OCULAR SURFACE

DISEASE
Ruth Antolin
1st Year

COMMON CLINICAL FINDINGS

Eyelids

Common Clinical Findings of the External Eye Exam

Tissue

Finding

Description

Macule

Spot of skin color change

Papule

Solid raised spot

Vesicle

Blister filled with serous fluid

Bulla

Large blister

Pustule

Pus filled blister

Keratosis

Scaling from accumulated keratinizing cells

Eczema

Scaly crust on a red base

Erosion

Excoriated epidermal defect

Ulcer

Epidermal erosion with deeper tissue loss

Conjunctival Signs

Papillae

Elevated, polygonal, hyperemic mounds

Vascular changes seen on the palpebral

conjunctiva

fibrous septa: anchor the conjunctiva to

the tarsus

Has a central red dot: dilated capillary

Smooth velvety appearance

With progression: surrounded by

capillaries

Chronic/ progressive changes: vascular

tufts which obscures the underlying
vessels

Giant papillae: > 0.3mm dm

Follicles

Round or oval clusters of

lymphocytes

Follicular conjunctivitis:

involves redness and new or

enlarged follicles

Vessels surround and encroach the

raised surfaces

seen usually in the inferior and

superior tarsal conjunctiva

Table 1-1. Conjunctival Signs

Finding

Examples

Papillary conjunctivitis

Allergic
Bacterial

Follicular conjunctivitis

Bacterial (Molluscum contangiosum,

Chlamydia)
Viral (HSV, Adenovirus)
Drug induced

Conjunctival pseudomembrane/
membrane

Severe viral/ bacterial conjunctivitis*

SJS
Chemical burn

Conjunctival granuloma

Cat scratch disease

Sarcoidosis
Foreign body

Conjunctival erosion/ ulceration

SJS
Mucous membrane pemphigoid
Graft vs. Host disease
Factitious conjunctivitis

Corneal Signs

Cornea

Keratitis

Can be described according to the following

Distribution: diffuse, focal, multifocal

Depth: epithelial, subepithelial, stromal, endothelial

Location: central, peripheral

Shape: dendritic, disciform etc

Cornea

Punctate Epithelial Keratopathy: non specific term that includes a spectrum of

biomicroscopic changes

Punctate epithelial Granularity - erosive and inflammatory changes

Punctate Epithelial Erosions: staining lesions of abnormal and degenerated

corneal epithelial cells

Stromal inflammation: presence of new blood vessels

Epithelial defect

Direct interlamellar infiltration

Endothelial injury

Cornea

Stromal inflammation

Suppurative vs. non suppurative

Distribution: focal vs non focal

Location: central, paracentral, peripheral

Necrotizing stromal keratitis is a severe form of infiltrate without liquefaction

associated with suppuration

Endothelial dysfunction

Inflammatory pseudoguttae: dark areas of normal mosaic pattern

Keratic precipitates: clumps of inflammatory cells during active inflammation

Fibrin, clumped proteins

Neutrophils and lymphocytes: punctate opacities

Macrophage: Large mutton fat clumps

Cornea

Production of corneal scar is said to be brought about by:

Altered stromal keratocytes

Accumulation of different complexes: calcium, lipids, proteinaceous material

Neovascularization

Superficial stromal blood vessels from palisades of Vogt

Pannus

Usually remain at a single lamellar plane unless stroma becomes disorganized

CLINICAL APPROACH TO DRY EYE

16 of 80

Dry Eye

Multi factorial disease

Discomfort, visual disturbance and tear film instability with potential damage
to ocular surface

Disturbance of the LFU

LFU regulates the major components of tear film

Responds to environmental, endocrinologic and cortical influences

Overall functions

Tear film integrity (lubricant, antimicrobial, nutritional)

Ocular surface health: maintains corneal transparency and surface stem cell population

Image quality

Dry Eye

Most common reason for ophthalmic consult

Prevalence increases with age

F>M

Equal amongst all racial and ethnic groups

It is associated with poor quality of life especially those patients with

moderate to severe dry eyes.

MECHANISM OF DRY EYE

Driven by:

Tear hyperosmolality breakdown of the surface epithelium

Tear film instability

xerophthalmia, allergy, contact lens wear, diet, prolonged use of video usage, prolonged
topical medications with preservatives

Obstructed tear delivery (cicatricial conjunctival scarring, loss of sensory reflex drive to
the lacrimal gland)

Inflammation

Epithelial injury

Aqueous deficient vs Evaporative

Aqueous Tear Deficient

Evaporative

Inflammation of the lacrimal gland

Diminished tear production
Propagation of inflammatory
mediators on the ocular surface

Primary abnormality is meibomian

gland dysfunction
Obstruction of the glands
Altered lipid metabolism

Tear film instability

Tear evaporation
Tear hyperosmolality
INFLAMMATION

AQUEOUS TEAR DEFICIENCY

Mild to severe disabling disease

Symptoms tend to be worse toward the end of the day

Prolonged use of eyes

Exposure to environment

Symptoms:

Burning, dry sensation, photophobia, blurred vision

Stare test: blurring of vision below 8 seconds

Signs:

Hyperemia, decreased tear meniscus, irregular corneal surface, tear film debris

AQUEOUS TEAR DEFICIENCY

Assess inferior tear meniscus: 1.00mm height and convex (0.3mm abnormal)

(+) epithelial keratopathy : rose Bengal/ lissamine green

Nasal/ temporal limbus, inferior paracentral cornea

Filaments and mucous plaques

Marginal and paracentral thinning

Band keratopathy

Keratinization of the cornea and conjunctiva

TREATMENT

Imperative to carefully examine the eye for other structural disorders such as
conjunctivochalasis, floppy eyelid, superior limbic keratoconjunctivitis

Determine if with systemic symptoms/ medications

Artificial tear supplementation, topical cyclosporine, topical steroids, omega

3 fatty acid supplements

TREATMENT: MEDICAL MANAGEMENT

Aqueous Tear Deficiency

Lifestyle change: smoking cessation, environment; use of moisture shields/

humidifier; discontinuation of medications

Topical tear substitutes: mainstay of treatment

Preservative free is usually recommended

Demulcent solutions: hypromellose

Brief substitute for glycoproteins lost late in the disease process

But cannot be used for restoration of lost glycoproteins

Topical cyclosporine 0.05%: anti inflammatory; used early in the disease

In combination with steroids

Dilute solutions of hyaluronic acid and autologous serum drops: trophic functions

TREATMENT: MEDICAL MANAGEMENT

Filamentary keratopathy:

acetylcysteine 10%

Topical low dose steroids, cyclosporine, tacrolimus

Contact lenses: therapeutic, soft, scleral

Use of goggles, shields, moisture bubbles

Pharmacologic stimulation of tear secretion: pilocarpine and

cevimeline

Approved for xerostomia, long term benefit ?

Diet: omega 3 fatty acids

Recommended Treatment for Aqueous Tear

Deficiency
Severity

Therapeutic Options

Mild

Artificial tears with preservatives 4x/ day

Lubricating ointment at bedtime
Hot compresses and eyelid massage

Moderate

Artificial tears without preservatives 4x/ day to hourly

Lubricating ointment at bedtime
Topical anti inflammatory treatment (cyclosporine 0.05% 2x daily)
Reversible occlusion, lower puncta plugs

Severe

All of the above

Punctal occlusion, lower and upper
Topical serum drops 20% 4-6x daily
Topical corticosteroids (non preserved)
Moist environment
Tarsorrhaphy
Bandage Lenses

EVAPORATIVE DRY EYE

Symptoms: burning, foreign body sensation, redness of the lids and

conjunctiva, filmy vision worse in the morning

Confined to the posterior eyelid margin: irregular, prominent blood vessels

(telangiectatic)/ brush marks

Metaplasia of the Meibomian glands

White plug of keratin protein found within the glandular orifice

Secretions are turbid and more viscous

EVAPORATIVE DRY EYE

Non obvious obstructive MGD

Symptomatic; no obvious clinical signs of meibomian disease

Mild compression of the glands: obstruction; filamentous secretion due to

narrowing of the ducts

Atrophic meibomian glands

Shortening/ absence of the vertical lines of the MGs

Other tear findings:

Foam in tear meniscus

Linear staining along inferior cornea and inferior conjunctiva

Episcleritis

Infiltrates: marginal epithelial and subepithelial

Pannus

Corneal scarring/ thinning

TEAR BREAKUP TIME

Functional measure of tear stability

In MGD: rapid TBUT- less than 10 seconds

Flourescein strip is used, applied to tarsal conjunctiva, then tear film evaluated with blue
filter light using broad beam

No manipulation should be done prior/ instillation of any eye drops

TREATMENT: MEDICAL MANAGEMENT

Evaporative Dry Eye

Based on staging

Eyelid hygiene: mainstay

Warm compress, massage, cleaning of the eyelid margin (non irritant shampoo/
blephagel/ dilute sodium chloride solution 1tsp salt: 1 pint water

Topical antibiotics: ophthalmic azithromycin

Systemic antibiotics: tetracyclines; long term

Side effects: photosensitization, GI upset, azotemia; long term use: oral/ vaginal
candidiasis

Contraindicated: children below 8 years

TREATMENT: MEDICAL MANAGEMENT

Topical steroids: moderate to severe inflammation; corneal infiltrates/

vascularization

Diet: omega 3 1000mg 3x a day- not yet established

Gentle pulsatile pressure and thermal energy increased blood flow to the lids,
open obstructed MGs

TREATMENT: SURGICAL MANAGEMENT

For patients with severe disease not refractory to meds or are impractical

Punctal occlusion: collagen implants, silicon punctal plugs

Can be dislodged

Can be inadvertently inserted into the nasolacrimal system

Complications: conjunctival abrasion, granuloma formation, reduction in tear flow

Irreversible punctal occlusion: disposable cautery, radiofrequency probe

Indications: minimal basal tear secretion, punctate keratopathy with no significant

ocular inflammation/ infection

Correction of eyelid malposition

Treatment Algorithm for Meibomian

Gland Dysfunction

Stage I

NO SYMPTOMS of ocular discomfort

Tx: Patient education (causes) and symptomatic management; eyelid hygiene,

warm compress

Stage 2

MINIMAL TO MILD symptoms

MINIMAL TO MILD clinical signs

scattered along the eyelid margin

Tx: Modify environmental factors, eyelid hygiene, topical meds (eye lubricants,
azithromycin, emollient lubricants, oral tetracycline derivatives)

Treatment Algorithm for Meibomian

Gland Dysfunction

Stage 3

MODERATE symptoms with limitation of activities

MODERATE signs

Increased eyelid margin features: plugging, vascularity

Tx: all of the above plus oral tetracycline, lubricant ointment at bedtime, (+) anti
inflammatory therapy for dry eye

Stage 4

MARKED symptoms with definite limitation of activities

SEVERE signs: dropout, displacement, increased signs of inflammation with

hyperemia, and/or phlyctenules

Tx: all of the above + anti inflame therapy

Treatment Algorithm for Meibomian

Gland Dysfunction

Plus disease

Can be secondary to MGD but can also occur incidentally

Exacerbated inflammatory ocular surface disease

Mucosal keratinization

Phlyctenular keratitis

Trichiasis

Chalazion

Anterior blepharitis (also includes demodex)

Tx: steroids, BCL/ SCL, epilation, cryotherapy, Topical antibiotcs, tea tree oil
scrubs

OTHER EYELID DISEASES ASSOCIATED

WITH OCULAR SURFACE DISORDERS

Rosacea

Pathogenesis

Chronic, acneiform disorder

Skin and eye

No proven cause; may be related to overexpression of cathelicidin

antimicrobial keratitis

Cutaneous sebaceous gland dysfunction of the face, neck and vit hem

More common in fair skinned

Clinical Presentation

Excessive sebum secretions with chronic blepharitis

Eyelid margin telangiectasia; MG distortion- chalazia

Ocular surface involvement- conjunctivitis, marginal corneal infiltrates,

ulceration, episcleritis, iridocyclitis, *corneal neovascularization, scarring

Rosacea

Clinical Presentation

30- 60 years old

F>M

Facial lesions: telangiectasia, recurrent papules, pustules, midfacial erythema

Malar rash associated with consumption of alcohol

Rhinophyma- thickening of the skin and connective tissue of the nose

Rosacea

Tx:

Systemic tetracyclines (doxycycline, minocycline)

Topical metronidazole/ azelaic acid gel for facial erythema

Papulopustular rosacea

Ulcerative keratitis (sterile)- topical corticosteroids

Scarrring and neovascularization- poor prognosis (PKP); conservative management

Eyelid erythema- light pulse treatment

Seborrheic Blepharitis

Occur alone or in association with staphylococcal blepharitis or

MGD

Occurs at the anterior eyelid margin

Crusting

Eyelids, eyelashes, scalp, eyebrows

Turbid secretion of the meibomian gland

Burning sensation, chronic eyelid redness and FB sensation

15%: Keratitis: punctate epithelial erosions

1/3 have evaporative dry eye

Tx: eyelid hygiene

Selenium sulfide for patients with scalp disease

Staphylococcal Blepharitis

Caused usually by S. Aureus

Bacterial infection of the eyelids, conjunctiva

Younger population

Sx:

burning, itching (AM)

FB sensation and crusting upon awakening

Signs:

Scales
Matted crusts per cilia
Ulcers
Eyelid margin injection and
telangiectasia

Poliosis
Madarosis
Trichiasis

Staphylococcal Blepharitis

May present as chronic ( > 4 week duration) conjunctivitis

Papillary reaction of the tarsal conjunctiva (inferior)

Bulbar, tarsal injection

Scant mucopurulent discharge

ATD/ lipid induced tear film instability

S. Aureus

Matted golden crusts

ulcers

inferior punctate keratopathy

marginal corneal infiltrates

Staphylococcal Blepharitis

Conjunctival/ corneal phlyctenules (M. tuberculosis)

Unilateral

Limbus

Round, elevated, yellowish to grayish, hyperemic with zone of engorged vessel

M. lacunata

Chronic angular blepharoconjunctivitis- associated with S. aureus

Staphylococcal Blepharitis

Laboratory evaluation

initial diagnosis is questionable

refractory to treatment

Chronic unilateral cases

Masquerade syndrome (conjunctival malignancy)

Factitious illness

Lab finding: (+) heavy confluent growth of S. Aureus

Other bacterial growth does not exclude the diagnosis

PEK, marginal infiltrates, phlyctenulosis

Susceptibility testing: refractory to empiric treatment

Staphylococcal Blepharitis

Tx:

eyelid hygiene (base of lashes)

Topical bacitracin, erythromycin or azithromycin

Antibiotic treatment: narrow spectrum antibimicrobial effective against staph

Anti inflammatory treatment: mild doses of topical corticosteroids

*for epithelial defects, diagnostic cultures should be ascertained before steroid treatment

Hordeola and Chalazia

Hordeola- painful, tender, red nodular masses near the eyelid margin

External/ styes: glands of Zeis

Internal: meibomian gland inspissation

Self limiting (1-2 weeks)

S. Aureus

Hordeola

Tx:

Warm compress with massage

Antibx ONLY if with infectious blepharoconjunctivitis

Systemic: eyelid cellulitis

Oral doxycycline: chronic meibomitis

Hordeola and Chalazia

Chalazion- evolved internal hordeola

Chronic lipogranulomatous nodules (MG/ Zeis)

Weeks to months

Blurring of vision

Malignancy must be ruled out!

Tx:

Intralesional injection of corticosteroid (0.1-0.2 triamcinolone 40mg/ml)

Small, eyelid, multiple

Dark skin: depignmentation

Incision and drainage

Drainage and curettage

Internal chalazia : vertical incisions

Recurrent: biopsy

STRUCTURAL AND EXOGENOUS

DISORDERS ASSOCIATED WITH OCULAR
SURFACE DISORDERS

Exposure Keratopathy

Secondary to

lagophthalmos
proptosis

Punctate epithelial keratopathy (inferior)

Coalesce: ulceration, melting, perforation

Signs and symptoms similar to dry eye + corneal anesthesia

Tx:

Non preserved artificial tears AM; ointment PM

Lid taping

Permanent/ Temporary Tarsorrhapy

Correction of eyelids for long standing eyelid malposition

Gold or platinum weights

Complications: infection, shifting, extrusion, astigmatism, ptosis, inflammatory response

Floppy Eyelid Syndrome

Usually seen in obese patients

Flimsy, lax upper tarsus that everts with minimal upward force

Repeated eversion of the eyelid during sleep

Signs:

Papillae

Mucus discharge

Corneal involvement: Punctate epitheliopathy (mild); vascularization

Keratoconus

Tx:

Metal shield

Lid taping

Tarsorrhapy

Superior Limbic Keratoconjunctivitis

Mechanical trauma from upper eyelid to the superior

bulbar and tarsal conjunctiva

Autoimmune thyroid disease associated

chronic, recurrent condition of ocular irritation and

redness

20- 70 years of age

Self limiting, recur over 1-10 years

Bilateral; 1 eye more severely affected than the other

Signs:

Fine papillary reaction on superior tarsal conjunctiva

Injection and thickening of superior bulbar conjunctiva

Hypertrophy of superior limbus

Fine punctate lesions and filamentary keratopathy (superior);

Superior Limbic Keratoconjunctivitis

Diagnostics

Histologic:

Hyperproliferation, acanthosis, loss of goblet cells

Keratinization

Nuclear pyknosis with snake nuclei

Increased epithelial cytoplasm-nucleus ratio

Thyroid panel

Tx:

Topical anti inflammatory

BCL

Superior punctal occlusion

Thermocauterization of superior bulbar conjunctiva

Autologous serum drops

Amnion/ conjunctival transplant

Recurrent Corneal Erosion

history of trauma (sudden, sharp, abrading injury)

pre existing epithelial basement membrane dystrophy

Recur (days to years) spontaneously, periodically

Poor adhesion of the epithelium

Abnormality in the epithelial basement membrane and filament

network

Upregulated gelatinase activity (MMP2- MMP9)

MGD

Recurrent Corneal Erosion

Sx:

Sudden onset of eye pain at night or upon first awakening

Redness, photophobia, tearing

Ocular discomfort out of proportion to the degree of observable pathology

Slitlamp exam: epithelial cyst

Post traumatic vs dystrophic erosion

Examine contralateral eye after maximal dilation

Identification of loosely adherent epithelium by applying pressure with surgical

sponge after instillation of topical anesth - primary

Recurrent Corneal Erosion

Tx: Acute phase

Eye lubricants non preserved

Antibiotic ointments

AM: Hypertonic saline solution (5%)

PM: ointment (6-12 months)- promotes proper epithelial attachment

Low dose oral doxycycline

Topical corticosteroids

Topical antibiotics (broad spectrum)

BCL: flat base and high oxygen transmissibility

Topical broad spectrum antibx 3-4x daily

Recurrent Corneal Erosion

Recalcitrant cases

Anterior stromal micropuncture: promotes basement membrane formation

Dystrophic, degenerative, severe secondary basement membrane dse

Epithelial debridement: procedure of choice

Anesthesia

Debridement of loose epithelium using surgical sponge/ surgical blade/ spatula

Application of ophthalmic diamond burr

Topical antibx, cycloplegics, BCL application until

Re epithelialization is complete

Excimer laser phototherapeutic keratectomy:

Recalcitrant recurrent erosions

Neurotrophic Keratopathy

Caused by:

damage of CN V

Leading to corneal anesthesia/ hypoesthesia

Herpetic keratitis

Drugs

Diabetic neuropathy

Central or paracentral areas of non healing epithelium

Elevated, round/ oval, grayish edges with underlying stroma inflammation

Inferior, inferonasal

Bells phenomenon

Vascularization, corneal opacification, scarring

Perforation

Topical anesthetics
Topical NSAIDS
Trifluridine
B- blockers
CAI inhibitors
Drops with BAK

Causes of Neurotrophic Keratopathy and Persistent

Epithelial Defects
Damage to CN V
CVA
Aneurysms
MS
Tumors
Herpes simplex keratitis/ herpes zoster keratitis
Leprosy
Toxicity to topical medications (topical NSAIDs, b
blockers, CAIs trifluridine)
DM
Familial dysautonomia (Riley Day syndrome)

Neurotrophic Keratopathy

Tx:

Discontinuation of offending agent

Eye lubricants preservative free

Autologous serum drops

temporary or permanent punctal occlusion (dry eyes)

Patching: contact lenses (low water content, highly oxygen permeable)

Tarsorrhapy (lateral, medial)

Systemic tetracyclines- has specific activity agains MMPs

Crosslinking

Amnion grafting

Conjunctival flaps (last resort)

Trichiasis and Distichiasis

Trichiasis:

eyelashes from normal anterior origin curve inward toward the

cornea

Secondary to cicatricial entropion, other inflammatory conditions

Idiopathic

Distichiasis

Extra row of eyelashes emerges from MG ducts

Congenital (AD) or acquired

Trachoma

Mucous membrane pemphigoid

SJS

Chronic blepharitis

Chemical burns

Trichiasis and Distichiasis

Tx

Epilation

Electrolysis

Cryotherapy: eyelid margin thinning, loss of adjacent normal lashes, persistent

lanugo

Should be limited to -20 C for less than 30 secs

Tarsotomy with eyelid margin rotation

Factitious Ocular Surface Disorders

Factitious

Self induced injuries, intentional, assume a sick role

Patients are usually have knowledge of medicine or work in a medical setting

Factitious conjunctivitis: mechanical injury to the inferior and nasal quadrants of

the cornea and conjunctiva

Factitious Ocular Surface Disorders

Mucus fishing syndrome

Well circumscribed pattern of rose Bengal/ lissamine green (nasal/ inferior bulbar conj)

Increased mucus production

KCS inciting event

Patients demonstrate vigorous eye rubbing and compulsive removal of mucus strands from
fornix (mucus fishing)

Factitious Ocular Surface Disorders

Topical anesthetic abuse

Anesthetics: inhibit epithelial migration and division

Failure of corneal abrasions or infectious keratitis to respond to appropriate therapy

Defects look similar to a neurotrophic appearance

Mimic infectious course: KPs, hypopyon

Stromal edema, stromal infiltrates, large ring opacity

Removal of anesthetics

Dellen

Dessication of the epithelium and sub epithelial tissues due to poor ocular
surface

Inability to wet areas near elevated ocular surface

Saucerlike depressions

Epithelium shows punctate irregularities overlying a thinned area of

dehydrated corneal stroma

Tx:

Eye lubricants

Pressure patching

Scleral dellen

Post op complication of pterygium removal with the use of MMC

Delayed conjunctival wound closure

LIMBAL STEM CELL DEFICIENCY

Limbal Stem Cell Deficiency

Limbal epithelial stem cells
reside in the basal layer of the
epithelium (Ep), which
undulates at the limbus.
Daughter transient amplifying
cells (TACs) divide and migrate
towards the central cornea
(arrowed) to replenish the
epithelium, which rests on
Bowman's layer (BL).

Limbal Stem Cell Deficiency

25- 33% of the limbus must be intact to ensure normal ocular resurfacing

Normal limbus: acts as a barrier against corneal vascularization as well as

invasion of conjunctival cells from the bulbar surface

Absence of the limbal stem cells

Ocular surface compromised

migration of conjunctival cells

Clinical presentation:

recurrent ulceration and decreased vision

Corneal neovascularization

Limbal Stem Cell Deficiency

Primary:

PAX6 gene mutations (aniridia)

ectrodactyly-ectodermal dysplasia clefting syndrome

Sclerocornea

keratitis-ichthyosis deafness syndrome

congenital erythrokeratodermia

Secondary:

chemical burns

Radiation

contact lens wear

ocular surgery

mucous membrane conjunctivitis (trachoma)

Pterygium

topical meds

neoplasm of the limbus

Limbal Stem Cell Deficiency

Tx: Mild cases

Discontinuation of inciting agent

Topical steroids

Sectoral: epithelium debridement

Severe cases

Scleral contact lens

Limbal transplant: contralateral eye, allograft

Systemic immune suppression

SYSTEMIC CONDITIONS ASSOCIATED

WITH OCULAR SURFACE DISORDERS

Sjogren Syndrome

Associated with hypergammaglobulinemia, RA, circulating antibodies

T cell mediated inflammatory disease leading to destruction of the lacrimal

glands

Salivary glands and other mucous membranes involvement

Primary

Lacks any systemic immune dysfunction/ connective tissue disease

Secondary

Well defined generalized connective tissue disease- RA

Ichthyosis

Hereditary skin disorders characterized by excessive

dryness of skin and accumulation of scales

Diagnosed at 1st year of life

Ichthyosis vulgaris- most common hereditary scaling

disorder, AD

1: 250-300

Ocular involvement varies with form of ichthyosis

SSx: eyelid scaling, cicatricial ectropion, conjunctival

thickening

Primary corneal opacities: 50% with X-linked ichythyosis

Tx:

hydration of the skin and lids

Removal of scales

Ectodermal Dysplasia

Presence of abnormalities at birth

Non progressive

Diffuse involvement of the epidermis + 1 appendage (hair, nails, teeth, sweat glands)

Various inheritance patterns

Ocular abnormalities:

Sparse lashes, brows

Blepahritis

Ankyloblepharon

Hypoplastic lacrimal ducts

Corneal scarring

Ocular surface disease secondary to limbal deficiency

Xeroderma Pigmentosum

Impaired ability to repair sunlight induced damage to DNA

Focal hyperpigmentation, atrophy, actinic keratosis, telangiectasia

Skin neoplasm

Ophthalmic and Eyelid

Photophobia, tearing,
blepharospasm
Conjunctiva: dry,
hyperpigmented
Ocular neoplasm- 11%
Pterygium and pinguecula
Corneal complications

Atrophy
Madarosis
Trichiasis
Scarring
Symblepharon
Entropion/ ectropion
Loss of lids

Vitamin A Deficiency

Xerosis

Systemic: loss of mucus production in epithelial cells of GI, GU and respiratory tracts

Dryness of the conjunctiva and cornea

Loss of mucus production of goblet cells

Bitot spot: gray, triangular area on the bulbar conjunctiva

Keratinized epithelium, inflamm cells, debris and Corynebacterium xerosis

Prolonged: corneal ulcers and scars diffuse corneal necrosis (Keratomalacia)

3 stages

XIA conjunctival xerosis w/o Bitot spot

XIB conjunctival xerosis w/ Bitot Spot

X2 Corneal xerosis

X3 Corneal ulceration with keratomalaacia

X3A less than 1/3 corneal surface

X3B more than 1/3

Vitamin A Deficiency

Xerophthlamia: 20000-100000 new cases of blindness annualy

Highest risk:

malnourished infants born to vitamin A deificient mothers

Have had measles, diarrhea

Superficial concurrent infections: herpes simplex, measles, bacterial infections

Vitamin A deficiency : dietary practices, lifestyle related, malabsorption, surgery

Keratomalacia: systemic vitamin A deficiency

Px present with PEM

Tx:

Vitamin and protein calories supplements

Eye lubricants, topical antibiotics

THANK YOU!