Vesiculobullous, Autoimmune & Connective Tissue Disease

Question 1

If positive in a newly diagnosed patient with Dermatomyositis, what autoantibody results would indicate your patient is at an increased risk for interstitial lung disease?

Answer 1

Anti-Jo-1

Question 2

Which finding is 2 times more common in juveniles than adults with dermatomyositis?

Answer 2

Calcinosis

Question 3

Which cutaneous findings are considered pathognomonic for dermatomyositis?

Answer 3

Periorbital heliotrope and gottron’s papules

Question 4

You have a patient with refractory dermatomyositis and want to order IVIG. You are reviewing the baseline labs. What result would indicate the patient is at increased risk for anaphylaxis?

Answer 4

Low or absent IgA level

Question 5

What lab results would be most likely indicating an increased risk of life threatening bone marrow toxicity in a patient taking Azathioprine?

Answer 5

Low or absent TPMT activity

Question 6

Ehlers-Danlos syndrome is caused by a genetic mutation in

Answer 6

Collagen fibers

Question 7

The most common type of Ehlers-Danlos syndrome

Answer 7

Classic type

Question 8

A baby born with bilateral hip displacement indicating which type of Ehlers Danlos syndrome?

Answer 8

Arthrochalasia Type

Question 9

The term micrognathia refers to

Answer 9

Undersized jaw

Question 10

The typical facial appearance that occurs with some types of Ehlers Danlos Syndrome

Answer 10

Eyes that appear to be staring

Question 11

Typical skin findings in Ehlers-Danlos Classic Type include

Answer 11

Increased elasticity

Question 12

The perilesional biopsy of a patient with a suspected linear IgA bullous dermatitis should demonstrate

Answer 12

Neutrophils

Question 13

What medication is most likely to cause linear IgA bullous dermatitis?

Answer 13

Vancomyocin

Question 14

More than half of all children with linear IgA bullous dermatitis will demonstrate lesions in which body area?

Answer 14

Genitalia

Question 15

Adult onset of linear IgA bullous dermatitis is more likely to occur in which age group?

Answer 15

60 to 70 years of age

Question 16

The lesions of linear IgA bullous disease in an adult patient are most likely to occur in what body area?

Answer 16

Extensor areas of extremities

Question 17

Definitive diagnosis for linear IgA bullous dermatitis is

Answer 17

Linear deposits of IgA autoantibodies on the DIF

Question 18

The drug of choice to treat linear IgA bullous dermatitis is

Answer 18

Dapsone

Question 19

A lesion that has a scleortic center appearing shiny, white with a violaceous outer ring describes which type of morphea lesion?

Answer 19

Progressive Lesion

Question 20

Which type of biopsy is recommended for suspected morphea?

Answer 20

Punch

Question 21

Which treatment option has the highest degree of efficacy in treatment of morphea?

Answer 21

PUVA

Question 22

Which type of pemphigus is the most common?

Answer 22

Pemphigus Vulgaris

Question 23

Which medications are most likely to cause drug-induced pemphigus?

Answer 23

Captopril, penicillamine, thiol drugs, PCN, Cephalosporin, vancomyocin, ace inhibitors.

Question 24

Paraneoplastic pemphigus is associated with

Answer 24

Lymphoma

Question 25

First line treatment of pemphigus is

Answer 25

Systemic glucocorticoids

Question 26

Histology examination of a patient with suspected pemphigus should demonstrate an abundance of what cell in the dermis?

Answer 26

Eosinophils

Question 27

A skin finding that occurs where the tip layers of skin shear away from lower layers with slight rubbing describes

Answer 27

Nikolsky sign

Question 28

The most common type of autoimmune blistering disorder is

Answer 28

Bullous pemphigoid

Question 29

The onset of bullous pemphigoid usually starts after age

Answer 29

60

Question 30

The initial antigen involved in the development of bullous pemphigoid is

Answer 30

BP antigen 180

Question 31

Bullous pemphigoid has a significant association with what condition?

Answer 31

Neurological conditions

Question 32

Drug induced bullous pemphigoid can be associated with which medication

Answer 32

Furosemide

Question 33

A patient suspected of having bullous pemphigoid is to have a diagnostic punch biopsy(DiF). The correct location to perform this lesion is:

Answer 33

Perilesional area

Question 34

The recommended first line treatment for a patient with severe bullous pemphigoid is

Answer 34

Systemic corticosteroids

Question 35

The lesions of cicatricial pemphigoid are most likely to occur where

Answer 35

Eyes and mouth

Question 36

Pemphigoid gestational generally occurs during

Answer 36

Both 2nd and 3rd trimesters

Question 37

Lesions of pemphigoid gestationis often start where

Answer 37

Umbilicus

Question 38

A patient diagnosed with dermatitis herpetiformis has just started dapsone therapy. When he presents for his 4 week check, he complains of fever, itchy rash, and right lower quadrant abdominal pain. Your first concern is

Answer 38

Dapsone hypersensitivity syndrome

Question 39

Your patient with newly diagnosed dermatitis herpetiformis asks if he will have GI symptoms like celiac disease. Your response is

Answer 39

20%

Question 40

You have a patient with a diagnosis of new onset sarcoidosis. What is the likelihood your patient has associated pulmonary disease?

Answer 40

90-95%

Question 41

Lupus period can be associated with

Answer 41

Development of lytic and cystic bone lesions of the hands and feet

Question 42

Lofgren syndrome is a triad of which symptoms

Answer 42

Erythema nodosum, polyarthritis and hilar lymphadenopathy (also fever)

Question 43

Erythema nodosum can be described as

Answer 43

Most common non-specific eruption of sarcoidosis

Question 44

Epidermolysis bullosa simplex located form primarily affects

Answer 44

Palms and soles

Question 45

What are characteristics of Kindler syndrome of epidermolysis bullosa?

Answer 45

Skin fragility, dyspigmentation, acral blister formation at birth, photosensitivity in childhood, diffuse palmoplantar hyperkeratosis

Question 46

The cleavage line associated with epidermolysis simplex is located at

Answer 46

Intraepidermal

Question 47

A patient is suspected of having epidermolysis bullosa and needs a biopsy to confirm the diagnosis. The correct technique includes:

Answer 47

Obtaining 1/2 to the whole blister with surrounding skin

Question 48

Treatment of pruritis symptoms in patients with epidermolysis bullosa should include antihistamines and topical application of

Answer 48

Petroleum jelly

Question 49

Abnormalities of scleroderma include

Answer 49

Vascular dysfunction, tissue fibrosis and autoantibody production

Question 50

What percentage of scleroderma patients experience Raynaud's phenomenon?

Answer 50

95%

Question 51

Patients with diffuse scleroderma may develop scar tissue in

Answer 51

Heart, lungs, skin

Question 52

Mortality associated with scleroderma is usually due to problems with which system?

Answer 52

Pulmonary

Question 53

The CREST acronym stands for

Answer 53

C - calcinosis R - Raynaud's E - esophageal dysfunction S - sclerodactyl T - telangiectasia

Question 54

The early stages of scleroderma begin with

Answer 54

Edema in the fingers

Question 55

Lupus erythemstous is more common in

Answer 55

African American Females

Question 56

An environmental trigger for lupus erythematous is

Answer 56

UV exposure

Question 57

Which type of lupus erythematous is characterized by scarring alopecia, follicular plugging and indurated lesions?

Answer 57

Discoid lupus erythematous

Question 58

Which medication is associated with drug-induced subacute lupus erythematous

Answer 58

Hydrochlorothiazide

Question 59

The inflammation in lupus panniculitis is in the

Answer 59

Subcutaneous tissue

Question 60

Neonatal lupus can be associated with

Answer 60

Heart block

Question 61

Systemic lupus erythematous is commonly associated with

Answer 61

Weight loss

Question 62

Acute cutaneous lupus erythematous may mimic what conditions?

Answer 62

Rosacea, sunburn, drug induced photosensitivity

Question 63

The systemic drug of choice in treatment of lupus erythematous is

Answer 63

Hydroxychloroquine sulfate

Question 64

A potential adverse effect associated with antimalarial medicatio is

Answer 64

Eye toxicities