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Ocular Dx Exam 2 Autoimmune
Ocular Dx Exam 2 Dr. Erickson
57
Medical
Graduate
03/12/2011

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Cards

Term
What is poliosis?
Definition
Auto immune attack of eyelash melanocytes --> white lashes
Term
What causes poliosis?
Definition
staph bleph; albinism; Vog-Koyanagi-Harada syndrome
Term
What does VKH stand for?
Definition
Vogt-Koyanagi-Harada syndrom
Term
Poliosis tx?
Definition
treat the bleph; refer if albinism/VKH present
Term
What is vitiligo?
Definition
hypopigmentation of skin
Term
What are s/sx of vitiligo?
Definition
usually bilateral eyelid presentation, frequently positive FHx
Term
What age does vitiligo present?
Definition
Usually before 20
Term
What is the cause of vitiligo?
Definition
idiopathic
Term
Is vitiligo progressive?
Definition
yes
Term
What is the occurrence of vitilgo?
Definition
1-2% worldwide, more often in dark skinned people
Term
Vitiligo tx?
Definition
determine of FHx;

rule out associated syndromes,

use cosmetic camouflage,

sunscreen,

melanocyte stimulation and skin color blending w/ amino acids
Term
What is the long term tx for vitiligo?
Definition
steroids
Term
What must be done with vitiligo pts?
Definition
Referred to dermatologist
Term
What is Vogt Koyanagi Harada Syndrome?
Definition
rare, multisystem disorder.

Common age 20-50
Term
What is VKH phase 1?
Definition
Prodromal phase

Lasts a few days with meningitis, encephalopathy, auditory disturbances
Term
What is VKH phase 2?
Definition
Acute uveitic phase: anterior and posterior uveitis
Term
What is VKH phase 3?
Definition
Convalescent phase; first three of the sx below most common

Alopecia, poliosis, vitiligo, fundus lesions, depigmented limbal lesions
Term
What is VKH phase 4?
Definition
Chronic-Recurrent phase

smoldering anterior uveitis w/ exacerbations
Term
Three of what four signs must be present to diagnose VKH?
Definition
bilateral chronic anterior uveitis

posterior uveitis

cutaneous lesions

neurological features
Term
What is a severe hallmark sign of VKH?
Definition
acute serous retinal detachment
Term
Where is sunset glow fundus found?
Definition
Convalesent phase of VKH
Term
What is the tx plan for VKH?
Definition
oral steroids

periocular injected steroids

topical steriods for anterior uveitis

If steroids are not sufficient, use immunosuppressive agents cyclosporine or chlorambucil
Term
What does OCP stand for?
Definition
Ocular Cicatricial Pemphigoid
Term
What is OCP?
Definition
systemic autoimmune inflammatory disease

mucous membrane disorder involving the conjunctiva, often asymmetric
Term
What type of hypersensitivity is OCP?
Definition
Type 2 hypersensitivity
Term
What is the prevalence of OCP?
Definition
Rare

1/8k to 1/45k
Term
What is a pemphigoid?
Definition
blister or swelling
Term
What is a significant side effect of OCP?
Definition
bilaterally blindness/visual loss
Term
Why does OCP scar?
Definition
Blisters form, swell, and burst repeatedly until scars are formed
Term
What blisters in OCP?
Definition
skin and mucous membranes
Term
What can be asymmetric in OCP?
Definition
time of onset

severity

rate of progression
Term
When is conjunctival dx seen in OCP?
Definition
75% w/oral involvement; 25% w/ skin lesions
Term
Name some characteristics of OCP
Definition
idiopathic

chronic

progressive

female > male
Term
What is the age of onset for OCP?
Definition
6th or 7th decade, often subtle and hard to detect, though early detection crucial for preventing vision loss
Term
What is stage1 of OCP?
Definition
Conjunctivitis
Term
What is stage 2 of OCP?
Definition
Conjunctival scarring and shrinkage

worsens over 3-4 years

continued scarring and shrinkage
Term
What is stage 3 of OCP?
Definition
symblepharon formation
Term
What is stage 4 of OCP?
Definition
end stage with corneal scarring and anklyblepharon
Term
What is a symblepharon?
Definition
fibrous tissue adhering bulbar to palpebral conj
Term
What is anklyoblepharon?
Definition
Severe overgrowth of tissue over the cornea?
Term
What is the endstage of OCP?
Definition
scarred, opaque cornea
Term
What are other DDx related to OCP?
Definition
Chemical trauma

radiation injury

other mucous membrane disorders
Term
What % of OCP cases are confirmed with a conjunctival biopsy?
Definition
83.00000%
Term
How can OCP cause dry eye?
Definition
destroys goblet cells and meibomian glands
Term
What is Dapsone used for?
Definition
Treating the acute inflammatory stage of OCP
Term
What are ocular therapies for OCP?
Definition
treat w/ surgery:

trichiasis, entropion and symblepharon
Term
What is Stevens-Johnson Syndrome?
Definition
acute but self limiting (non-progressive) inflammation with minor and major variants
Term
What is EM minor?
Definition
skin and maybe a single mucosal membrane affected
Term
What is EM major?
Definition
Involves skin and two or more mucosal membranes
Term
How long can EM last?
Definition
1-4 weeks
Term
What is usually seen with SJS?
Definition
Systemic toxicity causing malaise, fever, sore throat, HA
Term
What is the mortality rate of SJS?
Definition
1-5%
Term
How long can SJS last?
Definition
6 weeks
Term
What % of SJS is seen in children?
Definition
20.00000%
Term
Who are more likely to get SJS?
Definition
men, though no racial predilection
Term
What is the etiology of SJS?
Definition
Thought to be delayred hypersensitivity response to drugs and epithelial cell antigens
Term
What is common pharmacological cause of SJS reaction?
Definition
sulfonamides***

penicillin, nsaids, etc
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