Chapter 148 - Vascular and Lymphatic Cutaneous Diseases Flashcards
The most classic symptom of PAOD
Intermittent claudication
Acute limb ischemia presents with 5Ps
Pain, persistent at rest Pallor Pulselessness Paresthesia Paralysis \+ Poikilothermia
Hallmark noncutaneous findings in PAOD
Decreased or absent pulses distal to the stenotic arterial segment
Collateral circulation is deemed adequate if elevation if the limb at ____ degree angle for _____ minutes should not produce ____
45 degrees, 2 mins, pallor
Collateral circulation is deemed inadequate if the patient resumes sitting position and time for filling of foot veins and flushing of feet is measured beyond _____
30 seconds
Normal: within 20 seconds
Major direct complication related to PAOD
Limb loss
Risk factors for PAOD
Diabetes mellitus HPN Hyperlipidemia Smoking Family history of vascular disease Obesity
Most significant risk factors for PAOD with doubling of relative risk
Diabetes mellitus
Smoking
Under resting conditions, normal blood flow to extremity muscle groups averages _____ ml/min
300 to 400
The recommended diagnostic test for PAOD
Ankle-brachial index
Normal ABI is ____
Borderline ABI is _____
Abnormal ABI is ____
1 to 1.4
0.91-0.99
Less than or equal to 0.90
Differentiating points of arterial ulcers vs ulcers secondary to diabetic neuropathy
- very tender
- do not have preference for pressure points on the foot
- lack a surrounding callus
It causes intermittent claudication, ulcers, gangrene in young (less than 45 years old) smokers, associated with migratory superficial thrombophlebitis and vasospasm.
Thromboangiitis obliterans (medium - sized, small arteries)
Most common artery affected in PAOD
Distal superficial femoral artery
Tibial pulses disappear with dorsi(plantar)flexion of foot and full extension of knee.
Popliteal artery occlusion
In popliteal artery occlusion, pain is more severe with walking than running.
True or False
True
Leg pain occurs in erect position and affected by changes in pressure
Neurogenic claudication (Pseudoclaudication)
Treatment for neurogenic claudication
Lean forward against a solid surface or sitting
Etiologic factors of neurogenic claudication
- Prolapsed intervertebral disks
- Congenital stenosis
- Hypertrophic bong ridging of the spinal canal
Patients with diabetes tend to have a progressive disease and ___ fold risk for amputation
4
5 year mortality rate of PAOD is ____
30% secondary to cardiovascular causes
Associated with greater risk of CV mortality
Lower ABI value
For patients with intermittent claudication, the treatment of choice is
Exercise regimen
30- 60 mins/ day, 3 or more times in a week
Comparison of leg ulcer types
- Below the knee; commonly the medial malleolar area
- Tips of toes, lateral heels, lower calves
- Heels, toes, shins, pressure points
Venous
Arterial
Neuropathic
Comparison of Different Leg ulcer types
- atrophic, dry, shiny
- Smooth
- Hyperpigmented
- With edema
- With anhidrosis
- With sensory deficiency
- With stasis dermatitis
- With varicosities
Arterial Neuropathic Venous Venous Neuropathic Neuropathic Venous Venous
Comparison of Different Leg Ulcer types
- Painless ulcer
- Very tender ulcer
- With irregular borders
- Pale base
- Red base
- Surrounding callus
- Gangrene
- Superimposed infection
Neuropathic Arterial Both arterial and venous Arterial Venous Neuropathic Arterial Arterial
First line therapy for PAOD (3)
Exercise program
Cilostazol (Pletaal)
Pentoxifylline (Trental)
Risk reduction for PAOD (5)
Smoking cessation Statin therapy (LDL < 100mg/dl) Aspirin Glycemic control Antihypertensive therapy
Conventional vasodilators have immense therapeutic value in PAOD
True or False
False
Phosphodiesterase inhibitor, with antiplatelet and vasodilatory properties, is an effective treatment to improve symtpoms and increase walking distance
Cilostazol
Highly effective for aortoiliac disease
Indicated for moderate, or lifestyle-limiting claudication
Endovascular intervention with angioplasty or stenting
Angioplasty or stenting is limited by
High restenosis rates
Cilostazol improves maximal walking distance by ____ after ____ of therapy
40 to 60%
12 to 24 weeks
Side effects of Cilostazol
GI symptoms, headaches
Sympathectomy is of great value for intermittent claudication.
True or False
False, not of value
Pain medication is usually necessary for ____ months with digital gangrene
2 to 3 months
Most consistent adverse risk factor associated with the progression of disease
Continued tobacco smoking
Smoking ____ cigarettes per day can interfere with PAD treatments
1-2
Presents with blue or discolored toes, livedo racemosa, gangrene, necrosis, ulceration, and fissure
Atheromatous embolism
Major risk factor for development of atheromatous embolism
Atherosclerotic disease of thoracic or abdominal aorta
The risk facors for atheromatous embolism (4)
Coronary artery disease
PAOD
Abdominal aortic aneurysm
Older age
More common sites for atheromatous disease
Abdominal aorta
Iliac arteries
Often the presenting complaint of atheromatous embolism
Dermatologic manifestations related to digital or tissue ischemia
- cyanosis
- necrosis
- gangrene
- ulcerations
- fissures
Specific but insensitive finding of atheromatous emboli
Hollenhorst plaques (cholesterol embolus within the branching points of retinal arteries)
One of the ominous complications of atheromatous embolism associated with high mortality
Development of renal failure
Most common site in GI affected by atheromatous embolism
Colon
Most common finding of atheromatous embolism in noninvasive imaging techniques
Stenotic atherosclerotic disease
More definitive diagnostic modaity to assess thoracic aorta for plaque
Transesophageal echocardiography
Most sensitive test for atheromatous embolism
Muscle biopsy (>95%)
Definitive diagnosis of cholesterol embolization requires demonstration of
Cholesterol crystals (birefringent on polarized light)
Renal failure caused by atheromatous emboli usually develops over ____ after an angiographic procedure and shows partial recovery
Vs
Contrast-induced renal failure typically appears soon after testing and recovers within ____
1-4 weeks
3-5 days
Strong predictor of death in atheromatous embolism
End stage renal failure
Mainstays of therapy of atheromatous embolism (3)
Preventing further ischemic insult
Supportive care
Removal of atheromatous source
Aid in plaque stabilization and has clear benefits in prevention of cardiovascular ischemic events
Statins
Most common initial complaints in thromboangitis obliterans (Buerger disease) (3)
Claudication of foot or lower calf
Digital cyanosis or gangrene
Rest pain
Nailfold examination of thromboangiitis obliterans
Multiple dilated capillary loops
In Thromboangiitis obliterans, there is increased sensitivity to type ___(2)____ collagen
Type 1 and Type 3
Thromboangiitis obliterans is considered serologically negative.
True or False
True, meaning ESR/CRP are usually normal during active disease
Arteriographic findings of ____ are typical but not pathognomonic for Thromboangiitis obliterans
Corkscrew- shaped collaterals
First line therapy for thromboangiitis obliterans for improvement of bloodflow (2)
Smoking cessation
IV iloprost
Sympathectomy may help patients with a vasospastic component in Thromboangiitis obliterans.
True or False
True
Only strategy proven to prevent progression of Thromboangiitis obliterans
Absolute discontinuation of tobacco use
Differentiating livedo reticularis vs livedo racemosa
- Asymmetric, irreversible, and broken
- Aysmptomatic
- Fishnet like
- Aggravated by cold, relieved by rewarming
- May be caused by amantadine
- With purpura, nodules, macules, ulcerations, and atrophie blanche
1 and 6 racemosa
2 to 5 reticularis
Testing should be done in ALL patients with livedo racemosa
APAS
25% - primary anti phospholipid syndrome
70% - SLE - associated anti phospholipid syndrome
Pathophysiologically, livedo arises from (2)
Deoxygenation
Venodilation
Interaction of APAS antibodies and endothelial cells lead to increased production of procoagulant substances (3)
Tissue factor
Plasminogen activator-inhibitor 1
Endothelin
Biopsy findings of livedo racemosa indicate the highly variable etiology
- Cholesterol clefts
- Calcification of vessels and interstitium
- Noninflammatory arterial obstruction
- Extensive fibrin deposition and microthrombi
- Fibrinoid necrosis
- Atheroembolic disease
- Calciphylaxis
- Sneddon syndrome
- Livedoid vasculopathy
- Polyarteritis nodosa
Relapsing condition marked by recurrent painful ulcerations and subsequent atrophie blanche type scars
Livedoid vasculopathy
Treatment for livedo racemosa and APAS
Anticoagulation
Intermittent intense burning pain with marked erythema typically of lower extremity
Erythromelalgia (Erythermalgia)
Effective for secondary erythromelalgia
Aspirin
Drugs that may precipitate erythromelalgia
Pergolide
Bromocriptine
Calcium channel blockers (Nifedipine, Felodipine, Nicardipine)
Mutation in ___, gene encoding voltage-gated sodium channels of sensory nerves in inherited eryhtromelalgia
SCN9A
Definitive diagnosis of erythromelalgia
Provoking a flare with use of exercise or immersion of affected area in hot water for 10 minutes
Risk factors for chronic venous disease (8)
Heredity Age Female sex Obesity Pregnancy Prolonged standing Phlebitis Previous leg injury Greater height
Symptoms of peripheral Venous Disease (5)
Leg fullness Aching discomfort Heaviness Nocturnal leg cramps Bursting pain on standing
Very early sign of peripheral venous disease
Tenderness to palpation
Early signs of peripheral venous disease (4)
Edema
hyperpigmentation
Stasis dermatitis
Varicose veins
Late signs of peripheral venous disease (5)
Venous ulcers Atrophie blanche Lipodermatosclerosis Acroangiodermatitis of Mali Postphlebitic syndrome
Earliest finding of chronic venous disease
Perimalleolar edema
Region where microangiopathy is most intense
Medial supramalleolar region
Allergic contact dermatitis occur in ____ % of chronic venous disease
80%
Fully established lesions consist of irregular, smooth, atrophic stellate plaques with surrounding hyperpigmentation and telangiectasia
Atrophie blanche
Purple macules, nodules, or verrucous plaques on dorsal feet and toes of patients with longstanding venous insufficiency
Acroangiodermatitis (pseudo Kaposi sarcoma, congenital dysplastic angiopathy, AV malformation with angiodermatitis)
Due to loss of lymphatic drainage from the lower leg with verrucous changes and cutaneous hypertrophy
Elephantiasis nostras
Severity of venous disease is influenced by the number and distribution of incompetent valves.
True or False
True
The most common cause of venous valvular failure
Thrombosis
Calf muscle pump failure after DVT is referred to as
Postphlebitic syndrome
Used to document valvular incompetence and to evaluate patients for possible sclerotherapy or surgery
Duplex Doppler ultrasonography
Loss of valvular function is reversible
True or False
False
Cornerstone of venous ulcer managemenr
Duke boot : Unna boot + reproducible, graduated, inelastic compression to counter the outflow from perforator incompetence
Mainstay of treatment for all clinical manifestations of chronic venous insufficiency
Mechanical therapy
Herbal remedy safe and effective as short term treatment for leg pain and swelling
Escin 50 mg BID
Aesculus hippocastanum
The only proven method to reduce the risk of postthrombotic syndrome
Elastic compression stockings
Essential for clearing of extravascular fluids and debris
Lymphatics
Genetic defects causing lymphedema are the ff:
VEGFR3
FOXC2
Key event during maturation of lymphatic vasculature
Valve formation
The most common cause of secondary lymphedema worldwide is
Parasitic infection
Cooccurrence of primary lymphedema and myelodysplasia/acute myeloid leukemia
Emberger syndrome
Emberger syndrome is an ___ condition caused by mutations in ____
Autosomal dominant
GATA2
Crucial for development and maintenance of lymphatic vessel valves and hematopoietic stem cell development during both embryogenesis and adulthood
GATA 2
Congenital primary lymphedema that leads to disability and disfiguring swelling of lower extremities
Hereditary lymphedema IA
Nonne-Milroy lymphedema
Milroy disease leads to a more severe phenotype in females leading to hydrops fetalis and fetal death.
True or False
False, males
Inability to pinch a fold of skin between second and third toe on dorsum of foot as seen in Milroy disease
Kaposi Stemmer sign
Presence of bilateral lower limb asymmetric swelling, ‘ski jump’ dysplasia, skin papillomatosis (second toe), hypoproteinemia
Milroy disease
Presence of unilateral or bilateral lymphedema of lower extremities with low CD4:CD8 ratio, immune dysfunction, sensorineural deafness, and genital lymphedema
Emberger syndrome
Milroy syndrome is an ____ condition caused by mutation of ____
AD, VEGFR3 gene aka FLT4 gene
Skin biopsy of affected extremity in Milroy disease
Abdundant nonfunctional lymphatics by fluorescence microlymphangiography
Presence of lymphedema during puberty with double row of eyelashes
Lymphedema - distichiasis
Lymphedema- distichiasis is an ___ condition with ____ mutation
AD, FOXC2
Early signs of lymphedema (2)
Erysipelas and Pitting edema
Late signs of lymphedema (6)
Nonpitting edema Papillomatosis Secondary infection Ski jump upturned toenails Thickened woody skin Verrucous tissue over growth
25% of chronic edema was related to ___
Malignancy
Caused by chronic inoculation kf microparticles of silica through the soles of barefoot walkers
Podoconiosis or nonfilarial elephantiasis
Bilatera lymphedema does not rule out anatomic obstruction.
True or False
True
Association with postmastectomy lymphedema
Stewart Treves syndrome
MRI should reveal characteristic ‘___’ pattern of subQ tissue present in chronic lymphedema
Honeycomb
Syndrome caused by bilateral adipose deposition usually int he buttocks and lower extremities leading to enlargement that stops at malleoli ‘armchair sign’ common in overweight women
Lipedema of the leg
There is no cure for lymphedema
True or False
True
Mainstays of management of lymphedema (5)
Elevation Exercise Compression garments/devices Skin care aimed at preventing infection Manual lymphatic drainage via massage
Diuretics may worsen condition and should not be used as primary treatment for lymphedema
True or False
True
Lymphedema is common in the ff locations (3)
Face
Vulva
Penis
Blister like lesions full of fluid that are painful, pruritic, exudative
Lymphangiectasia
Favored treatment modality for superficial lymphedema
CO2 laser ablation
Pain upon walking that is relieved by rest
Intermittent claudication
Falsely elevated ABIs are greater than ___ and due to ____
1.4, noncompressible or heavily calcified vessels common in persons with DM or old age
To determine precise anatomic location and extent of disease in arteries
Magnetic resonance or Ct angiography
For definitive evaluation in patients about to undergo vascular surgery
Conventional Catheter based angiography
Cilostazol is contraindicated in those with
Congestive heart failure
Prevents progression of PAOD, as well as MI and stroke
Statins
Atheromatous embolism often occurs after ____
Invasive procedure
Impaired perfusion of skin and muscles due to obstruction of smaller vessels
Atheromatous embolism
Poorest outcome of atheromatous embolism
Suprarenal location
In thromboangiitis obliterans, there is increased prevalence of HLA
A9
A54
B5
2 Us approved agents for intermittent claudication of PAOD
Cilostazol and Pentoxifylline
Tender, cool blue toes with normal pulse
Blue toe syndrome (atheromatous embolism)
3 most common affected location of atheromatous embolism
Kidneys
Mesenteric location
GIT
Distal pulses (3) are absent in thromboangiitis obliterans while proximal pulses are preserved
Dorsalis pedis
Posterior tibial
Ulnar
First line therapy for symptom relief of thromboangiitis oblierans
Local wound care
Analgesics
Physiologic response to cold exposure as form of livedo reticularis
Cutis marmorata
Blood test to rule out polycythemia vera in erythromelagia
CBC
Erythromelalgia resembles ____ phase of Raynaud phenomenon
Hyperemic
Erythromelalgia commonly affects UE, face, and ears
True or False
False, Uncommonly
Given vasodilator therapy only for cosmetic appearance
Livedo reticularis
Livedo racemosa may be an independent factor for pregnancy loss in the absence of APAS
True or False
True
The ___ ABI ratio, the more severe the disease
Lower
Venous ulcers are ulcers occurring anywhere below the ___
Knee
Use of topical antifungal therapy in lymphedema is encouraged
True or False
True
Cause of deoxygenation within the vascular plexus
Decreased arteriolar perfusion
Physiologic arteriolar vasospasm produces irreversible livedo reticularis
True or False
False, reversible
Cutaneous findings of hypoperfusion
Dry skin
Hair loss
PAOD most commonly affects
Distal superficial femoral artery