
identify at the bedside and act as useful guide to predict the severity of inflammation

• Areas of conjunctival staining less than 1 cm in diameter without coexistent corneal or lid margin staining
may be treated with topical medications

• Staining involving more than one-third of the lid margin also is concerning because of high risk of scarring
of the meibomian gland orifices and subsequent dry eye problems

• Extent of fluorescein staining is crucial because it indicates how much normal tissue remains to repopulate
the damaged areas that slough off
• If AMT is considered necessary, it should be performed as soon as possible
• Using these above guidelines described, we can decide which patients require urgent AMT and
which patients may be observed and treated with more conservative measures

• In extremely severe cases, the need to repeat the AMT in the acute phase is more likely

• Beyond the first week after the onset of symptoms, the effectiveness of AMT begins to
decrease *

• (Cherof AM. Acute StevensJohnson syndrome: the effect the timing of amniotic membrane
transplantation has on the occurrence of significant ocular sequelae. November 16, 2015)
• Purpose: To highlight and compare the outcomes of management for the ocular sequelae of Stevens–
Johnson syndrome (SJS) over 25 years in a tertiary eye care institute.
• A retrospective study of 798 eyes of 399 patients with SJS evaluated between January 1990 and
December 2004 (group I) and of 847 eyes of 517 patients between January 2005 and December 2014
(group II)
• Groups were subdivided into procedures for ocular surface stabilization (A) and visual rehabilitation (B)
and those managed conservatively (C)
• Following parameters were analyzed for the outcome measures:
• 1. best-corrected visual acuity (BCVA);
• 2. Schirmer I test (graded as 1- <5 mm; 2- 5–10 mm; 3, 11–15 mm; and 4, >15 mm)
• 3. fluorescein corneal staining scores from 0 to 9
• Subgroup A -included procedures for surface stabilization and fornix reconstruction that included-
• Punctal occlusion (collagen or silicone plugs),
• Adnexal corrective procedures,
• Symblepharon release with amniotic membrane grafting in group I and

• Punctal cautery, MMG for lid margin keratinization, prosthetic replacement of ocular surface ecosystem (PROSE)
lenses, symblepharon release with amniotic membrane grafting and MMG , cultivated oral mucosal epithelial
transplantation, and adnexal corrective procedures in group II

• Subgroup B included- limbal allograft with penetrating keratoplasty with systemic azathioprine (2 mg/ kg) for 6
months to 1 year in group I and cataract extraction/ optical iridectomy/keratoprosthesis in group II

• Subgroup C included conservative management that comprised primarily artificial tear substitutes and topical
steroids in group I and artificial tear substitutes in group II
 RESULTS
• In 54.3% (434) of eyes in group I, which did not undergo
any surgical intervention and received only medical
treatment, a significant deterioration in BCVA was noted.
• In group II, 114 (13.4%) eyes of 57 patients did not undergo
any intervention
• Limbal status (increase in corneal vascularization)
worsened in 66.9% of eyes treated conservatively and in
60.4% of eyes after intervention in group I
• The limbal status stabilized/improved (decrease in corneal
vascularization) in 96.6% (630/652) of eyes treated by
procedures for surface stabilization (punctal cautery, MMG,
and fornix reconstructive procedures) in group II.

Conclusions: Stabilization procedures show a beneficial role while conservative management can lead to deterioration in
chronic ocular sequelae of SJS.
Keratoprosthesis, specifically the modified osteo-odonto-keratoprosthesis, forms the mainstay for visual rehabilitation in
the end-stage disease
 ( JUNE 2019)

• Stabilizing the ocular surface

• 1. Dry eye– the most common sequelae of SJS, is often treated with topical
lubricants.
• In addition, in eyes with the severe dry eye with absent emotional and reflex
tearing, punctual cautery is very useful
• In cases of severe dry eye, prosthetic replacement of ocular surface ecosystem
(PROSE) lenses have a role to play in improving the comfort of the patient
• For grades 2 and above, excision with Mucus membrane grafting is advisable to
reduce the microtrauma to the ocular surface
• Tectonic procedures -
• Include those procedures that are applicable for a persistent epithelial defect or corneal perforation

• Such as use of cyanoacrylate glue, amniotic membrane grafting (AMT), lamellar or full-thickness patch grafts
or keratoplasties

• Visual rehabilitation -

• Optical iridectomy can be attempted to improve vision in eyes with central or paracentral corneal opacities
with a relatively clear zone of the peripheral cornea

• Keratoprosthesis forms the last resort for visual rehabilitation in bilateral end-stage disease

• Modified osteo-odonto keratoprosthesis (MOOKP) forms the first choice of Kpro in eyes with SJS

• Type 2 Boston Kpro or the osteo Kpro can be performed in eyes not suitable for the MOOKP
MANAGEMENT - OPHTHALMIC DISEASE -
ACUTE STAGE
• Ocular surface hygiene should be maintained - frequent conjunctival irrigation and installation
of prophylactic antibiotics to combat secondary infection

• Frequent preservative-free artificial tear supplements should be used to lubricate the corneal
and conjunctival epithelium

• Topical steroids decrease ocular inflammation but can also cause secondary bacterial infection :
so close follow up is required
MANAGEMENT - OPHTHALMIC DISEASE -
ACUTE STAGE
• Bacterial keratitis  Rapid corneal perforation

• Daily lysis of the symblepharon with glass rod

• Symblepharon ring in association with a bandage soft contact lens to line

the palpebral surface and prevent symblepharon formation

• Cryopreserved amniotic membrane can be applied to the bulbar and tarsal

conjunctiva and the cornea

• Lamellar or penetrating keratoplasty can be used if perforation is

impending or occurs
MANAGEMENT - OPHTHALMIC DISEASE -
CHRONIC STAGE
• Goals of treatment are to:-
1. Restore eyelid and forniceal anatomy and function
2. Supplement tear function
3. Restore ocular surface

• Trichiasis is a constant and recurring problem – Epilation, cryotherapy, electrolysis to destroy lashes

• Entropion repair, combined with mucous membrane grafting for correcting shortening of the fornices

• Nasolacrimal duct and canalicular obstruction can occur, which sometimes help with DRY EYE state

• In case of dacrocystitis or chronic epiphora occurs, dacryocystorhinostomy and silicone tube insertion
MANAGEMENT - OPHTHALMIC DISEASE -
CHRONIC STAGE
• Frequent artificial tear supplementation to treat the keratoconjunctivitis sicca due to conjunctival scarring
and loss of goblet cells

• Non - preserved methylcellulose lubricants

• Mucolytic agents (10% N-acetylcysteine) used to control filament formation or abnormal mucous discharge

• Lateral tarsorrhaphy - decreasing the surface area available for evaporation

• Bandage soft contact lenses can be used to manage persistent epithelial defects
MANAGEMENT - OPHTHALMIC DISEASE -
CHRONIC STAGE
• Keratoprosthesis have been described for use in patients with Stevens-Johnson syndrome who
have poor epithelial healing

• Potential complications associated with keratoprosthesis use include :

• Eyelid cellulitis
• Extrusions of the keratoprosthesis
• Aqueous leaks
• Retro-prosthetic membranes
• Endophthalmitis
• Retinal detachment
• Progressive glaucoma
KERATOPROSTHESIS
• Improved results have recently been seen with the Boston K Pro due to
prophylactic use of vancomycin and more aggressive treatment of
glaucoma

• For more advanced cases with extremely dry eye osteo-odonto

keratoprosthesis (OOKP) may be the only option
 KERATOLIMBAL ALLOGRAFT
(KERATOEPITHELIOPLASTY)
• Lenticules of cadaveric kerato-limbal, living-related conjunctivo-limbal, combination of the
above, or cultivated alloepithelial donor limbal tissue, with or without amniotic membrane,
are transplanted to the limbus of an affected eye after superficial keratectomy

• Once the epithelium has been stabilized by performing the kerato-limbal graft, penetrating
keratoplasty can be performed to improve vision

• Penetrating keratoplasty should be performed months after the kerato-limbal allografts

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