Immuno Skin Disorders Flashcards

0
Q

other autoantibdoies that may be in lupus

A

autoabs that cause HSR II–>cytopenias

anti-smith antibodies–>APL

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1
Q

genetic etiology of lupus

A

antinuclear autoantibdoies (ANA) to dsDNA–>form immune complexes–> deposit in multiple tissues–> Hypersensitivity 3

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2
Q

skin findings in lupus

A
malar rash
red scaly rash
discoid rash
photosensitivty
oral ulcers
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3
Q

skeletal findings lupus

A

arthritis of PIP and MCP (like RA, but not deforming)

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4
Q

CV issues in lupus

A

serositis (pleuritis or pericarditis)

*linman-sacks endocarditis)

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5
Q

renal disease in lupus

A

CRF

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6
Q

heme findings in lupus

A

cytopenias

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7
Q

neuroendocrine findings in lupus

A

seizures, stroke, psychosis

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8
Q

labs in lupus

A

ANA antibodies
Anti-dna, anti-sm, APL
decrease in compliment
direct combs

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9
Q

what confirms lupus dx

A

biopsy

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10
Q

three types of cutaneous rashes in lupus

A

acute cutaneous
subacute cutaneous
chronic cutaneous

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11
Q

acute cutaneous percentage that is systemic

A

90%

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12
Q

main rash in ACLE

A

malar or butterly rash

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13
Q

% systemic in subacute cutaneous lupus

A

50% systemic

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14
Q

labs for subacute cutaneous lupus

A

usually ANA negative

anti-Ro, anti- La antibodies often positive though

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15
Q

rash in SCLE

A

widespread red scaly rash on arms, chest, upper back, face

very photosensitive

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16
Q

what can SCLE be secondary to

A

meds- procainamide, terbinafine, HCTZ, nsaids, diltizaem

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17
Q

how do the drugs cause SCLE

A

drugs increase photosensitivity or bind histones and cause +autoantibodie

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18
Q

how to confirm SCLE secondary to drugs

A

ANA is negative
but histone Ab is positive
no decrease in comp, no renal/cns disease and this will all decrease when drug is stopped

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19
Q

neonatal lupus is due to what antibody

A

anti-ro antibodies (cross placenta)

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20
Q

what is the rash for neonatal lupus

A

transient skin rash on face, around eyes, trunk

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21
Q

what category does neonatal lupus fall into

A

SCLE –>annular and scaly

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22
Q

what is the risk of neonatal lupus

A

complete heart block

*can require pacemaker

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23
Q

what can neonatal lupus be associated with

A

hepatobiliary disease, thrombocytopenia

24
Q

percentage of chronic cutaenous lupis that is systemic

A

10%

25
Q

ccle aka

A

discoid lupus

26
Q

rash in ccle

A

very photosensitive and favors light exposure (face and scalp)

27
Q

what is different about CCLE from the other two rashes

A

this one can lead to scarring and the others dont

56
Q

tx lupus

A

sun protection
topical and intralesional steroids
antimalarials (hydroxychloroquine)
biologics-retinoids, immunsups, thaliodmide

57
Q

dematomyositis etio

A

skin + muscle inflammation secondary to CD4 cells–>autoantibodies that target muscle capillaries

58
Q

amyopathic derm

A

no muscle involvment-skin only

59
Q

skin findings in dermatomyositis

A
helicotrope face
gottron's papules
gottron's sign
perungal telangiectasias
erythema and hyperkeratosis on hands
photosensitive red scaly rash (shawl sign)
60
Q

gottron’s papules

A

flat red papules on dorsal surface of hands, over jts

61
Q

gottron’s sign

A

symmetric, scaly, erytehmatous rash over the extensor surfaces of the hands (usually over jts)

62
Q

muscular findings in dermatomyositis

A

proximal muscle weakness (difficulty rising from chair or combing hair)
increase muscle enzymes (CPK)
abnormal EMG
abnormal bx

63
Q

what does the biopsy show

A

muscle tissue necrosis and atrophy

64
Q

what do you see in children

A

calcinosis cutis–>ectopic calcium deposits in tissues (skin & lung–>breathing problems)
*can be painful and debilitating

65
Q

what may also be involved in dermatomyo

A

cardiac and pulmonary involvment

66
Q

what is gower’s sign

A

cant get off the floor

67
Q

severity of skin and muscle involvment + antibodies

A

do not necessarily correlate

68
Q

antibodies in dermato

A

ANA+ (remember- ANA- in subacute LE)

anti-trna synthetase (Jo1) antibodies –>pulm dz

69
Q

tx of dermatomyo

A

sun protection
systemic corticosteroids for skin involvment–>FIRST LINE
steroid sparing agents like MTx and azathiprine
IVIG for refractory

70
Q

malignancy in adults

A

10-50% of cases–check!
age >60
w

71
Q

when should you suspect malignancy

A

skin refractory to treatment

72
Q

morphea and scleroderma are dz characterized by

A

thickening of the skin (sclerosis/fibrosis) and vessel walls secondary to increases in collagen

can sometimes involve deeper tissues in underlying areas such as subC tissue, muscle, bone

73
Q

gender and race in sclerodermas

A

F>M

black >white

74
Q

morphea

A

localized and superficial form of scleroderma that is strictly skin– no raynaud’s, no abnormal labs- NOT fatal

75
Q

what do you see in morphea

A

slowly expanding inflammatory patches and plaques; usually on the trunk, extremities, or face
**can initially be violaceous or hyperpigmented, can involve subcutaneous tissue, can cause disfigurment

76
Q

if mirphea is over joints

A

can form contractures and decrease ROM of knees and arms

77
Q

morphea eventually

A

burns out-stops expanding and softens, but some residual textural and pigment abnormality usually present

78
Q

therapy of morphea

A

MTX + prednisone

79
Q

scleroderma (systemic sclerosis) 10 year survival

A

10%

80
Q

T cells in scleroderma

A

CD4 TH2 cells–> positive ANA (95% of patients) to
-DNA topoisomerase I (scl 70, 30-70% diffuse–associated wtih pulm fibrosis)
centromeres (40% in crest)

81
Q

earliest endothelial finding in scleroderma

A

vasculitis secondary to finrosis of walls, decrease PG

82
Q

skin fidnings in sceleroderma

A
thickening and contration of skin
hyperpig
telangiectasias
digital ulers
sclerodacytly
83
Q

systemic features of scleroderma

A

raynaud’s
arthralgia
esopheal/GI, lung, kidney, heart

84
Q

what is usally the intiial sign scleroderma

A

raynauds

85
Q

CREST syndrome- subset of sclero

A
Calcinosis
Raynauds
esophageal dysmotility
Sclerodacyylyl
telangiectasias
86
Q

tx of scleroderam

A

Ca channel blockers for raynauds
AceI for renal dz
immunosup-mTX, azathiprine