Cutaneous Vascular Disease

Question 1

Raynauds

Answer 1

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

Question 2

Raynauds

Answer 2

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

Question 3

Raynauds dz

Answer 3

Usually BL, gangrene in <1%

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

Question 4

Erythromelalgia

Answer 4

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

Question 5

Red ear syndrome

Answer 5

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

Question 6

Med induced livedo

Answer 6

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

Question 7

Vasculopathy

Answer 7

Etiology is thrombosis (vs inflammation of vessels)

Question 8

Livedoid vasculopathy evaluation

Answer 8

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

Mononeuropathy multiplex associated

Question 9

Mixed cryoglobulinemia

Answer 9

Hcv in >90%

Question 10

Waldenstroms hyperglobulinemic purpura

Answer 10

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

Question 11

Drug induced purpura

Answer 11

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

Question 12

Purpuric contact derm

Answer 12

Rubber and textile dyes

Question 13

Superficial migratory thrombophlebitis

Answer 13

Linear painful red induration
Anything that causes hypercoag
Mondors = breast

Question 14

Postcardiotomy syndrome

Answer 14

2-3 weeks after

Fever, pleuritis, pericarditis, arthritis, petechiae

Question 15

Eculizumab

Answer 15

C5 ab

Used in PNH

Question 16

Achenbach syndrome

Answer 16

Rapid onset and resolution paroxysmal hand hematoma

Question 17

Painful bruising sundrome (Gardner diamond syndrome, psychogenic purpura)

Answer 17

Localized purpura in young women with personality do

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

Question 18

Pigmented purpuric contact dermatitis

Answer 18

Nickel, fragrance, disperse blue dye

Purpuric agave derm from agave tropical plant

Question 19

LCV recurrence rate

Answer 19

10%

Question 20

LCV

Answer 20

Immune complex mediated

Question 21

Vasculitis work up

Answer 21

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

Consider spep, cryoglobulins, ancas

Question 22

LCV

Answer 22

Rest, elevation, NSAIDS - steroids or colchicine - dapsone

Concentrated along wallaces line

Question 23

UV MC systemic sx

Answer 23

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

Rare hypocomlementemic form with Jacquets arthropy and valvular heart dz

Question 24

Golfers/exercise related vasculitis

Answer 24

Not a true LCV

1/2 are on antithrombotic agents

Question 25

PAN

Answer 25

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

Question 26

Cutaneous PAN

Answer 26

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

Question 27

Antiproteinase 3 ab relatively specific for

Answer 27

Wegeners and MPA

Question 28

Both p anca and c anca

Answer 28

Think drug induced Thrombophlebitis in 8% of anca vasculitis

Question 29

Microscopic polyangitis

Answer 29

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

Question 30

MC initial presentation of wegeners

Answer 30

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

Question 31

Cocaine associated vasculitis

Answer 31

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

Question 32

Giant cell arteritis/temporal arteritis

Answer 32

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

Question 33

Takayasu arteritis

Answer 33

F, 2nd or 3rd decade | EN and PG like lesions (UE)

Question 34

Thrombophlebitis obliterans

Answer 34

Instep and foot claudication

Question 35

Diffuse dermal angiomatosis

Answer 35

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

Question 36

Early finding in KD (first week)

Answer 36

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

Question 37

MC sign in HHT

Answer 37

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

Question 38

MCC of lynphedema in US

Answer 38

Postsurgical

Question 39

Lipedema

Answer 39

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)

Question 40

Nonne milroy

Answer 40

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

Question 41

Emberger syndrome

Answer 41

Primary lymphedema associated with myelodysplasia | Skeletal, deafness, multiple warts

Question 42

WILD

Answer 42

Warts, immunodeficiency, lymphedema, anogenital dysplasia | Th reduced

Question 43

Hypotrichosis-telangectasia-lymphedema syndrome

Answer 43

Vascular dilations on palms/soles | Sox18

Question 44

Secondary lymphedema

Answer 44

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

Question 45

Marshall white syndrome and bier spots

Answer 45

Mottling from BP cuff