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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

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2
Q

Childhood DLE

A

Lower rate of photosensitivity and a higher rate of systemic involvement

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3
Q

DLE DIF

A

Old lesional skin

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4
Q

Concomitant DLE in SCLE

A

20%

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5
Q

SCLE DIF

A

Granular fluorescence throughout the cytoplasm and nucleus, particulate

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6
Q

SCLE drugs

A

Hydrochlorothiazide MC per Andrews

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

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7
Q

Acral dermal mucinosis or telangectasias

A

May predominate in some NLE cases

*tel may occur in non sunexposed areas and be persistent

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8
Q

HLA in NLE

A

DR-3

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

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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

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10
Q

Skin involvement in SLE

A

80%

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11
Q

DIF in SLE

A

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

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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

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13
Q

Lupus hairs

A

Short frontal hairs from chronic telogen effluvium and increased fragility

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14
Q

Palisaded neutrophilic and granulomatous dermatitis

A

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

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15
Q

Systemic symptoms in SLE

A

IC mediated

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16
Q

MC earliest systemic sx in SLE

A

Arthralgias

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17
Q

MC COD in SLE

A

First 5 years - infection and inflammatory SLE lesions

Late - thromboses

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18
Q

MC cardiac finding in SLE

A

Pericarditis

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19
Q

Raynauds in SLE

A

15%

Less renal dz associated = less morality

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20
Q

Have been reported in DLE evolving to SLE

A

Insulin receptor antibodies

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21
Q

UV sensitivity in SLE

A

Both UVA and UVB

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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

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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

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24
Q

MC urine findings in lupus

A

Albumin, RBC’s, and casts

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25
Q

ANA positive rate in SLE

A

95%

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26
Q

Lupus band

A

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

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27
Q

SLE

A

Increased risk of osteoporosis independent of steroids

28
Q

Lupus treatment

A

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
Q

Antimalarial side effects

A

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
Q

Tocilizumab

A

IL-6 inhibitor

May cause neutropenia

31
Q

Wong type DM

A

DM with clinical findings of PRP

32
Q

In DM: anti jo 1, anti pl-7, anti pl-12, anti-DJ and anti EJ

A

Correlate with pulmonary disease which is frequent COD

33
Q

Higher risk of malignancy in DM

A

Highest in first 2 years of dx, age, constitutional sx, rapid onset, lack of raynauds, super high esr or ck, LCV

34
Q

Childhood DM

A

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
Q

Initial immune response in DM

A

IFN alpha and beta induced with secondary stimulation of IFN gamma

36
Q

Juvenile DM

A

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
Q

Adult DM

A

IL-1 alpha, TGF beta, myoblast production of IL-15 may be pathogenic

Terbinafine DM cases related to apoptosis induced by the drug

38
Q

Path of DM vs lupus

A

More likely to become atrophic, less eccrine, fewer vertical columns of lymphocytes and fibrous tract remnants

39
Q

ANA pos rate in DM

A

60-80%

40
Q

Crest eponym

A

Thibierge-Weissenbach syndrome

41
Q

MC initial presentation of SS

A

Raynauds

42
Q

Scleroderma nail fold changes

A

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
Q

Childhood scleroderma

A

Less raynauds, more cardiac (1/2 deaths)

44
Q

Major COD in scleroderma

A

Pulmonary

45
Q

MC organ involved in scleroderma

A

Esophageal in 90%

46
Q

Telangectasias in scleroderma associated with

A

Pulmonary vascular disease

47
Q

MC cancers in scleroderma

A

Malignancy associated in 10%

Lung and breast MC

48
Q

Most specific ANA Pattern in scleroderma

A

Anti nucleolar

49
Q

Linear scleroderma ab

A

Anti-SS DNA

50
Q

Sclerodermoid conditions

A

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
Q

Pathogenesis of scleroderma

A

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
Q

Balicatib

A

Cathepsin K inhibitor that causes drug induced morphea

53
Q

HFS with sclerodactyly

A

Capecitabine

54
Q

Raynauds treatment

A

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
Q

Eosinophilic fasciitis

A

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
Q

MCTD DIF

A

Particulate epidermal nuclear IgG deposition

57
Q

Anti-TS1 RNA antibodies

A

MCTD with lupus like clinical features

58
Q

Ab noted in NSF

A

Anti phospholipid

59
Q

Immunostains in NSF

A

Increased CD34 and procollagen 1 - cells may represent circulating fibroblasts recruited to the dermis

60
Q

Skin findings in sjogrens

A

Vasculitis (accounts for most patients with waldenstroms hypergamma) pruritus, xerosis, annular erythema, decreased sweating

61
Q

Pathogenesis of sjogrens

A

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
Q

Increased malignancy in sjogrens

A

NHL

*patients with palpable purpura, low C4 and cryoglobulinemia at a higher risk of malignancy

63
Q

Sjogrens Tx

A

Pilocarpine and cevimeline to stimulate salivation
Topical CSA and topical IFN for oral lesions
Acid maltose losenges

64
Q

JIA

A

Evanecsent eruption, neutrophilic panniculitis

IL1 beta, IL6, IL18 and s100 proteins important in patho

65
Q

Correlate with dz activity in relapsing polychondritis

A

Elevated ESR, CRP and urinary type II collagen levels

Th1 disease

Dermatology (28 decks)

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