Case Presentation of Recurrent Peripheral Infiltrative Keratitis

(PIK) of Unknown Cause

4/17/13 Northeastern State

University

60’s Caucasian male, red eye ODMedical/ocular hx:• No contact lenses, ocular surgeries or trauma• hx of left orbital pseudotumor (neg CT ‘03) • has mild dry eyes and seasonal allergies• no known autoimmune/collagen vascular dis. • hx of insect bite with rash• past blood work unremarkable • closely followed for > 10 yrs; + hypothyroidism• (NOTHING JUMPS OUT)

Some questions we want answered

• Is this red eye infectious?• Is this red eye autoimmune?• Is this red eye blepharitic?

Next slide: BEGINS THE EXAM

OCULAR EXAM FINDINGS INCLUDE:• Visual acuity and posterior

segment are unremarkable• Left eye never became involved

January 2013 Visits for Right eye

1st wk/visit 2nd week 3rd week 4th week

History Red/irritated/burn 1 wk

5/10 tenderness

light sensitive

Less red/irritated/burn

2/10 tenderness

Less light sensitive

Less red/irritated/burn

1-1.5/10 tenderness

Not very light sensitive

Less red/irritated/burn

0/10 tenderness

not light sensitive

Findings + infiltrates ,(+)tr.staining

2-3+ bulbar injection

3+ cells

+ infiltrates , (-) staining

1-2+ bulbar injection

1-1.5+ cells

Less dense infiltrates

mild bulbar injection

no cells

Less dense infiltrates

no injection, (+)pannus

A and P Peripheral Infiltr. Keratitis (PIK)Anterior Uveitis (AU)Pred Forte (PF)

PIK severe epi/mild scleritisAU

PF q1-2hrs/dayCyclo 3xVigamox 4x

PIKsevere epi/mild scleritisAU

PF 5x/dayCyclo 3xVigamox 3x

Resolving PIKmild episcleritisresolved AU

PF 4x/dayStop CycloStop Vigamox

Resolving PIKresolved episcleritis

PF 3x/day for 2wks, then 2x/day til return

RTC 2 days 5 days 1-2 weeks 3 weeks

February/March 2013 Visits for Right eye

Mid February

1st wk of March

3rd wk of March

4th week of March

History 0/10 tenderness

not light sensitive

no scalp/jaw pain

0/10 tenderness no complaintsPt did not stop taking PF

0/10 tenderness no complaintsStopped PF as directed

0/10 tenderness not light sensitive mild scalp pain

Findings IOP 33 (-) infiltrates (+) lipid deposits Gonio stable/open

IOP 32 no change

IOP normal 3 brightly staining infiltrates, no ulcer1+ cell

IOP normal 3 minimally staining infiltrates no cells

A and P Peripheral Infiltrative Keratitis (PIK)Anterior Uveitis (AU)Pred Forte (PF)

Resolved PIKOHTN vs steroid response (hx of 31 untreated pressure)Taper PF for 1 wk then stop PF

Same

Stop PF immediately

Recurring PIK AU

PF q2-3hrCyclo 2x

Recurring PIK, resolved AUPF q2-3hrCyclo 2xAdditional Labs in case of worsening/ reoccurrence

RTC 2 weeks 2 weeks 5days 2 weeks

BEGIN DIFFERENTIALS1. Infectious Keratitis

OCULAR, LOCAL, MOST SEVERE DISEASE

2. Systemic causes (includes infection)3. Blepharitis associated disease

OCULAR, LOCAL, LESS SEVERE NON INFECTIOUS DISEASE

OCULAR, LOCAL,MOST SEVERE DISEASEBACTERIAL OR FUNGAL KERATITIS

ABSENT PRESENTdischarge

decreased vision

epithelial defect

hypopyon

worse w/ steroid alone

Kanski: Characteristics of INFECTIVE vs STERILE corneal infiltratesSize Tend to be larger Tend to be smallerProgression Rapid slowEpithelial defect Very common and

larger when presentMuch less common and if present tend to be small

Pain Moderate to severe mildDischarge Purulent mucopurulentSingle or multiple Typically single Commonly multipleUnilateral or bilateral Unilateral Often bilateralAC Rxn Severe mild

Location Often central Typically peripheral

Adjacent corneal rxn Extensive limited

Features

HSV marginal ulcer

Staph. marginal infiltrate

Etiology Active HSV Immunologic response to staph. antigen

Epithelial defect Always

Absent (if present, late)

Neovasc. Often Never Progression

Centrally Circumferentially

Blepharitis

Unrelated Usually

Location Any meridian Typically 2, 4, 8, 10 o’clock meridians

Skin +/- vesicles blepharitis

OCULAR, LOCAL,MOST SEVERE DISEASE

HSV stromal keratitisSeen here but absent in our pt:-INFILTRATE SPREAD CENTRALLY-stromal neovasc. Present (THIS IS IMAGE IS ONE EXAMPLE OF INTERSTITIAL KERATITIS)

-not ruled out yet

SYSTEMIC (DISTANT CHANGES) 1. Interstitial Keratitis (IK)

-Hallmark are ghost vessels2. Peripheral Ulcerative Keratitis (PUK)

-EPITHELIAL DEFECT

DUANE’s ONLINE Systemic Disease

Corneal Change

Patho-gnomonic

Suggestive(distant

disease)

Nonspecific(local disease)

Infiltrates  NONE Crohn's disease

(peripheral infiltrate)Acne rosacea and other skin diseases

  (FORCEDTOINVEST-IGATE) 

Leukemia (marginal infiltrates and deposits)

Blepharitis associated marginal keratitis

(ADDRESS LATER)

Interstitial

Keratitis

  NONE Syphilis

MANY OTHERS(ADDRESS NEXT)

 

Hallmark of Interstitial Keratitis Stromal Ghost vessels/neo

Interstitial KeratitisBacterial infection Parasitic infectionSyphilis (could be latent) No skin changes, noTuberculosis (neg Tb tests) diarrhea, and no contactLeprosy (skin) lens wearLyme disease (+ hx insect bite with rash)Brucellosis (no fever) Trachoma (no conj scar)Viral infection Systemic diseaseHerpes simplex virus Cogan's syndr.(hearing okay)Herpes zoster virus (skin) Sarcoidosis (neg. chest XR)Epstein-Barr virus (no symp) Lymphoma (no symptoms)Mumps (no symptoms) Measles (skin)HTLV-1 (no demyelinating)

Kanski: Peripheral Ulcerative Keratitis

THINNED CONSIDERABLY

Peripheral Ulcerative Keratitis

ONLY SHOWING DIFFERENTIALS NOT ALREADY DISCUSSED

Parasitic infection

OcularSuperior limbic keratoconj.Systemic

Rheumatoid arthritis (negative)

Bacterial infection Wegener’s Gran. (neg ANCA)Syphilis (could be latent) Lupus (neg ANA 2002)Viral infection Malignancy (hx of bladder)Herpes simplex virus Lupus (neg ANA 2002)Hepatitis C (negative 1999)

Inflam. bowel dis (negative colonoscopy)

AIDS (normal WBC 2013) Others (including Sjogren’s)

I. Blepharitis Assoc. Keratitisincludes:

1. Marginal keratitis2. Phlyctenulosis3. Ocular Rosacea

LOCAL CAUSES

I. Blepharitis Associated KeratitisABSENT PRESENT

Less than 2 clock hours of the peripheral cornea

Less ulcerative tendency

Does not progress centrally

LOCAL CAUSES

Signs of blepharitisABSENT MILDLY PRESENT

crustiness, collarettes, flaking

telangiectasia of eyelids and face

chronic papillary conjunctivitis

meibomian gland dysfunction

LOCAL CAUSES

Marginal keratitis ABSENT PRESENT

surrounded by 1mm clear zone

Anterior stromal infiltrate

Usually less than 1 clock hour long

Predilection for 2, 4, 8, 10 o’clock positions

In some cases multiple infiltrates can coalesce to form a larger ring infiltrate

LOCAL CAUSES

Kanski: Marginal Keratitis

Phlyctenulosis ABSENT PRESENT

pinkish white nodule usually originating at limbus

as lesion evolves the elevated nodule ulcerates

recurrent lesions extend farther toward central cornea

LOCAL CAUSES

LOCAL CAUSES

Ocular RosaceaABSENT PRESENTsome infiltrates ulcerate and can perforate

cutaneous signs need not be severe for ocular involvement

diffuse gray opacification of peripheral stroma with superficial vascularization

Severe cases develop recurrent peripheral infiltrates central to peripheral vascularization

Kanski: Ocular Rosacea

Severe cases develop recurrent peripheral infiltrates central to

peripheral vascularization

Kanski: Summary of characteristics of chronic blepharitisAnterior blepharitis Posterior

bleph.Feature Staphylococcal Seborreic

Lashes Deposit Hard SoftLoss ++ +Distorted/trichiasis ++ +

Lid Margin Ulcer +Notch + ++

Cyst Hordeolum ++Meibomian ++

Conjunctiva Phlyctenule +Tear Film Foaming ++

Dry eye + + ++

Cornea(phlyctenules not included)

Punctuate erosions + + ++Vascularization + + ++infiltrates + + ++

Assoc. Disease Atopic disease Seb. dermatitis Acne RosaceaMarginal Keratitis clear zone

Some questions we want answered

• Is this red eye infectious?• Is this red eye autoimmune?• Is this red eye blepharitic?•None have been answered completely but we have identified the next steps

To address infection & autoimmunity:

• herpes antibody titers• Lyme titer• FT-ABS, VDRL• Culture the infiltrate• antithyroid peroxidase antibodies• Anti SSA and anti SSB• Rheumatologic consultation

To address blepharitic causes:

•Antibiotic ointment•Educate pt to greater attention to lid hygiene

•Responsiveness to doxycycline