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Pathology and Classification of Chronic Venous Disease
Chronic venous disease (CVD) is the most common disorder of the peripheral vascular system. In population studies, 10% to 35% of adults manifested some form of the disease. Its most severe manifestation is cutaneous venous ulceration. CVD and related ulcers affect an estimated 76 and 18 per 100,000 person-years, respectively. Treatment is estimated to cost $3 billion annually, with an average yearly cost of $7460 per treated venous ulcer.
CVD results from a disturbance of blood flow within the veins of the lower extremity because of obstruction or reflux. The resultant venous hypertension initiates a cascade of events that cause a visible deformation of the veins and a chronic inflammatory state that leads to a multitude of dermatologic changes.
An incomplete and inaccurate diagnosis of CVD hinders the elucidation of the pathophysiology, the efficacy of interventions, and a realistic assessment of costs. A physician-evaluated clinical classification (CEAP: clinical; etiology; anatomic distribution; pathophysiologic findings) and severity scoring systems have been developed and validated to facilitate accurate discussion and standardization of reporting. Patient-reported quality-of-life instruments complement these assessments.
In Search of Complete and Accurate Diagnosis
The clinical features of CVD involve a combination of historical, physical, and diagnostic findings. The CEAP classification system was proposed and initially adopted in 1994 in a consensus document. The CEAP clinical (C) classification is assigned during routine physical examination. The E, A, and P classifications serve to define the presence and nature of venous disease and to differentiate nonvenous causes that have similar physical findings ( Table 1 ). However, assignment of clinical class was inconsistent, and the E, A, and P portions were considered cumbersome and difficult to use, and thus the original classification system was underutilized. Accordingly, the classification was revised in 2004 to better define the clinical classifications and to provide a more user-friendly version.
TABLE 1
CEAP Classification
| 1994 | 2004 Modifications (Basic CEAP) ∗ | |||
|---|---|---|---|---|
| C: Clinical Classification | Addition of S (Symptomatic), A (Asymptomatic) | |||
| 0 | No visible signs of disease | No change | ||
| 1 | Telangiectasias, reticular veins, malleolar flare | Reticular veins: diameter 3 mm or less | ||
| 2 | Varicose veins | Varicose veins: diameter 3mm or more | ||
| 3 | Edema without skin changes | No change | ||
| 4 | Skin changes (pigmentation, eczema, lipodermatosclerosis) | 4a | Pigmentation or eczema | |
| 4b | LDS or atrophie blanche | |||
| 5 | C4 with healed ulceration | No change | ||
| 6 | C4 with active ulceration | No change | ||
| E: Etiologic Classification | ||||
| E C E P E S |
Congenital Primary Secondary |
Present since birth CVD of undetermined cause CVD with associated cause |
Addition of E N (no venous cause) | |
| A: Anatomic Classification | ||||
| A S1–5 | Superficial veins | A S | Superficial veins | |
| A D6–16 | Deep veins | A P | Perforating veins | |
| A P17–18 | Perforating veins | A D | Deep veins | |
| A N | No venous location | |||
| P: Pathophysiologic Classification | ||||
| P R P O P R,O |
Reflux Obstruction Reflux and obstruction |
Addition of P N (no venous pathophysiology identified) | ||
CVD , Chronic venous disease; LDS , lipodermatosclerosis.
Corollaries to the CEAP system, the Venous Clinical Severity Score (VCSS) and the Venous Disability Score (VDS), were developed and validated to objectively assess the severity of the clinical features of CVD.
Clues to Chronic Venous Disease
The clinical features of a varicose vein and advanced skin changes seem obvious, but other aspects are less straightforward. For example, pelvic vein incompetence, iliac vein compression, and the role of obesity can be subtle. Chronic skin changes and ulcerations can have etiologies requiring a different treatment. A detailed history and physical examination, coupled with a venous duplex evaluation, provides the necessary data to make a venous diagnosis, initiate therapy, and guide follow-up.
Family history is an important risk factor. Prolonged periods of standing or sitting can predispose to varicosities, as noted in 56% of workers. A history of deep vein thrombosis (DVT) may be subtle or even unrecognized in patients with post thrombotic syndrome (PTS). The incidence of PTS ranges from 30% to 80%, with 4% to 8% of these patients developing ulceration. Conversely, the clinical findings of PTS may be indistinguishable from correctable chronic superficial reflux.
Pain and heaviness in the legs are the most common symptoms of CVD, and some women display cyclical symptoms coinciding with menstruation. An intense itch or burning sensation, thought to arise from mast cell histamine release, has a prevalence of 66% in CVD. Nocturnal pruritus and scratching can be a source of morbidity and predispose to skin ulceration. In contrast, 36% of patients who have these complaints might have no anatomic evidence of venous disease.
A focused vein examination includes height, weight, body mass index (BMI), peripheral pulses, an ankle-to-brachial index (ABI), a sensory examination, and specifically a standing examination of the lower extremities. A statistically significant relationship exists between height and weight and the development of CVD. The height of the standing column of blood in the lower extremity is directly proportional to venous pressure. A correlation between BMI and CEAP clinical class supports the relationship between increased obesity and CVD. The chronic, sustained increase of intraabdominal pressure affects femoral venous outflow with resultant venous hypertension. Notably, weight loss after bariatric surgery facilitates and sustains ulcer healing.
Skin Pathology and Clinical Classification
Venous hypertension is central to the pathophysiology of CVD. A linear relationship exists between ambulatory venous pressure and skin changes with ulceration. The increased venous pressure facilitates extravasation of leukocytes from the microcirculation and establishes a chronic inflammatory response caused by deposition of a ferrous residue. Along with chronic edema, this inflammation results in thickened, discolored dermis with poor resistance to injury. The capacity for reparative healing is also negatively affected, perpetuating the cycle of ulceration and recurrence.
Increased venous pressure causes morphologic changes in the vein wall that can relate to varicosis. CEAP classifies these pathophysiologic changes in a stepwise fashion.
Clinical Class 1: Telangiectasias and Reticular Veins
Telangiectasias (spider veins) are small ectatic vein clusters that represent a confluence of dilated intradermal venules less than 1 mm in caliber. They are a marker for superficial venous reflux in 23% of patients and are a cosmetic issue.
Reticular veins are visible veins 1 to 3 mm in diameter and are dilated, bluish, and tortuous. A cluster of small veins in a fan-shaped pattern on the medial or lateral aspects of the ankle or foot is called corona phlebectatica (malleolar or ankle flare).
Clinical Class 2: Varicose Veins
Varicose veins are large, dilated, tortuous visible veins at least 3 mm in diameter and occur anywhere on the lower extremity during a standing examination. In general, greater saphenous vein (GSV) incompetence produces medial thigh varicosities, and varicosities from short saphenous reflux often appear on the posterior calf.
Clinical Class 3: Edema
Edema is a common clinical finding of many disease states. The edema of CVD has several distinguishing characteristics. Bilaterality is common in the edema of systemic disease such as congestive heart disease, liver failure, and chronic kidney disease. In contrast, the edema of CVD is usually unilateral, typically occurring in the malleolar or retromalleolar regions. The edema often worsens toward the end of the day. Duplex ultrasound assists in differentiating the etiology of the edema, and absence of venous reflux or obstruction suggests a systemic process.
Clinical Class 4: Eczema, Pigmentation, White Atrophy, and Lipodermatosclerosis
The dermatologic changes of CVD can be identical to other inflammatory conditions. Eczema, a common erythematous dermatitis, is seen in uncontrolled CVD. It can manifest as blistering, weeping, or scaling dermis. Cellulitis can mimic venous eczema, because both are erythematous, warm, and painful. However, venous eczema itches and is poorly demarcated, and it has associated scale and exudates in advanced cases.
Various shades of tan and brown skin pigmentation are a common finding in patients with CVD. The discoloration represents ferrous products from the breakdown of extravasated red blood cells. The resultant environment favors persistent inflammation of the dermis that further damages and delays healing. White depigmentation, or atrophie blanche (white atrophy), represents an atrophic area of skin affected by CVD, not a healed ulcer or scar. It is a sign of severe CVD and is usually surrounded by dilated capillaries and hyperpigmentation. Lipodermatosclerosis (LDS) is a localized scarring and contracture of the skin secondary to fibrosis and represents an advanced stage of CVD. It may be painful or associated with a diffuse inflammatory edema.
Clinical Classes 5 and 6: Ulceration
Cutaneous ulceration has the greatest morbidity of all the sequelae of CVD. The incidence of venous ulceration varies from 0.05% to 1.52% in the total population. Isolated superficial reflux is capable of producing severe CVD but responds well to appropriate therapy. The clinical appearance of ulceration resulting from postthrombotic CVD is often indistinguishable and can have a completely different management plan and prognosis. The distinction between these highlights the need for accurate diagnosis and therapy.
Venous ulcers have unique identifying characteristics. Ninety-five percent of venous ulcers occur in the gaiter distribution, typically about the malleoli, and have a bed of granulation tissue covered with a fibrinous, yellow-gray exudate. The key physical finding is a surrounding area of diseased skin about the ulcer that includes hyperpigmentation, lipodermatosclerosis, or eczema. Chronic recurrent inflammation can produce a deformity described as resembling an inverted champagne bottle.
Other Etiologies of Leg Ulceration
Although 72% of ulcerations are caused by a venous etiology, the remainder can be caused by arterial ulcerations, combined venous and arterial disease, infections, malignancies, vasculitis, hypercoagulable states, and systemic diseases. The differential diagnosis includes many underlying pathogenic factors that might need specific therapy to induce healing ( Box 1 ).
BOX 1
Differential Diagnosis of Chronic Venous Ulcer
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