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Exit Viva > clinical features,criteria > Flashcards

clinical features,criteria Flashcards

1
Q

amyloidoses:macular ddx:

A

notalgia paresthetica; ashy; drug induced,atrophic LP

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2
Q

lichen amyloidoses ddx:

A

papular mucinosis, pemphigoid nodularis, EB pruriginosa

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3
Q

amyloidoses Px:

A
  1. amyloid=fibril precursor/protein(AL, AA..)+amyloid P,GAG, apoE
  2. friction, genetic, EBV, Keratinocyte derived precursor protein
  3. fibrillar theory: tonofilament degeneration ; vs secretory theory
  4. Genetic: men2a(sipple) ripples RET, OSMRb; IL31RA; PC+PPK
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4
Q

nodular amyloidoses features

A

waxy nodules/plaques, surveillance(7%systemic), paraproteinemia 40%;

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5
Q

nodular amyloidoses Px

A

-local production Ig LC, plasma cell derived

aw Sjogrens.

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6
Q

primary systemic amyloidoses Px:

A

plasma cell dyscrasia(missing myeloma criteria-↑ca;osteolytic;AL fibrils, lamda IgLC ;
secondary; HD-assoc; genetic: periodic fevers: muckle wells(deafness, urticaria, AA nephropathy); FMF-pyrin, erysipeloid

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7
Q

primary systemic amyloidoses workup

A

Bx, directed lab(UECr/UA/LFT; Echo/BNP;nerve condx) surveillance-serum electrophoresis

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8
Q

specific mucocutaneous:primary systemic amyloidoses

A

oral(enlarged tongue, hemorrhagic yellow-brown papules/blisters);
periorbital/ pinch petechiae/purpura(minor trauma,cough)
yellow-flesh waxy papules/nodules

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9
Q

primary systemic amyloidoses systemic: cx

A

CTS, postural BP, nephrotic syndrome; CCF(restrictive CMP); gastroparesis; hepatomegaly;

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10
Q

CRP-Fx

A

asymptomatic intermamary/scapular/epigastric,hyperkeratotic verrucous, confluent centrally, reticulate peripherally;

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11
Q

CRP-Px:

A 
insulin resistance- obesity/DM/endocrine vs
 keratinisation disorder(retinoid response), vs 
abnormal host response malasezia
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12
Q

CRP patho

A

hyperkeratosis, acanthosis, papillomatosis, club shaped bulbous rete ridge dirty feet,

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13
Q

CRP Rx:

A

po minocycline 50% effective, isotret/acitretin/salicyclic acid, azithro

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14
Q

prurigo pigmentosa: Fx

Boer’s criteria

A 
recurrent crops pruritic papulovesicles, neck/back/chest, heal reticulated hyperpigmentation
4 st: 
1.early urticarial 
2. papulovesicular 
3.crusted 
4.reticulated hyperpigmentation
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15
Q

prurigo pigmentosa: Rx, prognosis

A

minocycline(antiinflammatory,neutrophil chemotaxis); dapsone, doxy,isotret/NBUVB,hydroquinone/azelaic;
Px: good but relapsing

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16
Q

prurigo pigmentosa:histo

A

(stage dependent)- spongiosis, vesiculatoin, exocytosis, perivascular infiltration

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17
Q

prurigo pigmentosa Px

A

Px-young japanese spring/summer,
ketosis(DM, ketonuria, fasting,pregnancy),
frictional/ACD-bismuth,para-amino(textile); Hpylori
Sjogren/AoSD;

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18
Q

photosensitising drugs

A

abx: FQ,tetra,sulphonamide/antifungal:griseofulvin; antimalarial; antipsychotic: chlorpromazine, phenothiazine; Cardiac: amiodarone, CCB; Diuretics: thiazide, frusemide; Hypoglycemics-sulphonylureaNSAIDs)

19
Q

EPP-Fx

A

depressed linear,waxy induration knuckles-gallstone, urine porphrin normal, ;

20
Q

EPP- Px

A

reduced ferrochelatase–>↑protoporphyrin, AD,

21
Q

pellagra

A

well demarcated symmetric scaly, hyperpigmented, shellac , casal’s necklace,

22
Q

pellagra Px

A

B3/niacin/tryptophan deficiency- diarrhea dermatitis dementia; primary/secondary- alcoholism, CLD, chronic diarrhea, UC, drug ING/AZA –

23
Q

pellagra Rx

A

Rx PO/IV niacin/nicotinamide, sun protect, emollient

24
Q

HSP-

A

palpable purpura + 1 ( igA, Abdominal pain, Arthralgia, hematuria/proteinuria)

25
Q

HSP-Prognosis:

A

CRF 5-15%( ped); 50%(adult); poor factors(ped: >1m, relapsing; adult: age, macroscopic hematuria,proteinuria> 1 g/24H, baseline renal insuf, IgM/above waist NOT prognostic)
FU- BP UFEME yearly vs refer renal

26
Q

AHEI

A
27
Q

HUV:

A

24H pain LCV, uveitis, conjunctivitis, arthralgia, abdo pain

28
Q

Ax HUVS

A

(GN, COPD, fatal)

29
Q

cryopyrin syndromes-muckle wells

A

deafness, renal amyloid, 1L1Rantagonist anakinra;

30
Q

schnitzler

A

monoclonal IgM gammopathy, fever, LN, hyperostosis)

31
Q

Still’s -

A

high fevers, joint pain,

32
Q

Wells -

A

urticaria-pseudocellulitic , greenish, flame figures tissue eosinophilia

33
Q

VM description

A

Skin: Patch/plaque; red/blue; compressible; dependent filling ; P;warmth/thrill/bruitSegmental/geographic; unilateral/bilateral/generalised

34
Q

special:1.V1- SWS; 2. lumbosacral
3.twin-spotting 4. LL
5;centrofacial-
6.nape
multiple

A

special:1.V1- SWS; 2. lumbosacral occult
spinal dysphraphism 3.twin-spotting LL CMTC(CM);acroangiodermatitis(AVM),KTS(CVLM),PW(AVF);centrofacial-bonnet dechaume blanche(AVM), nape Cobb-intraspinal/vertebral AVM;
multiple:PTEN/RASA1(AVM)

35
Q

Vascular tumor cx

A

angiokeratoma, microcystic LM, twin-spotting
Tissue/bone hypertrophy/hypoplasia
Cx: ulceronecrosis, cellulitis, DVT/PE/LIC-↑D-dimer/↓fibrinogen

36
Q

CMTC-

A

unilateral/generalised; atrophic depressed scarred persist(warming)

37
Q

VM:

A

VM: FCMVM(AD TEK , TIE2) BRBNS,GVM(GLMN), Mafucci(+enchondroma, neuropth)

38
Q

1.Supf red

A

CM/CVM/, Abortive IH/dormant AVM

39
Q

deep

A

deep IH, KHE, A/VM, LM, LVM

40
Q

blue

A

VM,AVM, dermal melanocytosis,

41
Q

Vasx invx

A

Invx
T2 weighted/gad contrast MRI - ↑ signal/extent+depth/thrombi+phleboliths
Cephalic(bony*, brain MRI-CVM)/limb(joint) truncal(visceral-pleura GI, LS);
LIC/bleeding: FBC(Hb, plt)PT/PTT(normal); D-dimer(↑)/fibrinogen (↓)
Site-spec Cx: parapharyngeal(ENT); cheek/tongue(ENT, dental, PRAS); orbital(PRAS); Limb(#)

42
Q

VM: rx

A

VM: 5mg/kg/d aspirin ; LMWH( LIC + painful thrombosis; pregnancy+periop); avoid trauma/contactsport; scleroRx*/excision/RFA/endovascular ablation

43
Q

CM: rx

A

CM: PDL; SWS- MDT

44
Q

LM: rx

A

LM: Sx, CO2,scleroRx

Exit Viva (8 decks)

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