Atopic Keratoconjunctivitis

Austin Strohbehn, M3; Matthew Ward, MD; Anna Kitzmann, MD

March 8, 2013
Chief Complaint: Poor vision and severe ocular itching.
History of Present Illness: A 52-year-old Caucasian male presents to the cornea clinic with a
chief complaint of vision loss. In addition to his vision loss, he complains of itchy eyes and
mucoid discharge. He also reports a history of waxing and waning eczema over the past decade.
Two years prior to this visit, he presented with similar symptoms and was treated with
medroxyprogesterone and fluoromethalone (FML) three times a day in both eyes. This medical
regimen improved his symptoms. Unfortunately, the patient discontinued his ocular prescription
and was lost to follow-up after the initial visit.
Past Ocular History: Unremarkable for surgery or trauma.
Medical History: History of uncontrolled eczema for the past decade.
Medications: He uses artificial tears as needed. Otherwise, he takes no medications or other eye
drops.
Family History: Father with cataract. No other pertinent family history.
Social History: The patient is a habitual smoker (1 pack per day).

Ocular Examination:

Visual Acuity, with correction:

o Right eye (OD): 20/60-1
o Left eye (OS): 20/70-1

Extraocular motility: Full, both eyes (OU)

Pupils: Briskly constrict from 5mm in dark to 3mm in light. No relative afferent
pupillary defect.

Confrontation Visual Fields: Full OU.

Intraocular pressure: OD 13 mmHg; OS 12 mmHg

Slit lamp examination:

o Lids: Hypertrophy, hyperpigmentation, and erythema of the lid skin. Cicatricial
ectropion of the lower lid. Near entire lower lid madarosis with rolled scarred lid
margin (figures 1C and D).
o Conjunctiva/sclera: Fornix foreshortening and symblepharon of the conjunctiva
with 2+ diffuse injection, more prominently OD (figure 1E).
o Cornea: Near complete conjunctivalization of the cornea with haze entering the
visual axis inferonasally (figures 1C and D).
o Anterior chamber: Deep and quiet.
o Iris: Normal
o Lens: 2+ nuclear sclerosis and 2+ posterior subcapsular cataract OU (figure 1F).

Dilated Fundus Exam:

o Hazy view to posterior pole due to conjunctivalization and posterior subcapsular
cataract, but appears normal OU.

Figure 1. Clinical signs of atopic keratoconjunctivitis.

A and B: Right and left eyes, respectively, in 2010 with periocular eczema and corneal haze. C
and D: Right and left eyes, respectively, in 2012 with progression of the disease; dense pannus
entering visual axis. E: symblepharon. F: posterior subcapsular cataract, which can be associated
with atopic keratoconjunctivitis.

Course
The symptoms of itching and findings of chronic conjunctivitis, eczema, symblepharon, pannus,
and posterior subcapsular cataract are consistent with a diagnosis of atopic keratoconjunctivitis.
At the patients initial visit two years prior, his condition was moderately severe (figures 1A and
B). He was treated with low potency topical corticosteroids (FML and medroxyprogesterone)
which resulted in improvement in his symptoms. However, he discontinued these medications
sometime after he was lost to follow up. Unfortunately, his disease progressed at a rapid rate
over the next two years. His symptoms became severe enough that he decided to return for an
examination. On exam at this time, the conjunctiva had entered the visual axis, and his visual
acuity had significantly decreased (figures 1C and D).

Discussion
Atopic keratoconjunctivitis (AKC) is a chronic inflammatory disease of the eye that occurs
predominately between the late teenage years and the fifth decade of life.[1] It is a chronic,
bilateral disease that relapses and remits with little to no seasonal correlation (as opposed to
vernal keratoconjunctivitis). Atopic dermatitis is present in 95% of cases and asthma in 87% of
cases.[2] Incidence of AKC increases with a family history of atopic disease.[1] The primary
symptom is ocular itching. Mucoid discharge and tearing are other common symptoms.
Periocular eczema is almost always present. Lid manifestations include hypertrophy, crusting,
cicatrization, ectropion, and madarosis. Conjunctivitis of the palpebral, bulbar, and limbal

conjunctiva is present in varying degrees. Palpebral conjunctivitis is characterized by

micropapillae of the tarsal conjunctiva primarily of the lower eyelid. Bulbar conjunctivitis
includes hypertrophy of conjunctiva with excess mucous. Symblepharon, adhesion of the
palpebral conjunctiva to the bulbar conjunctiva, occurs in approximately 20% of cases.[3]
Corneal neovascularization related to stem cell deficiency occurs in approximately 60% of cases.
[3] Corneal ulcers and erosions are more common due to the poor epithelial adhesion and
predispose to infectious keratitis, which can permanently scar the corneal stroma and/or lead to
corneal perforation. Posterior subcapsular cataracts are also common in these patients.
The pathogenesis of atopic keratoconjunctivitis has not been fully elucidated. In simple terms,
the disease originates from a disorder of the adaptive immune system in predisposed individuals.
An admixture of mast cells, Th1 T-cells, Th2 T-cells, and eosinophils is present on biopsy of the
conjunctival epithelium.[1] This helps aid in the diagnosis. However, the diagnosis of AKC is
made clinically, based on both a history of atopy and the ocular examination.

Treatment
Management of atopic keratoconjunctivitis is aimed at decreasing the inflammatory response and
controlling symptoms. The majority of cases are co-managed with an allergist or dermatologist
to provide guidance and follow up of systemic therapy. In early disease, conservative
management with combination topical mast cell stabilizers and anti-histamine drops, lubricants,
cold compresses, and oral anti-histamines may keep the ocular disease under control. However,
in more advanced cases, additional pharmacologic therapy is necessary. Pharmacologic therapy
for these cases includes topical corticosteroid drops, cyclosporine drops, or tacrolimus ointment
to the eyelid skin.[2,4] When topical treatments fail to induce remission, oral prednisone,
tacrolimus, or cyclosporine are the next treatment options. These are also useful for
dermatologic manifestations of the disease.[5] It is important to note that as T-cells play a central
role in the pathogenesis of this disease, calcineurin inhibitors such as tacrolimus and
cyclosporine are an effective route for steroid-sparing therapy in both topical and systemic
applications. In the final stages of atopic keratoconjunctivitis where visual loss has occurred due
to corneal opacification from stem cell deficiency, the Boston keratoprosthesis may be used for
visual rehabilitation in motivated patients.

Diagnosis: Atopic Keratoconjunctivitis

Epidemiology

Signs

<1-8% of the adult population[1]

Chronic, bilateral conjunctivitis

Males > Females

Always associated with another atopic diseas

independent of its severity

Predominately presents between the late teenage

years and the fifth decade of life[1]

o Atopic dermatitis

Spontaneous resolution in elderly

o Asthma

Associated with atopic dermatitis in 95% of

cases[2]

o Rhinitis

Associated with asthma in 87% of cases[2]

Periocular

Eczema

Lids

Blepharitis

Hypertrophy

Crusting

Cicatrization

Ectropion

Madarosis

Conjunctiva

Hypertrophy of entire conjunctiva

Palpebral conjunctiva micropapillae

Hyperemia

Excessive mucous

Symblepharon in severe disease

Cornea

Erosions

Pannus

Anterior stromal scarring

Corneal thinning

Neovascularization

Perforation

Shield ulcers

Lens

Symptoms

Ocular itching

Mucoid discharge

Photophobia

Decreased visual acuity

Tearing

Anterior and/or posterior subcapsular catarac

Treatment

Therapy is aimed at treating both the ocular and the s

disease.
Early disease treatment

Cold compresses

Preservative free drops

Mast cell stabilizer/antihistamine (i.e. olopata

or lodoxamide 0.1%)

Antihistamine: (e.g. azelastine 0.05%) for rel

Advanced disease treatment

Topical cyclosporine A 0.05% eye drops

Topical tacrolimus 0.03% ointment to the eye

Topical corticosteroid

Consultation with allergist and/or dermatolog

Oral cyclosporine, tacrolimus, or corticostero

Boston keratoprosthesis (if visual loss from c

opacification has occurred)

Differential Diagnoses:

Vernal keratoconjunctivitis

Seasonal allergic conjunctivitis

Perennial allergic conjunctivitis

Giant papillary conjunctivitis

Phlyctenular keratoconjunctivitis

Toxic conjunctivitis

References
1. Bielory B and Bielory L. Atopic dermatitis and keratoconjunctivitis. Immunology and
Allergy Clinics of North America 2010; 30(3): 323-336.
2. Guglielmetti S, Dart JKG and Calder V. Atopic keratoconjunctivitis and atopic dermatitis.
Current Opinion in Allergy and Clinical Immunology 2010; 10: 478-485.
3. Power WJ, Tugal-Tutkun I, and Foster CS. Long-term follow-up of patients with atopic
keratoconjunctivitis. Ophthalmology 1998; 105(4): 637-642.
4. Tzu JH, Utine CA, Stern ME, and Akpek EK. Topical calcineurin inhibitors in the
treatment of steroid-dependent atopic keratoconjunctivitis. Cornea 2012; 31(6): 649-654.
5. Cornish KS, Gregory ME, and Ramaesh K. Systemic cyclosporine A in severe atopic
keratoconjunctivitis. European Journal of Ophthalmology 2010; 20(5): 844-851.

Suggested Citation Format: Strobehn A, Ward M, Kitzmann A. Atopic Keratoconjunctivitis.

EyeRounds.org. March 8, 2013; available from: http://www.EyeRounds.org/cases/167-atopickeratoconjunctivitis.htm

Atopik Keratoconjunctivitis
Diulas oleh Jill E. Bixler, MD ,mei 2008
www.kellogg.umich.edu/.../atopic-keratoconjunct.

Pada halaman ini:

Apa atopik Keratoconjunctivitis?

Gejala

Penyebab

Faktor Risiko

Tes dan Diagnosis

Pengobatan dan Obat

Klinik Informasi

Apa atopik Keratoconjunctivitis?

Atopic keratoconjunctivitis (AKC) adalah hasil dari suatu kondisi yang disebut "atopi". Atopi
adalah kondisi genetik di mana sistem kekebalan tubuh menghasilkan antibodi lebih tinggi dari
biasanya dalam menanggapi alergen tertentu. Meskipun AKC adalah abadi (sepanjang tahun)
penyakit, gejala Anda mungkin memburuk di musim dingin. Tidak seperti dermatitis atopik,
yang umumnya terlihat di awal masa kanak-kanak, keratokonjungtivitis atopik muncul selama
masa remaja akhir dan dewasa awal. Pria lebih sering terkena daripada wanita.
Dengan AKC, konjungtiva lapisan kelopak mata biasanya merah dan bengkak. Semakin rendah
kelopak mata umumnya mempengaruhi lebih dari kelopak mata atas. Ini adalah gejala
membedakan dari keratokonjungtivitis vernal mana kelopak mata atas yang paling sering
terkena. Jika tidak diobati, AKC dapat berkembang menjadi ulserasi, jaringan parut, katarak,
keratoconus, dan vaskularisasi kornea.

Gejala

Sensitivitas terhadap cahaya

Gatal

Membakar

Merobek

Kelopak mata merah dan mengeras

Gejala-gejala yang dijelaskan di atas mungkin tidak berarti bahwa Anda memiliki
keratokonjungtivitis atopik. Namun, jika Anda mengalami satu atau lebih gejala-gejala tersebut,
hubungi dokter mata Anda untuk ujian lengkap.

Penyebab
AKC adalah kondisi genetik.

Faktor Risiko

Riwayat keluarga beberapa alergi

Dermatitis atopik

Eksim

Asma

Tes dan Diagnosis

AKC biasanya didiagnosis dengan pemeriksaan klinis dan riwayat medis dan keluarga, meskipun
biopsi konjungtiva dapat membantu dalam membedakan AKC dari kondisi lain.

Pengobatan dan Obat

Kombinasi antihistamin oral dan topikal dan stabilisator sel mast biasanya efektif dalam
mengendalikan gejala. Dalam kasus yang lebih parah, ada potensi untuk kerusakan mata yang
disebabkan oleh goresan dan menggosok. Seorang dokter spesialis mata mungkin menyarankan
Anda untuk memakai sarung tangan katun pada malam hari untuk mencegah kerusakan yang
tidak disengaja pada permukaan okular. Kompres dingin dan irigasi saline untuk menurunkan pH
air mata tinggi juga dapat membantu. Hanya dalam kasus yang parah harus topikal terapi steroid
dipertimbangkan. Agen steroid-sparing lain dapat membantu, termasuk siklosporin atau
tacrolimus. Pengobatan sistemik dari kondisi yang menyertai AKC mungkin akan sangat
membantu.

http://www.uptodate.com/contents/atopic-keratoconjunctivitis

PENDAHULUAN
Ada lima jenis utama dari alergi okular: konjungtivitis alergi musiman (SAC), abadi
konjungtivitis alergi (PAC), keratokonjungtivitis vernal (VKC), keratokonjungtivitis atopik
(AKC), dan konjungtivitis papiler raksasa (GPC). VKC dan AKC adalah bentuk kronis, bilateral,
dan parah peradangan alergi mempengaruhi permukaan mata. Kedua jenis relatif jarang dari
penyakit mata alergi dapat menyebabkan kerusakan parah pada permukaan mata, yang
menyebabkan jaringan parut kornea dan kehilangan penglihatan jika tidak ditangani dengan baik.

Jenis reaksi hipersensitivitas I penting dalam penyakit ini, meskipun mereka bukan satu-satunya
mekanisme patofisiologis.
AKC Istilah ini tidak ideal, karena tidak spesifik dan tidak dengan sendirinya membedakan AKC
dari bentuk-bentuk lain dari penyakit mata atopik. AKC pada umumnya mengacu pada "abadi"
bentuk konjungtivitis alergi (AC) yang juga memiliki potensi keterlibatan kornea yang
signifikan, dimana VKC adalah "musiman" bentuk (setidaknya pada awalnya) dari AC yang
dapat mempengaruhi kornea. AKC ditinjau dalam topik ini. VKC dibahas secara rinci secara
terpisah. (Lihat "Vernal keratoconjunctivitis" .)
GPC adalah gangguan inflamasi yang merupakan reaksi terhadap gerakan tutup lebih zat asing,
seperti lensa kontak. Konjungtivitis Toxic tidak alergi di alam, tetapi sering bingung dengan
penyakit mata alergi. Ini mengembangkan dengan penggunaan berkepanjangan dari obat topikal,
sebagian besar karena pengawet. GPC dan konjungtivitis toksik dibahas secara rinci secara
terpisah. (Lihat "Raksasa papiler conjunctivitis" dan "konjungtivitis Toxic" .)
Musiman dan abadi konjungtivitis alergi, bentuk yang paling umum dari alergi okular, juga
dibahas secara terpisah. (Lihat "alergi konjungtivitis: Manifestasi klinis dan diagnosis" .)
EPIDEMIOLOGI
AKC adalah penyakit mata alergi kronis yang paling sering terjadi pada pasien dengan riwayat
dermatitis atopik [ 1,2 ]. Yang tepat prevalensi tidak diketahui. Namun, 4,4 persen dari 1.079
pasien dengan alergi okular memiliki AKC dalam satu studi [ 3 ]. Studi lain menunjukkan bahwa
77 persen dari pasien dengan dermatitis atopik (AD) menderita AKC [ 4 ], meskipun seri
berikutnya mencatat tingkat yang lebih rendah dari AKC pada pasien dengan AD, di kisaran 20
sampai 40 persen [ 5 ].

http://www.aao.org/publications/eyenet/201002/pearls.cfm?RenderForPrint=1&

Ophthalmic Pearls: External Disease

The Itchy Eye: Diagnosis and Management of Ocular Pruritus
By Jocelyn Kuryan, MD, Prabjot Channa, MD, and Roy S. Chuck, MD, PHD

Edited by Ingrid U. Scott, MD, MPH, and Sharon Fekrat, MD

(PDF 169 KB)

Ocular pruritus is a common symptom that brings patients to the ophthalmologists office. It may be
tempting to overlook this seemingly minor problem while assessing patients for potentially visionthreatening diseases. However, itchy eyes can be a major problem and source of anxiety for patients,
often affecting their day-to- day quality of life.
Although a physicians inclination may be simply to treat the symptom with topical mast-cell stabilizers
or antihistamines, it is important to identify the underlying cause. By carefully and methodically
determining the etiology of ocular pruritus, the clinician can select an appropriate treatment regimen and
provide patients with the relief they seek.

What Causes Itchy Eyes?

Pruritus can be the chief complaint for a number of ocular surface diseases. Atopic keratoconjunctivitis,
vernal keratoconjunctivitis, allergic conjunctivitis and atopic dermatitis are part of the spectrum of
ocular allergies. Other causes of pruritus include dry eye syndrome, meibomian gland dysfunction,
blepharitis, contact lensinduced conjunctivitis, giant papillary conjunctivitis and contact
dermatoblepharitis. A complete history, review of systems and examination can help differentiate among
these etiologies.

Pathophysiology
Many of the causes of ocular pruritus are immunologically mediated.

Allergic conjunctivitis and AKC are a result of IgE-mediated reactions that cause mast-cell
degranulation and histamine release. IgE-mediated processes as well as impaired cellular
immunity and genetic factors also contribute to atopic dermatitis. Both type I (immediate) and
type IV (delayed) hypersensitivity reactions are involved in vernal keratoconjunctivitis.
Conjunctival scrapings from patients with allergic conjunctivitis, AKC and vernal
keratoconjunctivitis will reveal eosinophils that are not part of the normal conjunctival histology.

Contact dermatoblepharitis is the result of a type IV T cellmediated hypersensitivity reaction,

which is why symptoms can take one to three days to manifest.

Blepharitis is also believed to have an immunologic basis, as symptoms may result from a

reaction to staphylococcal antigens.

The etiology of conjunctivitis induced by contact lenses is multifactorial, including mechanical

trauma, dry eye syndrome and hypersensitivity to contact lenses and/or solution as well as to
protein deposits on the lenses.

The pathogenesis of MGD involves the action of local inflammatory mediators in the setting of
hyposecretion and obstruction of the meibomian gland orifices.

DES can be caused by either aqueous tear deficiency (either Sjgren or non-Sjgren type) or
evaporative tear dysfunction (which includes MGD). Patients suffering from Sjgren syndrome
also have autoimmune dysregulation.

Acronyms
AKC

Atopic keratoconjunctivitis

DES

Dry eye syndrome

GPC

Giant papillary conjunctivitis

MGD

Meibomian gland dysfunction

History
A thorough history and review of systems should include special attention to onset, duration and
frequency of symptoms, as well as exacerbating factors and associated systemic complaints. The
following questions may help narrow the differential:

Do your eyes itch throughout the year? Perennial complaints are often associated with allergic
conjunctivitis and AKC.

Do your eyes burn and/or tear? Do you have foreign body sensation? All may be symptoms
of dry eyes and/or MGD.

Do you have asthma, allergic rhinitis and/or eczema/skin rashes? A positive response may
suggest allergic conjunctivitis, AKC and atopic dermatitis more strongly; these conditions are

frequently linked.

Do you wear contact lenses? Contact lens overwear, poorly fitting lenses and/or improper
hygiene can lead to contact lensinduced conjunctivitis or GPC. Soft contact lenses tend to
induce more pruritus than rigid gas permeable lenses.

Can you identify any triggers? Exposure to pet dander or environmental allergens can often
trigger allergic conjunctivitis. Patients with vernal keratoconjunctivitis tend to be more
symptomatic in warmer climates.

Have you used any new products, such as creams, makeup, soap or eyedrops? Itchiness
caused by exposure to a sensitizing agent is often seen with contact dermatoblepharitis or
blepharoconjunctivitis. Symptoms tend to develop within one to three days of exposure.

Examination
A thorough examination will help reveal the underlying pathology.
Eyelids. Pay particular attention to the eyelids for:

Eyelid margin erythema and edema, which can be seen with most of the conditions described.

Scaling of the eyelids, ectropion and leathery thickness of the eyelid (lichenification) are
associated with atopic dermatitis and chronic contact dermatoblepharitis.

Periorbital hyperpigmentation (allergic shiners) may point to an allergic cause.

Blepharitis can result in crusting of the eyelid margin and collarettes at the base of the eyelashes.

MGD can cause inspissation of the meibomian gland orifices, foamy tears, eyelid margin
telangiectasias, puckering and recurrent chalazia.

Papillary reactions of the palpebral conjunctiva are seen with many of the aforementioned
conditions, but the severity and location tend to vary.

Patients with vernal keratoconjunctivitis may have giant papillae or diffuse papillary
hypertrophy of the upper palpebral conjunctiva. Small papillae tend to be located on both the
upper and lower palpebral conjunctiva in AKC, while papillae are more prominent on the upper
palpebral conjunctiva in contact lensinduced conjunctivitis.

Conjunctiva. Conjunctival signs include:

Mild to moderate bulbar conjunctival hyperemia and/or chemosis, which is seen in most cases of
ocular pruritis.

Varying degrees of mucoid discharge may also be seen.

Cornea. Check the cornea for:

Punctate epithelial erosions, which are a common finding in the patient with itchy eyes. Pannus
and corneal vascularization may be present and vary according to disease severity.

Marginal epithelial infiltrates can be present in MGD and contact lensinduced conjunctivitis.

Horner-Trantas dots (raised, gelatinous collections at the limbus) and shield ulcers are classically
associated with vernal keratoconjunctivitis.

Management and Treatment

Once the diagnosis has been made, an appropriate treatment course can be selected (Common Causes
and Treatments for Ocular Pruritis, next page).
Contact dermatoblepharitis/blepharoconjunctivitis. First, it is imperative to identify and discontinue
use of the offending agent. Contact reactions to topical carbonic anhydrase inhibitors and brimonidine
are commonly delayed several weeks or months. Once the irritant is eliminated, supportive treatment,
including the use of cool compresses, is usually sufficient. Ocular lubrication with artificial tears or
ointment is also helpful. Additional therapies include the use of mast-cell stabilizers, topical
antihistamines and topical nonsteroidal anti-inflammatory drugs (e.g., ketorolac). Topical corticosteroids
applied to the eyelid can hasten recovery in more severe cases.
Atopic dermatitis. Eliminate environmental and food allergens. Exacerbations on the skin can be
treated with corticosteroid cream or immunomodulators (e.g., tacrolimus) in severe cases. Moisturizing

the facial skin (specifically the eyelid) is important for long-term treatment. Systemic antihistamines and
mast-cell stabilizers may also provide relief.
Dry eye syndrome. Lubrication of the ocular surface is the ultimate goal. Initial treatment with artificial
tears (if the tears are used more often than four times daily, then a preservative-free formulation is
necessary) and with lubricant at bedtime, is acceptable. Some patients may also require punctal plugs.
Those who remain symptomatic may need topical cyclosporine A to increase tear production.
Allergic conjunctivitis. Once again, it is important to avoid or eliminate allergic triggers whenever
possible. Supportive care with cool compresses can be helpful for some patients. The use of physical
barriers (such as glasses) is also useful in limiting allergen contact. Artificial tears will help dilute any
allergen remaining on the ocular surface. Topical vasoconstrictors (e.g., pheniramine, naphazoline,
oxymetazoline) can be used on a short-term basis for symptomatic relief. For patients with more severe
symptoms, topical (e.g., olopatadine, ketotifen), oral and intranasal antihistamines and mast-cell
stabilizers (e.g., cromolyn sodium, lodoxamide) are often beneficial. Topical NSAIDs and
corticosteroids should be used with caution and require frequent follow-up. Consultation with an
allergist for desensitization therapy may be necessary for those patients who remain symptomatic
despite these measures.
Vernal keratoconjunctivitis/atopic keratoconjunctivitis. Symptoms may be alleviated with topical
antihistamines and mast-cell stabilizers. However, these patients tend to require more aggressive
measures compared with those suffering from allergic conjunctivitis. Topical corticosteroids and even
immunomodulators (such as cyclosporine A) may be necessary. Supratarsal corticosteroid injections
have also been used to control symptoms. Patients with AKC are more prone to infectious complications
(especially herpes simplex keratitis) and should, therefore, be monitored closely. Vernal
keratoconjunctivitis classically affects young men in warm climates; these patients may find relief in
cooler climates or air-conditioned environments. Shield ulcers may require plaque debridement and
scraping at the ulcer base followed by aggressive treatment with topical corticosteroids and antibiotics.
Contact lens induced conjunctivitis/ giant papillary conjunctivitis. Patients should first be advised
to discontinue contact lens wear until the exacerbation has resolved. It is also appropriate to refit the
lenses or to try different lenses and to advise patients about proper hygiene. It may be helpful to change
to daily-wear contact lenses. Once the exacerbation hasresolved, mast-cell stabilizers are sometimes
used as maintenance therapy. For those who remain intolerant, refractive surgery is an alternative.
Meibomian gland dysfunction/blepharitis. Education regarding proper eyelid hygiene is imperative.
Warm compresses and twice-daily eyelid scrubs can help open inspissated meibomian glands. A clean
washcloth dipped into baby shampoo diluted with water is commonly used for eyelid massage and
scrubbing. Ocular surface lubrication with artificial tears can provide additional relief. Short-term use of
a topical antibiotic (macrolides are often used) may be beneficial, while some patients will require a
course of oral tetracyclines that are then tapered off. Staphylococcal marginal keratitis will often require
the use of topical corticosteroids to quell the inflammatory response.

Common Causes and Treatments of Ocular Pruritus

Contact dermatoblepharitis/
blepharoconjunctivitis

Remove offending agent

Cool compresses, artificial tears
Mast-cell stabilizers and topical antihistamines
Topical NSAIDs (e.g., ketorolac) and corticosteroids as
needed

Atopic dermatitis

Remove offending agent

Topical corticosteroid cream or immunomodulators
for skin symptoms (e.g., tacrolimus)
Lubrication of eyelids
Systemic antihistamines and mast-cell stabilizers

Dry eye syndrome

Artificial tears and lubricating ointments

Punctal plugs
Topical immunomodulators (e.g., cyclosporine A)

Allergic conjunctivitis
(seasonal and perennial)

Remove offending agent

Cool compresses
Eyeglasses to shield from allergens
Artificial tears
Topical vasoconstrictors (e.g., pheniramine,
naphazoline, oxymetazoline) for short-term
symptomatic relief
Topical (e.g., olopatadine, ketotifen) as well as oral or
intranasal antihistamines, and mast-cell stabilizers(e.g.,
cromolyn sodium, lodoxamide)
Topical NSAIDS and corticosteroids
Consultation with an allergist for desensitization
therapy

Vernal keratoconjunctivitis

Topical antihistamines and mast-cell stabilizers

Topical corticosteroids and immunomodulators
(e.g., cyclosporine A)
Supratarsal corticosteroid injection

Atopic keratoconjunctivitis

Similar treatment as for vernal keratoconjunctivitis

Contact lensinduced
conjunctivitis/giant papillary
conjunctivitis

Discontinue contact lens use

Refit contact lenses
Proper contact lens hygiene
Refit contact lenses
Mast-cell stabilizers for maintenance
May consider refractive surgery in refractory cases

Meibomian gland
dysfunction/blepharitis

Eyelid hygiene
Artificial tears

Topical antibiotics (e.g., macrolides)

Course of oral tetracyclines followed by taper
Short course of topical corticosteroids

https://www.inkling.com/read/cornea-krachmer-mannis-holland-3rd/chapter-49/atopickeratoconjunctivitis

Atopic Keratoconjunctivitis
Definition
Atopic keratoconjunctivitis (AKC) is a bilateral, chronic inflammation of the conjunctiva and
lids associated with atopic dermatitis. Hogan, in 1953, was the first to describe the findings of
chronic conjunctivitis and keratitis in patients with atopic dermatitis.66 Three percent of the
population has atopic dermatitis.67,68 From 15% to 67.5% of patients with atopic dermatitis have
ocular involvement, usually AKC.6769

Demographics
The onset of disease is usually in the second through fifth decade although the majority of
patients with atopic dermatitis are diagnosed by age 5 years. Recent series of patients report the

onset of symptoms between the ages of 7 and 76.7072 The male:female ratio is reported as 2.4:1
and less than 1.70,72 No racial or geographic predilection is reported.

Symptoms
Itching is the major symptom of AKC. This may be more pronounced in certain seasons or it
may be perennial. Other symptoms, in decreasing order of frequency, include watering, mucus
discharge, redness, blurring of vision, photophobia, and pain.72 Exacerbation of symptoms most
frequently occurs in the presence of animals.72

Signs
Signs of AKC include skin, lid margin, conjunctival, corneal, and lens changes (Table 49.2). The
periocular skin often shows a scaling, flaking dermatitis with a reddened base (Fig. 49.3). The
lids may become lichenified and woody, developing cicatricial ectropion and lagophthalmos.
Lateral canthal ulceration, cracking, and madarosis may also be present. This may be the
principal manifestation in a minority of cases. The lid margins may show loss of cilia,
meibomianitis, keratinization, and punctal ectropion. The conjunctiva of the tarsal surfaces has a
papillary reaction, follicles, and possibly a pale white edema (Fig. 49.4). In contrast to VKC, the
papillary hypertrophy of AKC is more prominent in the inferior conjunctival fornix.
Subepithelial fibrosis is present in many, fornix foreshortening in some, and symblepharon in a
few. The bulbar conjunctiva may have few findings besides erythema and chemosis. A
perilimbal, gelatinous hyperplasia may occur (Fig. 49.5). Horner-Trantas dots have been reported
to occur in AKC.73
Table49.2 Clinical signs in patients with AKC
Foster and Calonge70 (N = 45)
Condition
No. of patients
%
Lids
Eczema
28
62.2
Blepharitis
25
55.6
Meibomianitis
25
55.6
Tarsal margin keratinization
13
28.9
Trichiasis
8
17.8
Madarosis
6
13.3
Punctal ectropion

Ectropion
5
11.1
Entropion
2
4.4
Conjunctiva
Subepithelial fibrosis
26
57.8
Fornix foreshortening
13
28.9
Symblepharon
12
26.7
Giant papillae
11
24.4

Tuft et al72 (N = 37)

No. of patients
%
30
33

18

81.1
89.2

16.2

48.6

26

10
11

70.3

27.0
29.7

Condition

Foster and Calonge70 (N = 45)

No. of patients
%
6
13.3

Tuft et al72 (N = 37)

No. of patients
%
5
13.5

Follicles
Cornea
Superficial punctate keratitis
24
53.3
37
Neovascularization
17
37.8
24
Persistent epithelial defects
17
37.8
4
Filamentary keratitis
2
4.4
1
Fig. 49.3
Severe periocular and lid involvement of AKC.
Fig. 49.4
Lower tarsal conjunctiva in AKC. Note the fornix foreshortening and pale edema.
Fig. 49.5
Limbal gelatinous hyperplasia in AKC.

100
64.9
10.8
2.7

Significant vision loss in this disease usually results from pathologic conditions of the cornea.
Punctate epithelial keratopathy is the most common corneal finding. Persistent epithelial defects,
scarring, microbial ulceration, and neovascularization are the main corneal causes for decreased
vision (see Table 49.2). Penetrating keratoplasty typically results in similar surface problems but
has been shown to improve vision in some.74 Herpetic keratitis is reported to occur in 1417.8%
of patients.70,72 Keratoconus occurs in 6.716.2% of patients.70,72
Anterior uveitis and iris abnormalities are not reported. The prevalence of cataract associated
with AKC is difficult to determine, since steroids are so frequently used in the treatment of the
disease. The lens opacity typically associated with AKC, however, is an anterior or subcapsular
cataract. This cataract often has the configuration of a Maltese cross. Retinal detachment with or
without previous cataract surgery is the principal posterior manifestation of AKC reported.7577

Pathophysiology
Atopic keratoconjunctivitis is thought to consist of both type I and type IV hypersensitivity
mechanisms. Evidence of the pathologic process comes from histologic and
immunohistochemical analysis of conjunctival biopsy specimens and from tear fluid analysis for
mediators and cells.
Mast cells and eosinophils are found in the conjunctival epithelium of AKC patients but not in
normal individuals.78 Mast cells in the epithelium of AKC patients contain predominantly
tryptase as the neutral protease. Goblet cell density and squamous metaplasia are then examined
by impression cytology.79 The epithelium may become involuted, allowing pseudotubule
structures to form.80 Antibodies to HLA-DR stain diffusely throughout the epithelium.80 This
suggests an up-regulation of antigen presentation. There is an increase in the CD4:CD8 ratio in
AKC over normal conjunctival epithelium.80 This increase of CD4 or helper T cells (Th)
probably serves to amplify the immune response that is occurring. In vivo confocal microscopy
reveals fewer basal epithelial cells in the cornea.81 Mucin proteins and mRNA are increased in
the epithelium.82,83

The substantia propria in AKC has an increased number of mast cells compared to normal.
Conjunctival inflammatory cell density showed a negative correlation with tear stability and
corneal sensitivity and a positive correlation with the vital staining scores.84 Eosinophils, never
found in normal structures, are present in the substantia propria in AKC. These eosinophils are
found to have increased numbers of activation markers on their surface.85 A large number of
mononuclear cells is present in the substantia propria. Fibroblast number is increased, and there
is an increased amount of collagen compared to normal individuals. In addition, the substantia
propria demonstrates increased CD4/CD8, B cells, HLA-DR staining, and Langerhans cells.80
The T-cell receptor on lymphocytes in the substantia propria is predominantly of the or
subtype.80 The T-cell population of the substantia propria includes CD4 and memory cells.86,87
Th2 cytokines predominate in allergic disease yet lymphocytes with Th1 cytokines have been
found in the substantia propria of AKC patients.87
Tears of AKC patients contain increased levels of IgE, eosinophil cationic protein (reduced
following papillae resection), activated B cells, eotaxin, eosinophil neurotoxin, soluble IL-2
receptor, IL-4, IL-5, specific sIgA, and osteopontin.27,65,69,70,8894 AKC has reduced tear break-up
time and decreased Schirmers values (56% less than 5 mm) compared to controls.83,95 Serum of
AKC patients has been found to contain increased levels of IgE, eosinophil cationic protein,
eosinophil neurotoxin, and IL-2 receptor.69,9698 Tabbara et al. report adoptive transfer of AKC in
bone marrow recipients.99 Messmer and colleagues show eosinophils and their products
deposited in the ulcers and stroma of corneas from AKC patients.100
In summary, AKC patients demonstrate an increased number of conjunctival mast cells and
evidence of mast cell activation. Furthermore, a complex immune cell profile implicates more
than the mast cell alone, but the details of these cellular interactions remain speculative.

Diagnosis
Paramount to both diagnosis and treatment in AKC is a careful history. The patient typically
describes severe, persistent, periocular itching associated with dermatitis. There is usually a
family history of atopic disease in one or both parents and commonly other atopic manifestations
in the patient, such as asthma (65%) or allergic rhinitis (65%).71 A history of seasonal or
exposure-related exacerbations is usually present. History and examination reveal features to
help differentiate AKC from other atopic ocular conditions. The lack of contact lens wear aids in
differentiating AKC from GPC. AKC patients are usually older and have major lid involvement
compared to patients with VKC. SAC patients have no or markedly diminished symptoms out of
their season and show no evidence of chronic inflammation in the conjunctiva. The significant
past history or concurrent presence of eczema cannot be emphasized enough as a finding in
patients with AKC. The serum level of IgE is often elevated in patients with AKC. A Giemsa
stain of a scraping of the upper tarsal conjunctiva may reveal eosinophils.

Treatment
The approach to treatment is multifaceted and includes environmental controls as well as topical
and systemic medications. It is unlikely that the AKC patient will see the ophthalmologist

without also being under the care of a medical physician. However, the patient must remove
environmental irritants in both the home and the employment or school setting. The nature of the
irritants may be better defined through skin testing.
The topical application of a vasoconstrictorantihistamine combination may bring transient relief
of symptoms but is unlikely to intervene in the immunopathologic process or its sequelae. There
is potential for overuse due to the chronic nature of the disease. The potent topical antihistamines
offer much greater H1 receptor antagonism than over-the-counter antihistamines. The topical
administration of steroids such as prednisolone acetate eight times per day for 7 to 10 days is
clearly beneficial in controlling symptoms and signs. These agents, of course, must be used
judiciously, since the chronic nature of the disease may encourage overuse. The patient must be
instructed that steroid use must be transient only and must be carefully monitored for efficacy; he
or she must also be warned of the potential for causing cataract and glaucoma. Nonsteroid
medications have been shown to be effective in reducing itching, tearing, and photophobia.101103
Mast cell stabilizers two to four times daily is recommended year-round in patients with
perennial symptoms. If an exacerbation occurs and the patient is not taking a mast cell stabilizing
agent topically, its use should be initiated two to four times daily concurrent with a short burst of
topical steroids (for 710 days). Mast cell stabilizers alone such as cromolyn, nedocromil,
lodoxamide, or mast cell stabilizer combination antihistamines such as olopatadine, azelastine,
epinastine, and ketotifen may be helpful. Ciclosporin A and tacrolimus, both orally and topically,
have been shown effective in treating AKC as well as reducing the amount of topical steroid
use.55,104108 Foster and Calonge recommend maximizing the use of systemic antihistamines.70 H1
receptors seem most responsible for the symptoms of AKC, and newer antagonists are fairly
specific for the H1 receptor. Only in rare cases of uncontrolled dermatitis with vision-threatening
complications are oral steroids indicated. The role of systemic desensitization is similar to that in
VKC. Plasmapheresis has been shown effective in the treatment of AKC.109
Lid and ocular surface abnormalities may require treatment other than that directed toward the
underlying pathologic condition of AKC. Trichiasis or lid position abnormalities, if contributing
in any way to corneal compromise, must be corrected. Any staphylococcal blepharitis should
receive adequate antibiotic treatment. If, despite