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pathogenesis Flashcards

1
Q

scleredema: Px

A

↑collagen/mucin( increased synthesis-insulin/hypoxia/vascular damage, reduced degradation-collagenase) 1- post strep 2- monoclonal gammopathy, 3- diabeticorum(IDDM) irreversibly glycosylated collagen

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2
Q

scleromxydema Px

A

Px:mucin,Fibroblast proliferation;

  1. IgGlamda monoclonal gammopathy/plasma cell dyscrasia 2. circulating human factors
  2. absence thyroid disease
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3
Q

aGVHD: Px

A

1.HSCT, host APC activated 2. donor T cell proliferation(cytotoxic T/NK cells) 3. target tissue destruction(skin liver GIT)

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4
Q

cGVHD:Px

A

1.autoAb, sclerotic 2. BAFF(rituximab)

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5
Q

LSA

A

LSA: HLA DR1*12 , autoimmune(thyroid) ECM1, oxidative stress, infectious?borrelia, 8% SCC

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6
Q

EI

A

type4 CMI to Ag stimuli( infx-TB(

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7
Q

HV-

A

EBV, NKT lymphoma/hemophagocytosis; PMLE: persistent DTH response against photoinduced altered endogenous Ag; PCT:

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8
Q

actinic prurigo

A

-persistent PMLE variant, HLADR4, thalidomide anti TNFa, UVR

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9
Q

CAD -

A

1.UVB+UVA absorption by DNA/RNA; 2,DTH response(endog/photoinduced cutaneous Ag, CD8+ Infiltrate; 3.+photoallergy compositae/sunscreen, HIV(11% local)

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10
Q

phototoxic-

A

UVR activation , direct cellular injury; exagerrated sunburn,dose-dependent,photodistr telangiectasia(CCB); pseudoporphyria(frusemide, tetracycline, nalidixic acid) NSC: phenothiazine(chlorpromazine, promethazine); resolution time: T1/2 , persistent photoactive molecules(quinine, thiazide 9m)

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11
Q

photoallergy-

A

drug+photon->photoproduct+protein binding=Ag–>hypersensitivity e.g.

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12
Q

LABD

A

:LAD 97kDa ectodomain BP180

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13
Q

MMP-autoAb

A

BP180(BPAG2 C terminal), laminin 332(a3); a6b4(ocular MMP)

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14
Q

EBA

A

-acquired immune mediated, 290/145 kDa col7(anchoring fibril) autoab, HLADRB1501, response to immunosuppresion; A/W: crohn’s, SLE/RA/thyroiditis

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15
Q

Pvegetans-

A

drugs(ACEi), malignancy, dsg3

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16
Q

PNP:

A

BP230, dsg1/3, desmoplakins, envoplakin, periplakin/plectin,tumor Ag- NHL, CLL, castleman waldenstrom, thymoma sarcoma;

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17
Q

PV/PF-

A

dsg(desmosome) compensation theory blister localisation-expression: dsg1(supf dermis), dsg3(deep epidermis, mucosa); HLA susceptibility

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18
Q

DH:

A

HLA DQ0501/B102, gluten sensitive enteropathy, IgA anti TG3 Ab, neutrophilic, granular IgA, dapsone improves(anti-neutrophilic),iodide worsen

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19
Q

IgA pemphigus-

A

IgA desmocollin1 , dsg1/3, dapsone

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20
Q

EM

A

PGx: HLADQw3/drw53,HSV(REM-70%)/mycoplasma/drug-NSAID/AC/sulfonamide; IBD/Behcets

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21
Q

FDE- pGx:

A

1) resting lesion: quiescent primed intraepidermal CLA+CD8+ T cell;
2) activated by drug exposure, IFNy/cytotoxic granule release;
3) fully evolved- keratinocyte cytolysis;
4) resolution-Treg recruitment amelioration but memory cells persist

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22
Q

LCV Px

A

Px:type 3 Immune complex( ab+drug related haptens) post capillary venules, activate complement cascade neutrophil chemotaxis ,profinflammatory cytokine/vasoactive amine

23
Q

AHEI

A

PxLCV, IC deposition, Ag trigger( Drug, Infx, Vaccine)

24
Q

UV PGx

A

: mast cell degranulation via calcium/energy dependent steps; IgE receptor cell Xlink allergen, histamine granules, leukotrienes

25
Q

PAN

A

Px:1.immune complex-mediated DIF IgM/c3; ↑titre antiphosphatidyl PT complex ab/LAC; 2.classical complement pathway( PT bind apoptotic endothelial cells–>Ab)

  1. local circulatory dysfunction(LL)
    infx: GAS(Ag-IC Walls)/hepB(systemic 30%)/c/TB; IBD, minocycline(p ANCA+)
26
Q

LV

A

Px: unknown, coagulation dysfunction(local/systemic)–>fibrin thrombi (supf dermal)

27
Q

EAC PathoGx:

A
  1. hypersensitivity infx-dermatophyte, candida , EBV, VZV poxvirus, HIV,
  2. drugs-piroxicam penicilin, malignancy-lymphoma, 3.leukemia, TNFa IL2 link,
28
Q

RPC

A

scratching, keratinocytes- AGE(advanced glycated end)product-receptor interaction, induction terminal ddx, upward migration-TEE,↑fibronectin, TGFb3

29
Q

EB pruriginosa:

A

DEB variant AD/AR, COL7A1 mutation-glycine subtitution, rudimentary af

30
Q

Px ALHE:

A

reactive>neoplastic, mural damage, trauma, AV shunting

31
Q

Cutaneous mets-

A

precocious, synchronous, metachronous
pGx:mechanical-1st capillary bed, regional extension
paget’s seed+soil-microenvironmental, organ-selective,
breast-ectoderm derivation, similar signalling pathway embryogenesis: Wnt, hedegehog,

32
Q

NLD

A

11% have DM, 0.3% DM have NLD

unknown, microangiopathic changes, anticollagen Ab

33
Q

NXG

A

Px: IgG monoclonal gammopathy, indirect/paraneoplastic effect(monocyte proliferation, lipid proliferation)

34
Q

RDD

A

Px: HHV6 related, autoimmune lymphoproliferative syndrome-Fas mediated apoptosis

35
Q

XD

A

Px: normolipemic, reactive histiocytosis

36
Q

xanthoma- ERUPTIVE

A

primary(see below), secondary( DM/cholestasis/PBC/hypothyroidism)
145 ET(eruptive- hypertriglyceriderma type 1/IV/V, lipoprotein lipase def, familial,
obesity, DM, retinoid),

37
Q

plane xanthoma

A

primary(see below), secondary( DM/cholestasis/PBC/hypothyroidism)
Play Bad(planar, dysBetalipoproteinemia) familial Criminals!(homozygous hyperC)
or plain normal(ipemic)-monoclonal gammopathy

38
Q

tendinous xanthoma

A

Terms & Conditions(tubero/tendinous, hypercholesterolemia-familial/dysbetalip)

39
Q

disseminated GA

A

PX:1. DTH unknown Ag( trauma, insect vite, TB, UVB, viral) 2. Th1-IFNY, macrophage inhibitory factor, elastic tissue injury 3. generalised: HLABw55,DM, HIV, malignancies- lymphoproliferative, solid

40
Q

OFG

A

PathoGx: cell mediated hypersensitivity food additives

41
Q

PNGD/IGD-

A

immune complex deposition , small vessel vasculitis , collagen degeneration , trauma(extensor)

42
Q

follicular mucinosis

A

mucin sebaceous gland/ORS, circulating IC, CMI, S aureus Ag;

43
Q

EDV-

A

AR, EVER1/2 Susceptibility locus endoplasmic reticulum genes HPV immunity

44
Q

SDRIFE

A

: blactam/iodinated radiocontrast, valacyclovir,type IV DTH, +PT ni, me, drug
CD4+ infiltrate ↑ CD62P

45
Q

hailey hailey,

A

AD, ATP2C1, golgi associated Ca2+ ATPase(hSPCA1), intracellular ca signalling,

46
Q

Reiter’s

A

Px: seroneg spondyloarthropathy, HLAB27, post GUT(CT) GIT(shigella salmonella yersinia)

47
Q

angiosarc

A

malignant endothelial ddx, stewart treves(lymphedema, VEGFR3); post RT(breastCA); MYC/FLT4 gene amplification; env( vinyl chloride, arsenic)

48
Q

KS

A

HHV8 infx(SI,saliva, blood, HIV coinfection); transcriptional reprogramming-lymphangigenic molecules also in multicentric castleman disease, 1 effusion lymphoma

49
Q

Melanoma-

A

1) BRAF/cKIT-acral mucosal/CDKN2A-p14,16/NRAS mutations,affect cellular signalling pathways,
2) UVA, fitzpatrick1/2,
3) GCMN,>50 dysplastic

50
Q

BCC:

A

Px-1.intermmittent UVR 2.genomic stability without precursors, PTCH, p53 3.locally invasive(no mets) stroma Xtalk dependent

51
Q

Gorlin:

A

AD PTCH inhibit SHH/SMO/gli/downstream target;

52
Q

EMPD

A

PGx: primary-toker cells origin; secondary: cutaneous extension underlying adnexal carcinoma(GCDFP15+, CD 20-)/visceral malignancy- CLR, urothelial, cervical, prostatic, ovarian, endometrial

53
Q

HIES

A

AD STAT3, AR DOCK8;

54
Q

WAS

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