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

clinical features,criteria Flashcards by wan lin teo

amyloidoses:macular ddx:

notalgia paresthetica; ashy; drug induced,atrophic LP

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lichen amyloidoses ddx:

papular mucinosis, pemphigoid nodularis, EB pruriginosa

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amyloidoses Px:

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

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nodular amyloidoses features

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

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nodular amyloidoses Px

-local production Ig LC, plasma cell derived

aw Sjogrens.

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primary systemic amyloidoses Px:

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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primary systemic amyloidoses workup

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

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specific mucocutaneous:primary systemic amyloidoses

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

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primary systemic amyloidoses systemic: cx

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

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CRP-Fx

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

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CRP-Px:

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

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CRP patho

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

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CRP Rx:

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

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prurigo pigmentosa: Fx

Boer’s criteria

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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prurigo pigmentosa: Rx, prognosis

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

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prurigo pigmentosa:histo

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

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prurigo pigmentosa Px

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

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photosensitising drugs

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

EPP-Fx

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

EPP- Px

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reduced ferrochelatase–>↑protoporphyrin, AD,

pellagra

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

pellagra Px

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

pellagra Rx

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

HSP-

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

HSP-Prognosis:

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

AHEI

HUV:

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

Ax HUVS

(GN, COPD, fatal)

cryopyrin syndromes-muckle wells

deafness, renal amyloid, 1L1Rantagonist anakinra;

schnitzler

monoclonal IgM gammopathy, fever, LN, hyperostosis)

Still’s -

high fevers, joint pain,

Wells -

urticaria-pseudocellulitic , greenish, flame figures tissue eosinophilia

VM description

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

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

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)

Vascular tumor cx

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

CMTC-

unilateral/generalised; atrophic depressed scarred persist(warming)

VM:

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

1.Supf red

CM/CVM/, Abortive IH/dormant AVM

deep

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

blue

VM,AVM, dermal melanocytosis,

Vasx invx

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(#)

VM: rx

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

CM: rx

CM: PDL; SWS- MDT

LM: rx

LM: Sx, CO2,scleroRx

clinical features,criteria Flashcards

(44 cards)