Atherosclerosis, Arterial Insufficiency, and
Atheroembolization ICD-9: 440 ICD-10: I70
Atherosclerosis obliterans (ASO), especially of the lower extremities, is associated with spectrum of cutaneous findings of slowly progressive ischemic changes.
Symptoms range from intermittent claudication with exertional muscle pain and fatigue to limb ischemia with rest pain and tissue damage and acute ischemia.
Cutaneous findings range from dry skin, hair loss, onychodystrophy, gangrene, and ulceration.
Atheroembolism is the phenomenon of dislodgment of atheromatous debris from a proximal affected artery or aneurysm with centrifugal microembolization and resultant acute ischemic and infarctive cutaneous lesions.
More common with advanced age and invasive procedures.
Manifestations are blue or discolored toes (“blue toe”), livedo reticularis, and gangrene
Age of Onset. Middle aged to elderly. Males > females.
Incidence. Atherosclerosis is the cause of 90% of arterial disease in developed countries, affecting 5% of men >50 years; 10% (20% of diabetics) of all men with atherosclerosis develop critical limb ischemia.
Risk Factors for Atherosclerosis. Cigarette smoking, hyperlipidemia, low high-density lipoprotein, high low-density lipoprotein (LDL), high cholesterol, hypertension, diabetes mellitus, hyperinsulinemia, abdominal obesity, family history of premature ischemic heart disease, and personal history of cerebrovascular disease or occlusive peripheral vascular disease.
Atherosclerosis is the most common cause of arterial insufficiency and may be generalized or localized to the coronary arteries, aortic arch vessels to the head and neck, or those supplying the lower extremities, i.e., femoral, popliteal, anterior, and posterior tibial arteries. Atheromatous material in the abdominal or iliac arteries can also diminish blood flow to the lower extremities as well as break off and embolize downstream to the lower extremities (atheroembolization). In addition to large-vessel arterial obstruction, individuals with diabetes mellitus often have microvasculopathy (see Section 15, p. 384).
Atheroembolism. Multiple small deposits of fibrin, platelet, and cholesterol debris embolize from proximal atherosclerotic lesions or aneurysmal sites. Occurs spontaneously or after intravascular surgery or procedures such as arteriography, fibrinolysis, or anticoagulation.
Atherosclerosis/Arterial Insufficiency of Lower Extremity Arteries
Symptoms. Pain on exercise, i.e., intermittent claudication. With progressive arterial insufficiency, pain and/or paresthesias at rest occur in leg and/or foot, especially at night.
Pallor, cyanosis, livedoid vascular pattern (Fig. 17-1), loss of hair on affected limb. Earliest infarctive changes include well-demarcated maplike areas of epidermal necrosis. Later, dry black gangrene may occur over the infarcted skin (purple cyanosis → white pallor → black gangrene) (Fig. 17-2). Shedding of slough leads to well-demarcated ulcers in which underlying structures such as tendons can be seen.
Figure 17-1. Atherosclerosis obliterans, early The great toe shows pallor and there is mottled, livedoid erythema on the tip of the toe. In this 68-year-old diabetic man, the iliac artery was occluded.
Figure 17-2. Atherosclerosis obliterans (A) There is pallor of the forefoot and mottled erythema distally with incipient gangrene on the great toe and the second digit. This is a female diabetic with partial occlusion of the femoral artery. The patient was a smoker. (B) More advanced gangrene of the second to the fifth toe, the great toe is ebony white and will also turn black.
General Examination. Pulses. Pulse of large vessels usually diminished or absent. In diabetic patients with mainly microangiopathy, gangrene may occur in the setting of adequate pulses. Temperature of foot: cool to cold.
Bürger Sign. With significant reduction in arterial blood flow, limb elevation causes pallor (best noted on plantar foot); dependency causes delayed and exaggerated hyperemia. Auscultation over stenotic arteries reveals bruits.
Pain. Ischemic ulcers are painful; in diabetic patients with neuropathy and ischemic ulcers, pain may be minimal or absent.
Distribution. Ischemic ulcers may first appear between toes at sites of pressure and beginning on fissures on plantar heel. Dry gangrene of feet, starting at the toes or at pressure sites (Fig. 17-2B).
Symptoms. Acute pain and tenderness at site of embolization.
Skin Lesions. Violaceous livedo reticularis on legs, feet, but also as high up as buttocks. Ischemic changes with poor return of color after compression of skin. “Blue toe” (Fig. 17-3): indurated, painful plaques often following livedo reticularis on calves and thighs that may undergo necrosis (Fig. 17-4), become black and crusted, and ulcerate. Cyanosis and gangrene of digits.
Figure 17-3. Atheroembolism after angiography A mottled (“blue toe”), violaceous, vascular pattern on the fore-foot and great toe. The findings were noted after intravascular catheterization and angiography in an individual with ASO.
Figure 17-4. Atheroembolism with cutaneous infarction Violaceous discoloration and cutaneous infarctions with a linear arrangement on the medial thigh of a 73-year-old woman with atherosclerosis, heart failure, and diabetes.
General Examination. Pulses. Distal pulses may remain intact.
Hematology. Rule out anemia, polycythemia.
Lipid Studies. Hypercholesterolemia (>240 mg/dL), often associated with rise in LDL. Hyper-triglyceridemia (250 mg/dL), often associated with rise in very low-density lipoproteins and remnants of their catabolism (mainly intermediate-density lipoprotein).
Dermatopathology of Atheroembolism. Deep skin and muscle biopsy specimen shows arterioles occluded by fibrosis with multinucleated giant cells surrounding biconvex, needle-shaped clefts corresponding to cholesterol crystal microemboli.
Doppler Studies. Show reduced or interrupted blood flow.
Digital Plethysmography. With exercise can unmask significant atherosclerotic involvement of lower extremity arteries.
X-Ray. Calcification can be demonstrated intramurally.
Arteriography. Atherosclerosis is best visualized by angiography. Ulceration of atheromatous plaques seen in abdominal aorta or more distally.
Diagnosis and Differential Diagnosis
Clinical suspicion confirmed by arteriography and deep skin biopsy (atheroembolism).
Differential Diagnosis. Intermittent Claudication. Pseudoxanthoma elasticum, Bürger disease (thromboangiitis obliterans), arthritis, gout.
Painful Foot. Gout, interdigital neuroma, flat feet, calcanean bursitis, plantar fasciitis, rupture of plantar muscle.
Ischemic and Infarctive Lesions of Leg/Foot. Vasculitis, Raynaud phenomenon (vasospasm), disseminated intravascular coagulation, cryoglobulinemia, hyperviscosity syndrome (macroglobulinemia), septic embolization (infective endocarditis), nonseptic embolization, drug-induced necrosis (warfarin, heparin), ergot poisoning, intra-arterial injection, livedo reticularis syndromes, external compression (popliteal entrapment).
Course and Prognosis
Arterial insufficiency is a slowly progressive disease, punctuated by episodes of complete occlusion or embolism. Atherosclerosis of coronary and carotid arteries usually determines survival of patient, but involvement of lower extremity arteries causes significant morbidity. Balloon angioplasty, endarterectomy, and bypass procedure have improved prognosis of patients with atherosclerosis. Amputation rates have been lowered from 80% to <40% by aggressive vascular surgery. Atheroembolism may be a single episode if atheroembolization follows intra-arterial procedure. May be recurrent if spontaneous and associated with significant tissue necrosis.
Prevention. Goal of management is prevention of atherosclerosis.
Medical Management of primary hyperlipidemia: by statins, diet, and exercise. Reduce elevated blood pressure. Discontinue cigarette smoking. Encourage walking to create new collateral vessels. Position ischemic foot as low as possible without edema. Heparin and warfarin. IV prostacyclins. Analgesics.
Surgical Management. Endarterectomy or bypass for iliac occlusions. Debridement of necrotic tissue locally. Amputation of leg/foot: indicated when medical and surgical management has failed.
Thromboangiitis Obliterans (TO) ICD-9: 443.1 ICD-10: I73.1
A rare inflammatory occlusive disease of medium sized and small arteries and veins.
Predominantly in males, 20–40 years of age.
Very strong association with smoking.
An angiitis clinically indistinguishable from TO occurs in persons consuming cannabis.
Clinical manifestations are cold sensitivity; ischemia: claudication of leg, foot, arm, or hand.
Peripheral cyanosis, ischemic ulcers, gangrene (Fig. 17-5), and superficial thrombophlebitis.
Therapy: smoking cessation, analgesics, wound care; antiplatelet agents, prostacyclins, pentoxifylline, angioplasty, sympathectomy, amputation.
Synonym: Bürger disease.
Figure 17-5. Thromboangiitis obliterans Infarctive necrosis on the great toe of a 28-year-old man. The lesion is exquisitely painful. (The yellowish-brownish color is from iodine disinfection).
Thrombophlebitis and Deep Venous Thrombosis
ICD-9: 671.2 ICD-10: I 80 ICD-9: 433.40 ICD-10: I 80.2
Superficial phlebitis (SP) is an inflammatory thrombosis of a superficial normal vein, usually due to infection or trauma from needles and catheters.
Inflammatory thrombosis of varicose vein usually in the context of the chronic venous insufficiency (CVI) syndrome.
Deep venous thrombosis (DVT) is due to thrombotic obstruction of a vein with or without an inflammatory response.
Occurs due to slow blood flow, hypercoagulability, or changes in the venous walls.
The most common predisposing factors and causes are listed below.
Predisposing Factors and causes of Deep Venous Thrombosis
Congestive heart failure
Acute myocardial infarction
Pregnancy and postpartum
Spinal cord injuries
Less Common Factors
Sickle cell anemia
Protein C or S deficiency
Previous history of venous thrombosis
Leiden factor V mutation
Severe pulmonary insufficiency
Antithrombin III deficiency
Etiology and Pathogenesis
The thrombus originates in an area of low venous flow. An occlusion of a vein by thrombus imposes a block to venous return, which leads to increased venous pressure and edema in the distal limb. An inflammatory response to the thrombus causes pain and tenderness. If the venous pressure is too high, arterial limb flow may rarely be compromised and ischemia of the distal limb may occur. The thrombus in the vein often has a free-floating tail, which may break off to produce a pulmonary embolus. Organization of the thrombus in the vein destroys the venous walls, and this leads to the postthrombotic syndrome.
Patients complain of pain or aching in the involved limb or notice limb swelling. Some patients may have no symptoms. Pulmonary embolus may be the first indication of DVT.
Superficial thrombophlebitis is diagnosed by the characteristic induration of a superficial vein with redness, tenderness, and increased heat (Fig. 17-6A). DVT presents with a swollen, warm, tender limb (Fig. 17-6B) with prominent distended collateral veins. Pitting edema may occur but is not always present, and a tender cord may be felt where the vein is thrombosed. With iliofemoral thrombophlebitis, the limb is swollen from the foot to the inguinal region and tenderness is not present in the limb, but collateral veins may form from the thigh to the abdominal wall. Two types are recognized: the limb may be very pale and painful (phlegmasia alba dolens) (Fig. 17-6B) or may be cyanotic and painful with cold digits if the arterial inflow is also compromised (phlegmasia coerulea dolens). In thrombosis of calf veins, the calf and foot are swollen and warm, and there is deep tenderness of the calf, often without a palpable cord.
Figure 17-6. Superficial phlebitis and deep venous thrombosis (A) A linear painful erythematous cord extending from the popliteal fossa to the mid-calf in a 35-year-old man who had moderate varicosities. Phlebitis occurred after a 15-h flight. (B) The leg is swollen, pale, with a blotchy cyanotic discoloration, and is painful. The episode occurred after abdominal surgery (the circular marks are from a compression bandage).
Migratory phlebitis describes an inflammatory induration of superficial veins that migrates within a defined region of the body; may be associated with thromboangiitis obliterans and malignancies. Mondor disease (sclerosing phlebitis) is an indurated, subcutaneous vein from the breast to the axillary region that during healing leads to a shortening of the venous cord, which puckers the skin.
Venous imaging by color-coded duplex ultrasound and Doppler examination reveals an absence of flow or of the normal respiratory venous flow variations in proximal venous occlusions. For thrombophlebitis of the calf veins, intravenous [125I] fibrinogen or a venogram gives a definite diagnosis.
Lymphedema, cellulitis, erysipelas, superficial phlebitis, and lymphangitis. An uncommon differential diagnosis is rupture of the plantar muscle, which produces pain, swelling, and ecchymotic areas in the dependent ankle area.
The treatment of SP is compression, antiplatelet drugs, and nonsteroidal anti-inflammatory agents.
The treatment of DVT is anticoagulation. IV heparin. The partial thromboplastin time (PTT) should be 1.5–2 times normal. Low-molecular-weight heparin is also effective. Warfarin can be started orally at the same time and should overlap heparin for 5 days until the necessary factors for blood clotting are depressed. Elastic stockings and compression are mandatory and should be worn for at least 3 months; zinc paste–impregnated bandages (Unna boot) and ambulation should be started as soon as symptoms subside.
Chronic Venous Insufficiency ICD-9: 459.81 ICD-10: I87.2
Chronic venous insufficiency results from failure of centripetal return of venous blood and increased capillary pressure.
The resultant changes include edema, stasis dermatitis, hyperpigmentation, fibrosis of the skin and subcutaneous tissue (lipodermatosclerosis) of the leg, and ulceration.
Venous ulcers are the most common chronic wounds in humans.
Epidemiology and Etiology
Varicose veins: peak incidence of onset 30–40 years. Varicose veins are three times more common in women than in men.
Etiology. CVI is most commonly associated with varicose veins and the postphlebitic syndrome. Varicose veins are an inherited characteristic.
Aggravating Factors. Pregnancy, increased blood volume, increased cardiac output, increased venocaval pressure, progesterone.
The damaged valves of the deep veins of the calf are incompetent at restricting backflow of blood. Damaged communicating veins connecting deep and superficial calf veins also cause CVI in that blood flows from deep veins to superficial venous plexus. Fibrin is deposited in the extravascular space and undergoes organization, resulting in sclerosis and obliteration of lymphatics and microvasculature.
This cycle repeats itself: initial event → aggravation of venous stasis and varicose vein dilatation → thrombosis → lipodermatosclerosis → stasis dermatitis → ulceration.
Prior episode(s) of superficial phlebitis and DVT. Risk factors are listed on page 45.
CVI is commonly associated with heaviness or aching of leg, which is aggravated by standing (dependency) and relieved by walking. Lipodermatosclerosis may limit movement of ankle and cause pain and limitation of movement, which in turn increases stasis. Leg edema aggravated by dependency (end of the day, standing), summer season. Shoes feel tight in the evening. Night cramps.
The CAEP staging system for CVI is shown below.
Clinical Picture (C)
C0 no clinical signs
C1 small varicose veins
C2 large varicose veins
C4 skin changes
C5 healed ulcer
C6 active ulcer
Ap perforans (communicating vein)
Pr, o reflux + obstruction
Varicose Veins. Superficial leg veins are enlarged, tortuous, with incompetent valves; best evaluated with the patient standing (Fig. 17-7A). “Blow-out” at sites of incompetent communicating veins. Tourniquet test: A tourniquet is applied to the leg that has been elevated to empty the veins; when the patient stands up and the tourniquet is released, there is instant filling of a varicose vein due to absent or ill-functioning valves. Varicose veins may or may not be associated with starburst phlebectasia usually overlying the area of an incompetent communicating vein (Fig. 17-7B). Superficial venulectasias (spider phlebectasia) without a starburst pattern occur also and far more commonly without CVI, usually on the thighs and lateral lower legs in women.
Figure 17-7. Varicose veins (A) There are meandering and convoluted irregular varicose veins on the thigh and below the knee of a 70-year-old man who also had lipodermatosclerosis and stasis dermatitis on the lower legs. (B)Starburst venectasias on the calf. This is an area overlying an insufficient communicating vein.
Edema. Dependent; improved or resolved in the morning after a night in the horizontal position. Dorsa of feet, ankles, lower legs.
Eczematous (Stasis) Dermatitis. Occurs in setting of CVI about the lower legs and ankles (Fig. 17-8). It is a classic eczematous dermatitis with inflammatory papules, scaly and crusted erosions; in addition, there is pigmentation, stippled with recent and old hemorrhages; dermal sclerosis; and excoriations due to scratching. If eczematous stasis dermatitis is extensive, may be associated with generalized eczematous dermatitis, i.e., “id” reaction or autosensitization (see Section 2).
Figure 17-8. Stasis dermatitis in CVI A patch of eczematous dermatitis overlying venous varicosities on the medial ankle in a 59-year-old woman. The lesion is papular, scaly, and itching.
Atrophie Blanche. Small ivory-white depressed patches (Fig. 17-9) on the ankle and/or foot; stellate and irregular, coalescing; stippled pigmentation; hemosiderin-pigmented border, usually within stasis dermatitis. Often following trauma.
Figure 17-9. Chronic venous insufficiency. Atrophie blanche An area of diffuse and mottled pigmentation due to hemosiderin and ivory-white patches of atrophie blanche. Such lesions are both itchy and painful.
Lipodermatosclerosis. Inflammation, induration, pigmentation of lower third of leg creating “champagne bottle” or “piano leg” appearance with edema above and below the sclerotic region (Fig. 17-10A). “Groove sign” created by varicose veins meandering through sclerotic tissue. A verrucous epidermal change can occur overlying the sclerosis and can be combined with chronic lymphedema.
Figure 17-10. Chronic venous insufficiency and lipodermatosclerosis The ankle is relatively thin and the upper calf edematous, creating a “champagne bottle” or “piano leg” appearance. (A) Varicose veins are embedded in pigmented, sclerotic tissue. There are also areas of atrophie blanche. (B) Varicose veins are less visible here but can be easily palpated in the sclerotic plaque encasing the entire calf (“groove” sign). There is also pigmentation and minor papular stasis dermatitis.
Ulceration. Occurs in 30% of cases; very painful “hyperalgesic microulcer” in area of atrophie blanche; larger superficial or deep ulcers, sharply defined with deep margin, necrotic base surrounded by atrophie blanche, stasis dermatitis, and lipodermatosclerosis (Figs. 17-10B and 17–11). Venous ulcers usually occur medially and above ankles (Fig. 17-11). Venous ulcers and their differential diagnosis are discussed in more detail on pages 422 to 424.
Figure 17-11. Venous insufficiency (A) Two coalescing ulcers with a necrotic base in an area of atrophie blanche, lipodermatosclerosis, and stasis dermatitis. Scratch marks indicate itchiness of surrounding skin, while the ulcers are painful. (B) A giant ulcer, well-defined with scalloped borders and a beefy red base in a leg with lipodermatosclerosis.
Doppler and Color-Coded Duplex Sonography. These detect incompetent veins, venous occlusion due to thrombus.
Phlebography. Contrast medium is injected into veins to detect incompetent veins and venous occlusion.
Imaging. X-ray may show subcutaneous calcification (10% of chronic cases), i.e., postphlebitic subcutaneous calcinosis.
Dermatopathology. Early: dilated small venules and lymphatics; edema of extracellular space. Later: capillaries dilated, congested with tuft formation and tortuosity of venules; deposition of fibrin. Endothelial cell hypertrophy;venous thrombosis; angioendotheliomatous proliferation mimicking Kaposi sarcoma. In all stages, extravasation of red blood cells that break down forming hemosiderin, which is taken up by macrophages. Lymphatic vessels become encased in a fibrotic stroma, i.e., lipodermatosclerosis.
Usually made on history, clinical findings, Doppler and color-coded Duplex sonography, phlebography.
Prerequisite. Compression dressings or stockings; Unna boot.
Atrophie Blanche. Avoid trauma to the area involved. Intralesional triamcinolone into painful lesions. Compression.
Stasis Dermatitis. Topical glucocorticoids (short term). Topical antibiotic treatments (e.g., mupirocin) when secondarily infected. Culture for methicillin-resistant Staphylococcus aureus.
Varicose Veins. Injection Sclerotherapy. A sclerosing agent is injected into varicosities, followed by prolonged compression.
Vascular Surgery. Incompetent perforating veins are identified, ligated, and cut, followed by stripping long and/or short saphenous veins out of the main trunk.
Endovascular Techniques. These new technologies encompass endoscopic subfascial dissection of perforating veins (employed primarily in the elimination of insufficient perforating veins in CVI) and endoscopic endovenous diode laser or radio frequency thermal heating, which leads to occlusion of varicose vein.
Venous Ulcers. See page 424.
Most Common Leg/Foot Ulcers ICD-9: 707 ICD-10: I83.0
Leg ulcers occur commonly in late middle and old age.
They arise in association with CVI, chronic arterial insufficiency, or peripheral sensory neuropathy.
In some patients, a combination of these factors.
Particularly in diabetes, leg ulcers are common. An estimated 2½ million persons in the United States have leg ulcers, with an estimated loss of 2 million workdays per year.
Leg ulcers are associated with significant long-term morbidity and often do not heal unless the underlying problem(s) is (are) corrected.
Rarely squamous cell carcinoma (SCC) can arise in chronic venous ulcers.
Venous Ulcers. The prevalence of venous ulcers is estimated to be approximately 1%. It rises with patient age, obesity, previous leg injury (fractures), DVT, and phlebitis. Venous ulcers are associated with at least one or all of the symptoms of CVI (Fig. 17-11); single or multiple; they are usually on the medial lower calf, especially over the malleolus (medial > lateral), in the area supplied by incompetent perforating veins (Fig. 17-11). Can involve the circumference of the entire lower leg (Fig. 17-11B). They are sharply defined, irregularly shaped, relatively shallow with a sloping border, and usually painful. The base is usually covered by fibrin and necrotic material (Fig. 17-11A), and there is always secondary bacterial colonization. SCC can arise in a long-standing venous ulcer (Fig. 17-12) of the leg.
Figure 17-12. Squamous cell carcinoma in chronic venous ulcer A venous ulcer had been present >10 years in an area of lipodermatosclerosis and stasis dermatitis. Eventually, the base of the ulcer became elevated, hard, less painful. Deep biopsy (circular mark in the center) revealed necrosis and at the base invasive squamous cell carcinoma.
Arterial Ulcers. Arterial ulcers are associated with peripheral arterial disease (atherosclerosis obliterans, see p. 410). Characteristically painful at night and often quite severe; may be worse when legs are elevated, improving on dependency. Occur on the lower leg, usually pretibial, supramalleolar (usually lateral), and at distant points, such as toes. Painful. Punched out, with sharply demarcated borders (Fig. 17-13). A tissue slough is often present at the base, under which tendons can be seen.
Figure 17-13. Chronic arterial insufficiency with a sharply defined, “punched out” ulcer with irregular outlines The extremity was pulseless, and there was massive ischemia on the toes.
A special type of arterial ulcer is Martorell ulcer, which is associated with labile hypertension and lacks clinical signs of ASO. Ulcer(s) start with a black eschar surrounded by erythema and after sloughing of necrotic tissue are punched out with sharply demarcated borders, with surrounding erythema; very painful on the anterior lateral lower leg.
Combined Arterial and Venous Ulcers. These ulcers arise in patients who have both CVI and ASO and thus show a combination of signs and symptoms of both venous and arterial insufficiency and ulceration (Fig. 17-14).
Figure 17-14. Chronic arterial and venous insufficiency, “combined” arterial and venous ulcers Note pronounced lipodermatosclerosis and ulceration on the supramalleolar lower leg (venous component) and purple discoloration of forefoot and toes with punched-out ulcer revealing tendon over metatarsal site (arterial component).
Neuropathic Ulcers. Soles, toes, heel. Most commonly associated with diabetes of many years’ duration. (See “Diabetic Foot,” p. 383.)
A differential diagnosis of the three main types of leg/foot ulcers is shown in Table 17-1. Other differential diagnostic considerations include ulcerated SCC, basal cell carcinoma, injection drug use (skin popping), pressure ulcer (ski boot). Ulcerations also occur in vasculitis (particularly polyarteritis nodosa), [erythema induratum, calciphylaxis, and various infections [ecthyma, Buruli ulcer, Mycobacterium marinum infection, gumma, leprosy, invasive fungal infection, chronic herpes simplex virus (HSV) ulcer] and in sickle cell anemia, polycythemia vera, pyoderma gangrenosum, necrobiosis lipoidica with ulceration, factitia.
Table 17-1 DIFFERENTIAL DIAGNOSIS OF THREE MAJOR TYPES OF LEG ULCERS
Course and Prognosis
Course and prognosis are dependent on underlying disease.
General Management. In general, factors such as anemia and malnutrition should be corrected to facilitate healing. Control hypertension, weight reduction in the obese, exercise; mobilize patient; correct edema caused by cardial, renal, or hepatic dysfunction. Of utmost importance is treatment of underlying disease. Arterial ulcers do not heal unless arterial blood flow is corrected by endarterectomy or bypass surgery (see p. 414) Venous ulcers tend to be recurrent unless underlying risk factors are corrected, i.e., corrective surgery and/or elastic stockings worn on a daily basis (see management of CVI, p. 420). Beware of excess compression in patients with additional underlying arterial occlusion. In neuropathic ulcers, correct underlying diabetes, rule out underlying osteomyelitis, distribute weight of pressure points with special shoes in neuropathic ulcers. Note: diabetic patients are particularly predisposed to ulcers and frequently have several etiologic factors in play, i.e., peripheral vascular disease, neuropathy, infection, and impaired healing.
Local Treatment of Ulcer and Surrounding Skin. Treat stasis dermatitis in CVI with wet dressings and moderate to potent glucocorticoid ointment. Debridement of necrotic material mechanically (surgically) or by enzymatic debriding agents; antiseptics and antibiotics to counteract infection. Hydrocolloid dressings. For cleaned ulcers that heal slowly surgical procedures either by pinch grafts, split-thickness skin grafts, epidermal grafts, cultured keratinocyte allografts, or composite grafts.
Livedoid Vasculitis (LV) ICD-10: L95.0
LV is a thrombotic vasculopathy of dermal vessels confined to the lower extremities and starting mostly in the ankle region.
A triad of livedo reticularis, atrophie blanche, and very painful, small punched-out ulcers that have a very poor tendency for healing (Fig. 17-15).
Atrophie blanche in LV is clinically indistinguishable from that seen in CVI, except for varicose veins (compare Figs. 17-15 and 17-9). LV is a reaction pattern of the skin that often recurs in winter or summer (“livedo reticularis with winter and summer ulcerations”).
Histologically, there are fibrin thrombi in small and medium-sized dermal veins and arteries with wedge-shaped necrosis and hyalinization of the vessel walls (segmental hyalinizing vasculitis).
LV may be idiopathic or may be associated with Sneddon syndrome (see Fig. 14-42), antiphospholipid antibody syndrome, or conditions of hypercoagulability or hyperviscosity.
Treatment: bed rest, analgesics, low-dose heparin, and platelet aggregation inhibitors. Pain can be relieved and healing accelerated by systemic glucocorticoids. Anabolic agents such as danazol and stanazolol have been anecdotally reported to be effective.
Larger ulcers will have to be excised and grafted.
Figure 17-15. Livedoid vasculitis This is characterized by the triad of livedo reticularis, atrophie blanche, and small, painful, crusted ulcers. This is clinically indistinguishable from atrophie blanche seen in CVI except for the absence of varicose veins.
Chronic Lymphatic Insufficiency ICD-9: 459.81 ICD-10: I87.2
Lymphedema in childhood and early adult life are genetic and are often caused by defects in vascular endothelial growth factor receptor 3 and FoxC2, a transcription factor.
Acquired lymphedema of adults may be related to chronic venous insufficiency; chronic, recurring soft-tissue infections (erysipelas, cellulitis, see Section 25); node dissection and radiation after cancer; and in some geographic regions by filariasis.
Depending on etiology-acquired lymphedema most commonly occurs on the lower extremities but may also arise on the arm and hand.
Clinical manifestations: swelling of extremities, pitting edema initially slowly evolving into nonpitting woody induration.
Prolonged lymphedema may lead to grotesque enlargement of extremity; epidermal hyperplasia with verrucosis (Fig. 17-16).
Secondary, soft-tissue infection (erysipelas and cellulitis) is common, recurrent, and leads to worsening of the condition.
Treatment is mainly compression (as in CVI) and manual lymphatic drainage; antibiotics in secondary infection.
Lymphangiosarcoma (in postmastectomy lymphedema) is a rare complication: Stewart-Treves syndrome.
Figure 17-16. Chronic lymphatic insufficiency: lymphedema Lower legs are thickened of woody consistency and three is massive hyperkeratosis and pebbly and papillomatous overgrowths. The 60-year-old patient had had innumerable episodes of erysipelas and cellulitis. There is also diabetes and atherosclerosis.
Pressure Ulcers ICD-9: 707 ICD-10: L89
Pressure ulcers develop at body-support interfaces over bony prominences as a result of external compression of the skin, shear forces, and friction, which produce ischemic tissue necrosis.
Occur in patients who are obtunded mentally or have diminished sensation (as in spinal cord disease) in the affected region. Secondary infection results in localized cellulitis, which can extend locally into bone or muscle or into the bloodstream.
Synonyms: Pressure sore, bed sore, decubitus ulcer.
Age of Onset. Any age, but the greatest prevalence of pressure ulcers is in elderly, chronically bedridden patients.
Sex. Equally prevalent in both sexes.
Prevalence. Acute care hospital setting, 3–14%; long-term care settings, 15–25%; home-care settings, 7–12%; spinal cord units, 20–30%.
Risk factors: inadequate nursing care, diminished sensation/immobility (obtunded mental status, spinal cord disease), hypotension, fecal or urinary incontinence, the presence of fracture, hypoalbuminemia, and poor nutritional status. External compression with pressures >30 mm Hg occludes skin capillaries so that the surrounding tissues become anoxic and eventually necrotic. Secondary bacterial infection can enlarge the ulcer, extend to underlying structures (osteomyelitis), and invade the bloodstream, with bacteremia and septicemia. Infection also impairs or prevents healing.
Skin Lesions. Clinical Categories of Pressure Ulcers. Early change: localized erythema that blanches on pressure.
Stage I: Nonblanching erythema of intact skin.
Stage II: Necrosis, superficial or partial thickness involving the epidermis and/or dermis. Bullae → necrosis of dermis (black) → shallow ulcer.
Stage III: Deep necrosis, crateriform ulceration with full-thickness skin loss (Fig. 17-17); damage or necrosis can extend down to, but not through, fascia.
Figure 17-17. Pressure ulcer, stage III Well-demarcated crateriform ulcer with full-thickness skin loss extending down to fascia over greater trochanteric region.
Stage IV: Full-thickness necrosis (→ ulceration) with involvement of supporting structures such as muscle and bone (Fig. 17-18). May enlarge to many centimeters. May or may not be tender. Borders of ulcers may be undetermined.
Figure 17-18. Pressure ulcer, stage IV on the heel The black necrosis seen here extended into the calcaneal bone which also had to be debrided.
Well-established pressure ulcers with devitalized tissue at the base (eschar) have a higher chance of secondary infection. Purulent exudate and erythema surrounding the ulcer suggest infection. Foul odor suggests anaerobic infection.
Distribution. Occur over bony prominences: sacrum (60%) > ischial tuberosities, greater trochanter (Fig. 17-17), heel (Fig. 17-18) > elbow, knee, ankle, occiput.
General Examination. Fever, chills, or increased pain of ulcer suggests possible cellulitis or osteomyelitis.
Wound Culture. For aerobic and anaerobic bacteria.
Blood Culture. Bacteremia often follows manipulation of ulcer (within 1–20 min of beginning the debridement); resolves within 30–60 min.
Pathology. Skin Biopsy. Epidermal necrosis with eccrine duct and gland necrosis. Deep ulcers show wedge-shaped infarcts of the subcutaneous tissue.
Bone Biopsy. Essential for diagnosing continuous osteomyelitis; specimen is examined histologically and microbiologically.
Diagnosis and Differential Diagnosis
Usually made clinically. Differential diagnosis includes infectious ulcer (actinomycotic infection, deep fungal infection, chronic herpetic ulcer), thermal burn, malignant ulcer, pyoderma gangrenosum, rectocutaneous fistula.
Course and Prognosis
If pressure is relieved, some changes are reversible; intermittent periods of pressure relief increase resistance to compression. Osteomyelitis occurs in nonhealing pressure ulcers (32–81%). Septicemia is associated with a high mortality rate. Overall, patients with pressure ulcers have a fourfold risk of prolonged hospitalization and of dying when compared with patients without ulcers. With proper treatment, stages I and II ulcers heal in 1–4 weeks and stages III and IV ulcers heal in 6 to >12 weeks.
Prophylaxis in At-Risk Patients. Reposition patient every 2 h (more often if possible); massage areas prone to pressure ulcers while changing position of patient; inspect for areas of skin breakdown over pressure points; minimize friction and shear forces.
• Use interface air mattress to reduce compression.
• Clean with mild cleansing agents, keeping skin free of urine and feces.
• Minimize skin exposure to excessive moisture from incontinence, perspiration, or wound drainage.
• Maintain head of the bed at a relatively low angle of elevation (<30°).
• Evaluate and correct nutritional status; consider supplements of vitamin C and zinc.
• Mobilize patients as soon as possible.
Stages I and II Ulcers. Topical antibiotics (not neomycin) under moist sterile gauze may be sufficient for early erosions. Normal saline wet-to-dry dressings may be needed for debridement. Hydrogels or hydrocolloid dressings.
Stages III and IV Ulcers. Surgical management: debridement of necrotic tissue, bony prominence removal, flaps, and skin grafts.
Infectious Complications. Prolonged course of antimicrobial agent depending on sensitivities, with surgical debridement of necrotic bone in osteomyelitis.