Autoimmune Disease3

Question 1

Poliosis is characterized by immune reseponse to melanin. What are a few causes?

Answer 1

Staph blepharitis, albinism, VKH syndrome

Question 2

What is the name of the condition characterized by loss of pigmentation of the skin?

Answer 2

Vitiligo

Question 3

Vitiligo usually presents before what age?

Answer 3

20

Question 4

What perentage of the world’s population has vitiligo?

Answer 4

1-2%

Question 5

What are some treatment options for vitiligo?

Answer 5

Topical steroids, PUVA, grafting depigmentation

Question 6

VKH is a multisystemic disorder more common in what populations?

Answer 6

Darker people; ages 20-50

Question 7

What are the four phases of VKH?

Answer 7

Prodromal (meningitis, encephalopathy, auditory disturbances); Acute Uveitis Phase (anterior or posteroid uveitis, can lead to retinal detachment); Convalescent phase (alopecia, poliosis, vitiligo, fundus lesions, depigmented limbal lesions); Chronic-recurrent phase (anterior uveitis with exacerbations)

Question 8

In the chronic-recurrent phase of VKH, there are mutton fat (clumps of white blood cells) in the corneal endothelium. What other sign might accompany this phase?

Answer 8

Posterior synechiae

Question 9

In order to diagnose VKH, you need to match symptoms in three of four of which categories?

Answer 9

Bilateral chronic anterior uveitis, posterior uveitis, neurological features, cutaneous lesions

Question 10

What is a hallmark sign of VKH?

Answer 10

Acute serous retinal detachment

Question 11

Comparing Vogt-Koyanagi to Harada, which has the worse visual prognosis?

Answer 11

Vogt-Koyanagi (Hurray I have harada; Shoutout to Nikki.)

Question 12

If treatment of VKH with steroids is not sufficient, what other types of medications may be used?

Answer 12

Immunosuppressive agents: methotrexate, cyclosporine, chlorambucil

Question 13

Ocular cicatricial pemphigoid is ________ (chronic/acute), progresses __________ (quickly/slowly), ___________(bilateral/unilateral), potentially blinding, systemic, autoimmune disease.

Answer 13

Chronic, slowly, bilateral

Question 14

OCP involves what types of tissue?

Answer 14

Skin, mucous membranes (conjunctiva)

Question 15

OCP is what type of a hypersensitivity reaction?

Answer 15

Type II

Question 16

True or false: OCP is very common

Answer 16

False; it is rare

Question 17

Conjunctival disease is seen in how many cases of oral involvement? What about skin involvement?

Answer 17

75% oral, 25% skin

Question 18

True or false: OCP is bilateral, each eye starts at the same time, and each eye progresses at the same rate

Answer 18

False; time of onset, severity, and rate of progression can vary between eyes

Question 19

Which gender has a higher prevalence of OCP?

Answer 19

Female

Question 20

True or false: OCP is more common in Caucasians

Answer 20

False; it actually has no racial predilection

Question 21

When is OCP typically diagnosed?

Answer 21

Later in life (50-60 years), though it probably originates earlier

Question 22

What are the four stages of OCP?

Answer 22

Conjunctivitis (particularly if associated with subconjunctival fibrosis); Conjunctival scarring and shrinkage (occurs over the next 3-4 years; conjunctivitis starts in other eye); Symblepheron formation (connection between palpebral and bulbar conjunctivas); End stage with corneal scarring and ankyloblepheron (including dry eye, vision loss)

Question 23

In advanced cases of OCP, what are some corneal signs?

Answer 23

Vascularization, limbal inflammation (stem cell destruction), stromal thinning and ulceration), opacification

Question 24

OCP can be treated with topical, and probably oral medications. Which medications would you use?

Answer 24

Prednisone, dapsone, cyclophosphamide, azathioprine

Question 25

Erythema multiforme is divided into what categories?

Answer 25

Minor (typical targets or raised, edematous papules in extremities) and major (typical targets or raised, edematous papules distributed to the extremities with involvement of one or more mucous membranes, epidermal detachment less than 10% of TBSA)

Question 26

Erythema multiforme is typified by what sort of skin lesions?

Answer 26

Target or bull's eye lesion in extremeties: erythematous center with a pale surrounding ring with a larger erythematous ring

Question 27

What are the clinical features of SJS/TEN (Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis?

Answer 27

Widespread blisters on trunk and face, with erythematous or pruritic macules and one or more mucous membrane erosions.

Question 28

Stevens-Johnson Syndrome, if not overlapping with TEN, involves how much of the total body surface area?

Answer 28

Less than 10%

Question 29

If Stevens-Johnson Syndrome Toxic Epidermal Necrolysis overlap, how much of the total body surface area is affected?

Answer 29

Between 10-30%

Question 30

Toxic epidermal necrolysis, if not overlapping with SJS, involves how much of the total body surface area?

Answer 30

More than 30%

Question 31

SJS is a mild form of TEN (or TEN is a severe form of SJS, not sure which). How do these two conditions differ?

Answer 31

SJS is less common than TEN; SJS mortality is low at 1-5% while TEN is higher at 25-35%

Question 32

True or false: the onset of SJS/TEN is slow

Answer 32

False; onset is acute

Question 33

What age group is usually affected by SJS/TEN?

Answer 33

Children are 20% of patients, though the peak is between 10-30.

Question 34

Which gender has a higher prevalence of SJS/TEN?

Answer 34

Men

Question 35

It is thought that SJS/TEN is caused by…?

Answer 35

Delayed hypersensitivity response to drugs (sulfonamides**, tetracycline, penicillin, NSAIDs, etc), response to epithelial cell antigen, microbial infection (myoplasma pneumoniae**, bacteria, viruses such as HSV**)

Question 36

What are the systemic features of SJS/TEN?

Answer 36

Fever, malaise, HA, loss of appetite, N&V, cough, sore throat, URTI

Question 37

Diagnosis of SJS is partially based on skin involvement. What sort of signs might you find in a SJS patient?

Answer 37

Vesicles, bullae on trunk

Question 38

Diagnosis of SJS is partially based on mucous membrane involvement. Which membranes are commonly involved?

Answer 38

Mouth and eyes

Question 39

What are some of the ocular symptoms of SJS/TEN?

Answer 39

Lids (swelling, crusting, ulceration, entropion, trichiasis); conjuntiva (papillary or pseudomembranous conjunctivitus, fibrosis, necrosis, keratinization), lacrimal dysfunction, keratopathy

Question 40

What are some systemic treatment options for SJS/TEN?

Answer 40

No specific treatment, though steroids have been used, as have oral antibiotics and antivirals.

Question 41

How would you treat ocular SJS/TEN?

Answer 41

Broad spectrum antibiotics, topical steroids, soft CLs, surgery (lid scrubs, epilation, cool compresses as adjunct therapy)