Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Dermatology > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

Signs of active disease in DLE

A

Perifollicular erythema and easily extractable anagen hairs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Childhood DLE

A

Lower rate of photosensitivity and a higher rate of systemic involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

DLE DIF

A

Old lesional skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Concomitant DLE in SCLE

A

20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

SCLE DIF

A

Granular fluorescence throughout the cytoplasm and nucleus, particulate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

SCLE drugs

A

Hydrochlorothiazide MC per Andrews

ACE inh, CCB’s, IFNs, AEDs, griseofulvin, glyburide, piroxicam, penicillamine, spironolactone, terbinafine, statins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Acral dermal mucinosis or telangectasias

A

May predominate in some NLE cases

*tel may occur in non sunexposed areas and be persistent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

HLA in NLE

A

DR-3

Japanese infants have anti-dsDNA and 8% progress to SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

C’ deficiency associated with lupus

A

C2 and C4
Tend to have SCLE annular lesions
C4 may have hyperkerarosis of palms and soles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Skin involvement in SLE

A

80%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

DIF in SLE

A

IgG, IgM, IgA or C3 in a granular pattern at BMZ

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Nailfold changes in SLE vs DM

A

SLE - wandering capillary loops
DM/scleroderma - symmetrical dilation and drop out

HHT - ecstasia of half of the capillary loop

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Lupus hairs

A

Short frontal hairs from chronic telogen effluvium and increased fragility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Palisaded neutrophilic and granulomatous dermatitis

A

May be seen in SLE, RA or other IC mediated disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Systemic symptoms in SLE

A

IC mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MC earliest systemic sx in SLE

A

Arthralgias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

MC COD in SLE

A

First 5 years - infection and inflammatory SLE lesions

Late - thromboses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

MC cardiac finding in SLE

A

Pericarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Raynauds in SLE

A

15%

Less renal dz associated = less morality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Have been reported in DLE evolving to SLE

A

Insulin receptor antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

UV sensitivity in SLE

A

Both UVA and UVB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Gene susceptibility in lupus

A

Defective CRP response in flares
APRIL (TNF alpha family) polymorphism sin SLE
Increased expression of TNF alpha and IFN inducible protein myxovirus protein A noted in cutaneous lupus
Polymorphisms in C1qA are associated with systemic and cutaneous lupus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Immune alterations in lupus

A

Reduced T suppressor function

Overproduction of gamma globulins and B cells and reduced clearance of IC may contribute to C’ mediated damage

Externalizations of cellular ag like SSA in response to sun,ignited may lead to cell injury by antibody dependent cellular toxicity

HLA-DR4 people, who are slow acetylators, predisposed to hyralazine induced LE

Penacillamine and etanercept may unmask disease with anti dsDNA

Pegylated IFN and ribavirin have produced SLE

L-canavanine (alfalfa sprouts) - can induce or worsen SLE

Smoking associated with increased disease activity and interferes with antimalarial drugs

Globulin increased, especially gamma and alpha 2 fraction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

MC urine findings in lupus

A

Albumin, RBC’s, and casts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
ANA positive rate in SLE
95%
26
Lupus band
Continuous granular along the Dej in 75% of well established DLE lesional skin Positive in sun exposed skin in SLE A positive test in non sun exposed skin correlates with dsDNA and renal disease
27
SLE
Increased risk of osteoporosis independent of steroids
28
Lupus treatment
Sun avoidance vit d and ca supplementation Local - steroids, CCI, ILTAC Systemic - antimalarials (consider another agent if no response in 3 mo) --> retinoids --> immunosuppressants --> rituximab steroids for flares (limit to 3 weeks unless renal or CNS)
29
Antimalarial side effects
Ocular, erythroderma, EM, purpura, urticaria, nervousness, tinnitus, Abducens nerve paralysis, leukopenia, thrombocytopenia, psoriasis, N/V/D *may exacerbate (except in small doses) or cause hepatic necrosis in PCT
30
Tocilizumab
IL-6 inhibitor | May cause neutropenia
31
Wong type DM
DM with clinical findings of PRP
32
In DM: anti jo 1, anti pl-7, anti pl-12, anti-DJ and anti EJ
Correlate with pulmonary disease which is frequent COD
33
Higher risk of malignancy in DM
Highest in first 2 years of dx, age, constitutional sx, rapid onset, lack of raynauds, super high esr or ck, LCV
34
Childhood DM
Brunsting type - slower course, progressive weakness, calcinosis, steroid responsive Banker - vasculitis of muscle and GI tract, rapid onset of severe weakness, steroid unresponsiveness, high death rate *malignancy not associated but insulin resistance might
35
Initial immune response in DM
IFN alpha and beta induced with secondary stimulation of IFN gamma
36
Juvenile DM
DQA1*0501 in 85% TNF alpha 308A allele associated with increased TNF synthesis and thrombospondin-1 and small v. Occlusion Strep m protein may have homology to myosin
37
Adult DM
IL-1 alpha, TGF beta, myoblast production of IL-15 may be pathogenic Terbinafine DM cases related to apoptosis induced by the drug
38
Path of DM vs lupus
More likely to become atrophic, less eccrine, fewer vertical columns of lymphocytes and fibrous tract remnants
39
ANA pos rate in DM
60-80%
40
Crest eponym
Thibierge-Weissenbach syndrome
41
MC initial presentation of SS
Raynauds
42
Scleroderma nail fold changes
Dilated capillary loops, nailfold capillary hemorrhage in 2 or more fingers *HHT has dilation of only 1/2 of of the loop with no avascular areas
43
Childhood scleroderma
Less raynauds, more cardiac (1/2 deaths)
44
Major COD in scleroderma
Pulmonary
45
MC organ involved in scleroderma
Esophageal in 90%
46
Telangectasias in scleroderma associated with
Pulmonary vascular disease
47
MC cancers in scleroderma
Malignancy associated in 10% | Lung and breast MC
48
Most specific ANA Pattern in scleroderma
Anti nucleolar
49
Linear scleroderma ab
Anti-SS DNA
50
Sclerodermoid conditions
Occupational exposure to silica, epoxy resins, polyvinyl chloride, vibratory stimuli Bleomycin, INH, pentazocaine, valproate sodium, vit k injections, Spanish rapeseed oil, tryptophan, nitrofurantoin, hydantoin Presenting scleroderma like sx may be 1st sign of multiple myeloma and amyloidosis
51
Pathogenesis of scleroderma
Anticardiolipin and anti beta2 glycoproteins play a role Plasma d diner correlates with macrovascular complications Unregulated proteins - monocyte chemoattractant protein 1, pulmonary and activation-regulated chemokine, macrophage inflammatory protein-1, IL-8, PDGFR-beta, and TGF beta --> these factors stimulate ECM production, TGF-beta production and activation and chemoattraction of T cells PHET (protein highly expressed in testes) - ab noted in diffuse cutaneous scleroderma and lung involvement CD40 increased on fibroblasts - binding with ligand increases IL-6, 8, and monocytes chemoattractant protein 1 Etanercept shown to decrease TGF-beta1, tissue hydroxyproline, dermal fibrosis, and alpha SMA positive cells - bc TH2 cells reduce net collagen 1 synthesis through TNF alpha, biological should be used with caution
52
Balicatib
Cathepsin K inhibitor that causes drug induced morphea
53
HFS with sclerodactyly
Capecitabine
54
Raynauds treatment
Stop smoking - 3 to 4 times more likely to have digital vascular complications Nifedipine is first line - may use diltiazem if worsening esophageal function Vasodilation drug (CCBs, angiotensin II R antagonists, topical nitrates and prostanoids), ginkgo, L-arginine may reverse digital necrosis
55
Eosinophilic fasciitis
May occur after strenuous exercise Indurstion of forearms and legs, peau d orange, hands/face spared, dry riverbed sign Steroid responsive , hydroxyzine and cimetidine may also help Environmental triggers - L-tryptophan, borrelia, trichloroethylene Polycythemia vera, metastatic colorectal CA, multiple myeloma have been associated May see sys sx- carpal tunnel, neuropathy, sz, optic neuropathy, pleuropericard eff, pancytopenia, hemolysis, sjogrens, LAD, pernicious anemia and IgA nephropathy Striking elevation in TGF-beta1 TH17 mediated pathway Eosinophilia in 10-40%
56
MCTD DIF
Particulate epidermal nuclear IgG deposition
57
Anti-TS1 RNA antibodies
MCTD with lupus like clinical features
58
Ab noted in NSF
Anti phospholipid
59
Immunostains in NSF
Increased CD34 and procollagen 1 - cells may represent circulating fibroblasts recruited to the dermis
60
Skin findings in sjogrens
Vasculitis (accounts for most patients with waldenstroms hypergamma) pruritus, xerosis, annular erythema, decreased sweating
61
Pathogenesis of sjogrens
Aquaporin family of water channels is imp CD4 cells IL-12 and IFN gamma upregulated Th1 cytokines mediate the functional interactions bw APC's and cd4 cells
62
Increased malignancy in sjogrens
NHL *patients with palpable purpura, low C4 and cryoglobulinemia at a higher risk of malignancy
63
Sjogrens Tx
Pilocarpine and cevimeline to stimulate salivation Topical CSA and topical IFN for oral lesions Acid maltose losenges
64
JIA
Evanecsent eruption, neutrophilic panniculitis | IL1 beta, IL6, IL18 and s100 proteins important in patho
65
Correlate with dz activity in relapsing polychondritis
Elevated ESR, CRP and urinary type II collagen levels | Th1 disease

Dermatology (28 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions