22 - 148 - CUTANEOUS CHANGES IN ARTERIAL, VENOUS AND LYMPHATIC DYSFUNCTION Flashcards by Sharmaine Lozano

Disease in the distal superficial femoral artery causes:

a. Claudication in the thigh

b. Claudication in the calf muscle

c. Claudication in the buttocks

d. Claudication in the ankle

Page 2669. Because the disease is most common in the distal superficial femoral artery, patients most commonly present with claudication in the calf muscle area (the muscle group just distal to the arterial disease). When the disease affects the more proximal aortoiliac vessels, thigh and buttock muscle claudication predominates

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A 65-year-old male was seen in the OPD due to ulceration on his right foot associated with intermittent claudication. He is hypertensive and diabetic. ABI was 0.89. What is the interpretation of this result?

A. Normal

B. Abnormal

C. Borderline

D. Elevated

Page 2671. Normal ABI range is 1.00 to 1.40. A value less than or equal to 0.90 is considered abnormal, and values of 0.91 to 0.99 are defined as “borderline.” Of note, patients with heavily calcified or “noncompressible” vessels, most commonly persons with diabetes or of advanced age, may have falsely elevated ABIs (greater than 1.4) despite the presence of significant PAD.

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When the ABI is borderline or normal despite symptoms suggestive of claudication, what test is recommended?

A. Exercise ABI

B. Doppler waveform analysis

C. MRI

D. CT angiography

Page 2671. When the ABI is borderline or normal despite symptoms suggestive of claudication, an exercise ABI is recommended.

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First line therapy for the treatment of obstructive peripheral artery disease except

A. Cilostazol

B. Clopidogrel

C. Statin therapy

D. Exercise program

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The major risk factor for the development of atheromatous embolism is:

A. Atherosclerotic disease of the thoracic or

abdominal aorta

B. Coronary artery disease

C. Peripheral artery disease

D. Abdominal aortic aneurysm

Page 2676. The major risk factor for the development of atheromatous embolism is atherosclerotic disease of the thoracic or abdominal aorta.

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Arteriography findings, in particular findings of corkscrew-shaped collaterals, are typical, but not pathognomonic for the condition.

A. Peripheral artery disease

B. Atheromatous embolism

C. Thromboangiitis obliterans

D. Livedo Racemosa

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Characteristic of livedo racemosa except:

A. Symmetric, fishnet-like red or purple mottling with a pale conical core

B. May improve with warming but no complete resolution

C. Symptoms related to associated causative disorder

D. Surrounding skin shows purpura, nodules, macules, ulcerations, atrophie blanche– type scarring

Page 2682. Table 148-11. A is a characteristic of livedo reticularis. Livedo Racemosa - Asymmetric, irregular, and “broken”

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This is the cooccurrence of primary lymphedema and myelodysplasia/acute myeloid leukemia.

A. Emberger Syndrome

B. Milroy Syndrome

C. Lymphedema-Distichiasis

D. Elephanthiasis Nostras Verrucosa

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most classic symptom of PAD

intermittent claudication,

which is usually described as pain, fatigue, or tiredness in a defined muscle group distal to the diseased vascular segment upon walking that is relieved by rest

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peripheral arterial disease is most common in what artery

distal superficial femoral artery

patients most commonly present with claudication in the calf muscle area (the muscle group just distal to the arterial disease)

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Features of Acute limb ischemia

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hallmark noncutaneous finding in PAD

Decreased or absent pulses distal to the stenotic arterial segment

Bruits on auscultation over the diseased segment of vessel as a result of turbulent flow also may be present

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How do you test for collateral circulation

With the patient supine, elevation of the limb at a 45-degree angle for 2 minutes should not produce pallor.
Collateral circulation is deemed inadequate if the toes and feet become pale.
The patient then assumes a sitting position with the legs dependent, and the time for filling of the foot veins and flushing of the feet is measured.
The veins should fill within 20 seconds and the feet flush immediately in a warm environment.
When these times exceed 30 seconds, the collateral circulation is deemed inadequate, and the patient must be observed frequently for the development of rest pain, ulcers, or gangrene.

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Most significant atherosclerotic risk factors

DM and smoking

Atherosclerotic risk factors are similar to those identified for coronary artery disease and include diabetes mellitus, hypertension, hyperlipidemia, smoking, family history of vascular disease, and obesity.

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Under resting conditions, normal blood flow to extremity muscle groups

300 to 400 mL/min

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normal ABI

1.00 to 1.40

Of note, patients with heavily calcified or “noncompressible” vessels, most commonly persons with diabetes or of advanced age, may have falsely elevated ABIs (greater than 1.4) despite the presence of significant PAD

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Abnormal ABI

less than or equal to 0.90

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Borderline ABI

0.91 to 0.99

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How can you differentiate diabetic neuropathy ulcer from arterial limb ulcers

Diabetic neuropathic ulcers:
- may develop on the heel, toes, or shin in the presence of normal pulses. These painless (neurotrophic) ulcers are caused by repetitive trauma not noticed by the patient because of the peripheral neuropathy. With regard to the foot, ulcers often occur over pressure points with a surrounding callus.

Arterial limb ulcer
- exquisitely tender, do not have a preference for pressure points on the foot, and lack a surrounding callus.

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most feared consequence of PAD

severe limb-threatening ischemia leading to amputation

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Differential Diagnosis of Foot Ulcers

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Identify the type of ulcer

Arterial

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Identify the type of ulcer

Venous ulcer

Identify the type of ulcer

Neuropathic ulcer

Goal of medical management of PAD

measures to halt the progression of the disease as well as to alleviate the symptoms

measures to halt the progression of PAD

- cessation of smoking - optimization of risk factors, such as diabetes mellitus, hypertension, and hyperlipidemia

Treatment of choice for patients with symptoms of intermittent claudication

Exercise program

Patients with intermittent claudication often are instructed to exercise to the threshold of tolerable pain, briefly rest, and then exercise again for a total duration of ___________

30 to 60 minutes a day in excess of their normal activity, 3 or more times a week.

First line treatment for Obstructive Peripheral Arterial Disease

Target LDL for PAOD

< 100 mg/dL

Two agents have been approved for the indication of intermittent claudication in the United States

* **Cilostazol** - a phosphodiesterase inhibitor with antiplatelet and vasodilatory properties, is an effective treatment to improve symptoms and increase walking distance * **Pentoxifylline** - affects red cell deformability and blood viscosity and can be considered as second-line alternative therapy to cilostazol

phosphodiesterase inhibitor with antiplatelet and vasodilatory properties, is an effective treatment to improve symptoms and increase walking distance in patients with obstructive peripheral arterial disease

Cilostazol

affects red cell deformability and blood viscosity and can be considered as second-line alternative therapy to cilostazol

Pentoxifylline

highly effective for aortoiliac disease and is often indicated for moderate, or lifestyle-limiting claudication

Endovascular intervention with angioplasty or stenting

most consistent adverse risk factor associated with the progression of occlusive peripheral artery disease

Continued smoking

Target LDL in PAOD if with uncontrolled or multiple risk factors

<70 mg/dL ## Footnote hyperlipidemias should be treated with a target low-density lipoprotein of less than 100 mg/dL (or <70 mg/dL with uncontrolled or multiple risk factors)

embolization of small pieces of atheromatous debris from the more proximal arteries to the smaller distal arteries

Atheromatous embolism Synonyms include cholesterol embolism, atheroembolism, blue toe syndrome, and pseudovasculitis syndrome. ## Footnote often occurs after an invasive procedure

major risk factor for the development of atheromatous embolism

atherosclerotic disease of the thoracic or abdominal aorta

In **atheromatous embolism**, What do you call the Funduscopic examination finding revealing** cholesterol embolus **within branching points of the **retinal arteries**

Hollenhorst plaques It. is a specific but insensitive finding because most atheromatous emboli arise from a source distal to the aortic arch

Organ systems that can be involved in atheromatous embolism

1. Renal 2. CNS 3. Gastrointestinal; colon - most commonly affected

* rare, progressive, inflammatory and thrombotic disease that predominately affects **small- and medium-sized arteries **of the extremities * Predominantly affects **males**, at age 20 to 40 years. * Extremely strong association with **smoking**; often abates with smoking cessation. * Clinical manifestations include ischemia, cold sensitivity, or claudication of the foot, leg, or hand, which progress to ischemic ulcers, peripheral cyanosis, gangrene, or superficial thrombophlebitis

Thromboangiitis obliterans (TAO), also known as Buerger disease It is strongly associated with the use of tobacco products, and seen mostly in men between the ages of 20 and 40 years.

First-line therapy for Thromboangiitis obliterans

only strategy proven to prevent the progression of thromboangiitis obliterans

Absolute discontinuation of tobacco use

ischemic dermopathy characterized by a violaceous reticular or “net-like” mottling of the skin

Livedo

* primary disorder affecting young to middle-aged females that is benign. * The livid conical discoloration is **symmetric, reversible, and uniform** - **symmetric, fishnet-like red or purple mottling** surrounds a pallorous conical core * With the exception of a subjective feeling of coldness, the majority of patients are **asymptomatic** * This discoloration is **aggravated by cold exposure **and may completely **dissipate with warming**.

Livedo reticularis

* secondary disorder that is pathologic and permanent. * The livid conical discoloration is **asymmetric, irregular, irreversible, and “broken.”** * Although it may improve with warming, it does not resolve completely. * Attendant skin manifestations may include purpura, nodules, macules, ulcerations,

Livedo racemosa

It is the **most frequent dermatologic manifestation** in patients with **antiphospholipid syndrome, **present in 25% of patients with primary antiphospholipid syndrome, and in up to 70% of patients with systemic lupus erythematosus–associated antiphospholipid syndrome

Livedo Racemosa

Differentiate Livedo Reticularis and Livedo Racemosa

Symmetric and relatively uniform, conical rings of primary, reversible livedo reticularis (cutis marmorata).

Permanent and “broken” rings of secondary livedo racemosa on the buttocks and thighs in a woman who had cerebrovascular thrombosis. This is an example of Sneddon syndrome.

marker for predicting multisystem thrombosis in the antiphospholipid antibody syndrome

Livedo Racemosa

Intense, burning pain with marked erythema, typically of the lower extremity.

ERYTHROMELALGIA/ ERYTHERMALGIA

ERYTHROMELALGIA commonly affects what body part

Lowe extremities but may affect the upper extremities as well as other body parts, such as the face and ears.

Triggers of erythromelalgia

precipitated by activities that increase the body temperature, such as exercise, ambient temperature, or the use of heavy blankets at night. The episodes also can be triggered by dependency of the limb, wearing socks, or tight shoes, and sometimes with the ingestion of alcohol or spicy foods. There are also reports of episodes triggered by ingestion of some drugs, such as pergolide, bromocriptine, and calcium channel blockers (nifedipine, felodipine, and nicardipine)

Relieving factor of erythromelalgia

cold exposure (eg, standing on a cold floor) or immersion in ice water often relieves the flares

T/F: The peripheral pulses are usually normal or bounding in erythromelalgia

True

What gene mutation is associated with inherited erythromelalgia

SCN9A, the gene encoding voltagegated sodium channels of sensory nerves

Most common complication of chronic venous disease

Venous ulcers

Risk factors of chronic venous disease

genetics, obesity, female gender, pregnancy, occupations requiring prolonged standing, surgery, trauma, malignancies

Early signs of Peripheral venous disease

TVESH - Tenderness - Varicose Veins - Edema - Stasis Dermatitis - Hyperpigmentation

Late signs of Peripheral venous disease

LAU - lipodermatosclerosis - atrophie blanche - ulcers

Risk factors for chronic venous disease

heredity, age, female sex, obesity, pregnancy, prolonged standing, phlebitis, previous leg injury, and greater height

Common Signs and Symptoms of Peripheral Venous Disease

Earliest finding in chronic venous disease

**perimalleolar edema** that ascends up the leg, followed by soft tissue tenderness, even of normal appearing skin

Characteristics of stasis dermatitis

erythema, scaling, pruritus, erosions, crusting, and occasional vesicles and serous drainage may occur during any stage of chronic venous insufficiency

Area most commonly involved in stasis dermatitis

medial supramalleolar region where microangiopathy is most intense

pliable subcutaneous fat is gradually replaced by fibrosis, and the skin begins to feel firm and indurated.

lipodermatosclerosis (sclerosing panniculitis, hypodermatitis sclerodermiformis)

refers to skin overlying areas of fibrosis that often appears porcelain white and atrophic

Atrophie blanche

purple macules, nodules, or verrucous plaques on the dorsal feet and toes of patients with longstanding venous insufficiency and mimics Kaposi sarcoma clinically and histologically

**Acroangiodermatitis** (pseudo-Kaposi sarcoma, congenital dysplastic angiopathy, arteriovenous malformation with angiodermatitis)

Venous ulceration in chronic venous disease is classically located in what area?

Gaiter area “ Although ulceration is classically located in the gaiter area (Fig. 148-13), venous ulcers have been described as ulcers occurring anywhere below the knee. Venous ulcers are typically tender, shallow, irregular, and have a red-base (Figs. 148-13 and 148-14). They are usually located on the medial ankle or along the line of the long or short saphenous veins.”

Loss of lymphatic drainage from the lower leg may lead to verrucous changes and cutaneous hypertrophy called

elephantiasis nostras

most common cause of venous valvular failure

Thrombosis

nidus for venous thrombosis

Valve cusp when the thrombus is lysed by plasmin, valve function is often lost as well

Calf muscle pump failure after deep venous thrombosis is often referred to as the

postphlebitic syndrome

ankle-brachial index is reliable except in the presence of

calcified vessels, which are noncompressible and therefore a true systolic pressure cannot be measured.

Histologic signs of venous hypertension

hemosiderin deposition, lobular superficial and/or deep dermal neovascularization, and fibrosis of the dermis and subcutaneous tissue in later stages

Contraindications to compression therapy

arterial occlusive disease or abnormal ankle, brachial indexes

zinc-impregnated gauze wrap

Unna boot

self-adherent second layer (Coban, for example) over a zinc wrap

Duke boot

cornerstone of venous ulcer management

“Duke boot” This has proven to be easy to apply with reproducible, graduated, inelastic compression to counter the outflow from perforator incompetence, and is the cornerstone of venous ulcer management.

cornerstone of venous ulcer management

fadherent second layer (Coban, for example) over a zinc wrap was introduced as the “Duke boot”. This has proven to be easy to apply with reproducible, graduated, inelastic compression to counter the outflow from perforator incompetence, and is the cornerstone of venous ulcer management.

mainstay of treatment for all clinical manifestations of chronic venous insufficiency

Mechanical therapy Daily use of elastic compression stockings reduces swelling in some patients with postthrombotic syndrome, and may prevent worsening of established postthrombotic syndrome and reduce recurrence of healed venous ulcers

herbal remedy that appears to be safe and effective as a short-term treatment for leg pain and swelling

Horse chestnut seed extract (Aesculus hippocastanum L.)

Measurement required for graduated stockings in CVD

Graduated stockings that provide a minimum of 30 to 40 mm Hg at the ankle should be carefully fitted to all patients and worn for their lifetime. It is a mistake to place elastic stockings on edematous limbs, especially those limbs that are tender. Compression bandaging should be used until all edema, inflammation, and tenderness have resolved before fitting the patient with stockings.

Once a patient develops venous thrombosis, this is the only proven method to reduce the risk of postthrombotic syndrome

elastic compression stockings

refers specifically to swelling of a part of the body due to impaired lymph transport capacity as a consequence of a malformation or malfunction of the lymphatics

Lymphedema

congenitally determined intrinsic or constitutional fault in lymphatic drainage

Primary lymphedema

Most common cause of secondary lymphedema

Parasitic infection

* cooccurrence of primary lymphedema and myelodysplasia/acute myeloid leukemia. * unilateral or bilateral lymphedema of the lower extremities * low CD4:CD8 ratio, * immune dysfunction (widespread cutaneous warts), * sensorineural deafness, and * genital lymphedema

EMBERGER SYNDROME

Mutation in EMBERGER SYNDROME

GATA2, which regulates the transcription of genes that are important for both development and maintenance of lymphovenous and lymphatic vessel valves

aberrant eyelashes arising along the posterior border of the eyelid margin, which frequently leads to eyelash irritation of the cornea and photophobia

double row of eyelashes (distichiasis) Seen in LYMPHEDEMA-DISTICHIASIS ## Footnote Lymphedema typically develops during puberty, but may be delayed until early adulthood, especially in females. The lymphedema will commonly worsen in severity over time, and may be complicated by recurrent infection and eventual papillomatosis

Signs and Symptoms of Lymphedema

Early signs of lymphedema

■ Erysipelas ■ Pitting edema

Late signs of lymphedema

■ Nonpitting edema ■ Papillomatosis ■ Secondary infections (bacterial, fungal, viral warts) ■ “Ski-jump” upturned toe nails ■ Thickened, woody skin ■ Verrucous tissue overgrowth

What do you call the sign where there is Thickening of the skin overlying the digits

Kaposi-Stemmer sign (inability to pinch up a fold of skin between the second and third toe on the dorsum of the foot). Seen in lymphedema

Therapeutic options for lymphedema

T/F: Diuretics improves lymphedema by improving lymph drainage.

False. Diuretics are frequently prescribed, and not only do they not alleviate the symptoms, they may actually worsen the condition and should not be used as a primary treatment for lymphedema

first-line therapy for symptom relief of PAOD

First-line therapy for risk reduction of PAOD

second-line therapy for symptom relief of PAOD

Second-line therapy for risk reduction of PAOD

Clopidogrel

Blue or ulcerated painful digits, livedo racemosa, petechiae, and tender calf muscles in the presence of normal pulses suggest what diagnosis?

atheromatous emboli ## Footnote high index of suspicion is needed, especially in the setting of a history of preexisting atherosclerotic disease and a specific precipitating event

strong predictor of death in atheromatous embolism

development of end-stage renal disease

This testing should be obtained on all patients presenting with livedo racemosa.

Antiphospholipid antibody testing

drug that is occasionally related to livedo reticularis

Amantadine

predominant cause of deoxygenation within the venous plexus.

Decreased arteriolar perfusion ## Footnote * Diminished arteriolar flow can result from vasospasm, hyperviscosity, and/or thrombosis. * Physiologic arteriolar vasospasm produces the reversible cutaneous discoloration of livedo reticularis.

* congenital primary lymphedema that leads to disability and disfiguring swelling of the lower extremities * Onset is usually at birth or in early childhood, and the severity can vary but often progresses with time * Bilateral lower-limb asymmetric swelling is most common with deep creases noted over the toes * Large superficial veins can be visible during infancy. * Nail and skin changes all occur, such as toenail upslanting (“ski jump”) dysplasia or skin papillomatosis (greatest over second toe) * Skin infections and hydroceles are also common

Milroy syndrome

MRI finding of chronic lymphedema

**“honeycomb” pattern** of the subcutaneous tissue present in chronic lymphedema