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DCNP Review > Vesiculobullous, Autoimmune & Connective Tissue Disease > Flashcards

Vesiculobullous, Autoimmune & Connective Tissue Disease Flashcards

1
Q

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?

A

Anti-Jo-1

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2
Q

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

A

Calcinosis

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3
Q

Which cutaneous findings are considered pathognomonic for dermatomyositis?

A

Periorbital heliotrope and gottron’s papules

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4
Q

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?

A

Low or absent IgA level

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5
Q

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

A

Low or absent TPMT activity

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6
Q

Ehlers-Danlos syndrome is caused by a genetic mutation in

A

Collagen fibers

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7
Q

The most common type of Ehlers-Danlos syndrome

A

Classic type

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8
Q

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

A

Arthrochalasia Type

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9
Q

The term micrognathia refers to

A

Undersized jaw

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10
Q

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

A

Eyes that appear to be staring

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11
Q

Typical skin findings in Ehlers-Danlos Classic Type include

A

Increased elasticity

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12
Q

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

A

Neutrophils

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13
Q

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

A

Vancomyocin

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14
Q

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

A

Genitalia

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15
Q

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

A

60 to 70 years of age

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16
Q

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

A

Extensor areas of extremities

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17
Q

Definitive diagnosis for linear IgA bullous dermatitis is

A

Linear deposits of IgA autoantibodies on the DIF

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18
Q

The drug of choice to treat linear IgA bullous dermatitis is

A

Dapsone

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19
Q

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

A

Progressive Lesion

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20
Q

Which type of biopsy is recommended for suspected morphea?

A

Punch

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21
Q

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

A

PUVA

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22
Q

Which type of pemphigus is the most common?

A

Pemphigus Vulgaris

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23
Q

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

A

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

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24
Q

Paraneoplastic pemphigus is associated with

A

Lymphoma

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25
Q

First line treatment of pemphigus is

A

Systemic glucocorticoids

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26
Q

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

A

Eosinophils

27
Q

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

A

Nikolsky sign

28
Q

The most common type of autoimmune blistering disorder is

A

Bullous pemphigoid

29
Q

The onset of bullous pemphigoid usually starts after age

A

60

30
Q

The initial antigen involved in the development of bullous pemphigoid is

A

BP antigen 180

31
Q

Bullous pemphigoid has a significant association with what condition?

A

Neurological conditions

32
Q

Drug induced bullous pemphigoid can be associated with which medication

A

Furosemide

33
Q

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

A

Perilesional area

34
Q

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

A

Systemic corticosteroids

35
Q

The lesions of cicatricial pemphigoid are most likely to occur where

A

Eyes and mouth

36
Q

Pemphigoid gestational generally occurs during

A

Both 2nd and 3rd trimesters

37
Q

Lesions of pemphigoid gestationis often start where

A

Umbilicus

38
Q

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

A

Dapsone hypersensitivity syndrome

39
Q

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

A

20%

40
Q

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

A

90-95%

41
Q

Lupus period can be associated with

A

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

42
Q

Lofgren syndrome is a triad of which symptoms

A

Erythema nodosum, polyarthritis and hilar lymphadenopathy (also fever)

43
Q

Erythema nodosum can be described as

A

Most common non-specific eruption of sarcoidosis

44
Q

Epidermolysis bullosa simplex located form primarily affects

A

Palms and soles

45
Q

What are characteristics of Kindler syndrome of epidermolysis bullosa?

A

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

46
Q

The cleavage line associated with epidermolysis simplex is located at

A

Intraepidermal

47
Q

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

A

Obtaining 1/2 to the whole blister with surrounding skin

48
Q

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

A

Petroleum jelly

49
Q

Abnormalities of scleroderma include

A

Vascular dysfunction, tissue fibrosis and autoantibody production

50
Q

What percentage of scleroderma patients experience Raynaud’s phenomenon?

A

95%

51
Q

Patients with diffuse scleroderma may develop scar tissue in

A

Heart, lungs, skin

52
Q

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

A

Pulmonary

53
Q

The CREST acronym stands for

A

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

54
Q

The early stages of scleroderma begin with

A

Edema in the fingers

55
Q

Lupus erythemstous is more common in

A

African American Females

56
Q

An environmental trigger for lupus erythematous is

A

UV exposure

57
Q

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

A

Discoid lupus erythematous

58
Q

Which medication is associated with drug-induced subacute lupus erythematous

A

Hydrochlorothiazide

59
Q

The inflammation in lupus panniculitis is in the

A

Subcutaneous tissue

60
Q

Neonatal lupus can be associated with

A

Heart block

61
Q

Systemic lupus erythematous is commonly associated with

A

Weight loss

62
Q

Acute cutaneous lupus erythematous may mimic what conditions?

A

Rosacea, sunburn, drug induced photosensitivity

63
Q

The systemic drug of choice in treatment of lupus erythematous is

A

Hydroxychloroquine sulfate

64
Q

A potential adverse effect associated with antimalarial medicatio is

A

Eye toxicities

DCNP Review (12 decks)

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