Cutaneous Vascular Disease Flashcards Preview

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Flashcards in Cutaneous Vascular Disease Deck (45)
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1
Q

Raynauds

A

14% with Raynauds progressed to CTD - predictors are F, old age at onset, initial positive ANA, thickening of fingers

Pallor - cyanosis - rubor

High homocysteine in 1’ and 2’ raynauds
Pts with SS and RP have ⬆️endothelin which correlates with nailfold changes and advanced dz

2
Q

Raynauds

A

Cold, CTD, nervous system (CRPS), pianist/typists, endocrine, nipple variant with pain during lactation

Meds- beta blockers (incl eye drops), IFN, bleomycin, ergot, CSA, vinyl chloride, cocaine

3
Q

Raynauds dz

A

Usually BL, gangrene in <1%

Tx CCB - sildenafil, SSRIs, losartan (angiotensin II R antagonist), bosentin (endothelin R antagonist), iloprost, Botox

4
Q

Erythromelalgia

A

Primarily feet
Min to days, triggered by exercise or heat
Romiplostin may case (induced thrombopoeisis), also isopropyl alcohol and mushroom (clitocybe)
Tx - cold, ASA, amytryptaline 1% in ketamine 0.5% gel
Neurological sequale like neuropathy, small fiber neuropathy
AD form, SCN9A encodes a na channel

5
Q

Red ear syndrome

A

Relapse of redness/burning, affects both ears but usually one at a time
More common in winter, precipitatited by touching and exposure to warmth
Associations- trigeminal or glossopharyngeal do, lupus, migraines
Tx - tcas
Lobe involved vs Relapsing polychomdritis

6
Q

Med induced livedo

A

Amantadine, quinidine, quinine, mino, gemcitabine, heparin, IFN, bismuth

Parkinson’s, moya moya, factor 5 leiden, oxalosis, graves (with anticardiolipin ab), TB, atrial myxoma, syphilis

Pt with SLE are apt to have more severe dz if livedo

7
Q

Vasculopathy

A

Etiology is thrombosis (vs inflammation of vessels)

8
Q

Livedoid vasculopathy evaluation

A

Anticardiolipin ab, lupus anticoagulant, factor v Leiden, protein c and s, antithrombin III, prothrombin, cryoproteins, homocysteine

Mononeuropathy multiplex associated

9
Q

Mixed cryoglobulinemia

A

Hcv in >90%

10
Q

Waldenstroms hyperglobulinemic purpura

A

Episodic showers of petechiae of the legs
Peak incr of IgG on spep
RF pos usually, 80% Ro/La pos
Sjogrens, RA, adverse fetal outcome, hep c in men
LCV in 1/3 - higher risk of aurticular inv
Benign chronic course

WM - proliferation of B cells, elevated igM, gum bleeding, epistaxis, purpura, old men, urticaria, disseminated xanthoma, amyloid, deck chair sign
2 types of skin lesions - Igm aggregates (translucent papules, subepi blister, pas pos but neg Congo red fissured pink deposits) and infiltrate of neoplasticism lymphocytes

11
Q

Drug induced purpura

A

NSAIDS, allopurinol, cephalosporins, thiazides, gold, sulfa, hydralazine, phenytoin, quinidine, PCN, Emla in 30 min (toxic effect on cap endothelium), Tylenol use in mono, pseudoephedrine (ppd like rash)

Benadryl + pyrithyldione cause purpuric mottling with necrosis

12
Q

Purpuric contact derm

A

Rubber and textile dyes

13
Q

Superficial migratory thrombophlebitis

A

Linear painful red induration
Anything that causes hypercoag
Mondors = breast

14
Q

Postcardiotomy syndrome

A

2-3 weeks after

Fever, pleuritis, pericarditis, arthritis, petechiae

15
Q

Eculizumab

A

C5 ab

Used in PNH

16
Q

Achenbach syndrome

A

Rapid onset and resolution paroxysmal hand hematoma

17
Q

Painful bruising sundrome (Gardner diamond syndrome, psychogenic purpura)

A

Localized purpura in young women with personality do

Painful bruises on ext > trunk or face resolves in 5-8 d

18
Q

Pigmented purpuric contact dermatitis

A

Nickel, fragrance, disperse blue dye

Purpuric agave derm from agave tropical plant

19
Q

LCV recurrence rate

A

10%

20
Q

LCV

A

Immune complex mediated

21
Q

Vasculitis work up

A

CBC, Ua, aso, hep b and c, ana, rf screening

Consider spep, cryoglobulins, ancas

22
Q

LCV

A

Rest, elevation, NSAIDS - steroids or colchicine - dapsone

Concentrated along wallaces line

23
Q

UV MC systemic sx

A

Arthritis 50%
May also have GI, obstructive pulmonary dz, glomerulonephritis

Rare hypocomlementemic form with Jacquets arthropy and valvular heart dz

24
Q

Golfers/exercise related vasculitis

A

Not a true LCV

1/2 are on antithrombotic agents

25
Q

PAN

A

Necrotizing vasculitis of small and medium a. AT branching pt
2 forms - benign cutaneous and systemic
10 dx criteria - livedo racemosa, polymorphonuclear arteritis, leg pain/ myopathy/weakness, neuropathy (foot drop), hep b pos, wt loss > 4kg, testified pain, DBP > 90, elevated bun/cr, arteriographic abnormality
Men age 50
Cutaneous vasculitis identical to PAN seen in IVDU
5-7% associated with hep b
Skin involved in 50% of pt with systemic form, MC subcutaneous nodules along bld v.
Livedo + subq nodules = PAN
Hep b associated PAN has skin inv in 30%
P anca is predominant (also MPA) - hep b rarely panca pos
Death due to renal, cardiac or GI
Cyclophosphamide +- steroids, IFN if hep

26
Q

Cutaneous PAN

A

10% of PAN pts with limited sys and only skin
Neuropathy in 20%
MC childhood pattern
Hep b, c, crohns, takayasu arteritis, RP, strep, tb, minocycline
Asa and NSAIDS first line

27
Q

Antiproteinase 3 ab relatively specific for

A

Wegeners and MPA

28
Q

Both p anca and c anca

A

Think drug induced

Thrombophlebitis in 8% of anca vasculitis

29
Q

Microscopic polyangitis

A

Most with constitutional sx
80-90% with segmental necrotizing and and crescenteric glomerulonephritis
Pulmonary capillaritis complicated by hemorrhage
Vasculitis neuropathy and eye dz
Anca pos in 70%, MC p anca
Separated from PAN by glomerulo, pulmonary sx, and abscense of HTN and microanyeurisms

30
Q

MC initial presentation of wegeners

A

Rhinnorhea, sinusitis, nasal mucosal ulceration, nodules in nose, larynx, trachea or bronchi

Granulomas may occur in ear or mouth
45% with skin
Nodules on ext ext, may ulcerated - livedo rare
Focal necrotizing glomerulonephritis in 85%
C anca pos (anti pr-3, cytoplasmic)
Bactrim decreases relapse rate due to decreased staph carriage, a known trigger

31
Q

Cocaine associated vasculitis

A

Cutaneous lesions resemble LCV but ecchymosis and necrosis is MC
*earlobe predilection
Agranulocytosis is seen
cANCA, reacts with human neutrophil elastase (vs wegeners and microscopic polyangitis which are hne-anca negative)
Eradication of staph carriage if prominent nasal findings

32
Q

Giant cell arteritis/temporal arteritis

A

Whites
UL HA, ear and parotid pain, mastication induced pain
Fever, anemia, high esr, polymyalgia rheumatica (all day stiffness/pain)
Blindness is most severe complication
Actinic granuloma may develop - actinically induced?
Affected a. May be pulsating or necrosis may occur
2 cm a. Biopsy
TNF polymorphisms may show genetic predisposition
Steroid responsive

33
Q

Takayasu arteritis

A

F, 2nd or 3rd decade

EN and PG like lesions (UE)

34
Q

Thrombophlebitis obliterans

A

Instep and foot claudication

35
Q

Diffuse dermal angiomatosis

A

Breast, next to scars from breast reduction
May be telangectasias, purpura or ulceration
Nipple spared
RF for hypercoag
Chronic ischemia leads to vascular proliferation

36
Q

Early finding in KD (first week)

A

Desquamating perianal eruption

In KD may also see pincer nails, pseudo intestinal obstruction, facial n. Paralysis, PSO 10-20 d later (superantigens trigger PSO)
CAA in 20-25% of untreated kids, 3-5% of treated (after day 10 with elevated plt)
Increased superantigens in stool
Kids of parents who had KD have twofold risk of developing
Linked to CAMK2D

37
Q

MC sign in HHT

A

Epistaxis, GI bleed is the presenting sign in 25%
Worsening epistaxis may herald high output heart failure
Pregnancy may exacerbate
Dental prophylaxis due to risk of cerebral abscess
Telangectasias first appear on the undersurface of the tongue/mouth floor at puberty
HHT with juvenile polyposis - MADH4
Increased VEGF levels in HHT
OCPs, topical tranexamic acid for epistaxis
Thalidomide blocks VEGF and reduces GI bleeding and transfusion dependence

38
Q

MCC of lynphedema in US

A

Postsurgical

39
Q

Lipedema

A

BL symmetric LE enlargement due to subcutaneous fat deposition
Feet are spared, buttocks to ankles affected
No response to compression
Skin fold at the base of 2nd toe is too thick to pinch in lymphedema but nl in lipedema (stemmers sign)

40
Q

Nonne milroy

A

Painless edema, pitting (vs lymphedema that is usually non)
Not associated with other d/o
MC UL

41
Q

Emberger syndrome

A

Primary lymphedema associated with myelodysplasia

Skeletal, deafness, multiple warts

42
Q

WILD

A

Warts, immunodeficiency, lymphedema, anogenital dysplasia

Th reduced

43
Q

Hypotrichosis-telangectasia-lymphedema syndrome

A

Vascular dilations on palms/soles

Sox18

44
Q

Secondary lymphedema

A

HL and KS (need chemo bc lymphatics inv)
Postinflammatory lymphedema from strep cellulitis in post op CABG patients
Secretan syndrome - facticial lymphedema from from blunt tx of dorsum hand

Podoconiosis- mossy foot, noninfectious lymphedema, tropical regions of Africa, S. America and India from walking barefoot in soil of volcanic origin

Hand edema persistent in divers from suit constriction

45
Q

Marshall white syndrome and bier spots

A

Mottling from BP cuff