Presence of fluid accumulation and perilymphatic fibrosis without clinical edema
Positive cacifo sign., edema reduces after limb elevation
Negative Cacifo sign. Edema does not reduce after limb elevation
Irreversible edema. Fibrosis and sclerosis of the skin and subcutaneous tissue
Although the stages do not help in the diagnosis, it allows the evaluation and monitoring of treatment efficacy.
In most cases, findings in physical examination associated with a careful history establish the diagnosis and may reveal the cause of the edema. Additional tests are used in the diagnosis assistance:
Duplex Scan: Should be performed for ruling out deep venous thrombosis .
Computed tomography and the magnetic resonance may demonstrate the lymphedema characteristics: presence of edema restricted to the subcutaneous tissue, limited by muscular fascia .
Lymphoscintigraphy : It is the diagnostic test of choice. It has a sensitivity of 70–90 % and specificity of nearly 100 % in distinguishing between lymphatic edema and other causes of peripheral edema. It evaluates the lymphatic function through the measurement of radioactive macromolecular marker clearance. It provides important anatomical and functional information if a surgical procedure is required. However, it should not be used for routine diagnosis, since it could worsen the disease .
Lymphography : The exam allows the study of lymphatic anatomy but not of lymphatic function. Due to its high cost, its technical difficulty, and large number of complications, this exam is no longer used in clinical practice .
Subcutaneous tissue biopsy : It may become necessary in some selected cases, especially in the advanced stages, due to the risk of malignancy. The purpose is not to establish the diagnosis of lymphedema but cancer .
The treatment of the lymphedema should be multidisciplinary . It is primarily based on conservative measures . The approach of secondary lymphedema is to prevent the onset of edema (Table 18.2). In primary lymphedema, the goal is to reduce swelling and to prevent its progression [3, 5, 6].
Lymphedema: Preventive measures
Dietary moderation in intake of fluids and sodium
Body weight control
Daily elevation of the lower limb, positioning it above the heart level during sessions of 20–30 min each
Avoid external pressure on the affected limb (e.g., jewelry, tight clothing)
Daily care and cleansing of the skin
Regular use of elastic compression stockings (not exceeding 15–20 mmHg)
Prophylactic antibiotic use in recurrent cases
Most patients will respond to conservative treatment, which can be done with a combination of complex decongestive physiotherapy, intermittent pneumatic compression, drug therapy, and immediate treatment of any suspected infection or lymphangitis . Despite of the treatment modality, intensive skin care with daily cleansing, low pH lotions, and continuous use of cotton clothes are mandatory. Likewise, after any initial treatment, the use of graduated compression stockings or sleeves combined with exercises and limb elevation is part of the treatment. The elastic stockings facilitate venous and lymphatic flow due to gradual compression [1, 3].
Complex Decongestive Physiotherapy
The Complex Decongestive Physiotherapy comprises two phases. An initial decongestive phase and a maintenance phase.
Phase one is based on manual lymph drainage massage followed by multilayered bandage wrappings. It aims to achieve reduction of the limb diameter, and should be promptly followed by phase two with manual lymph drainage in order to stimulate the lymphatic vessels that are still functioning and to drain the stagnant protein fluid accumulated in the subcutaneous tissue . It should be performed by qualified physiotherapists .
Intermittent Pneumatic Compression
It is based on lymphokinetic medications represented by the benzopyrones . This class of medication has the potential effects of hydrolyzed tissue proteins, enhancing their transport, and to stimulate lymphatic collectors . It comprises the rutosides and the bioflavonoids. The effectiveness of drug treatment alone is controversial . There are several poor quality trials addressing this issue. It is not possible to draw conclusions about the effectiveness of benzopyrones in reducing limb volume, pain, or discomfort in lymphoedematous limbs [7, 9, 10].
Diuretics do not demonstrate long-term benefits, and therefore have no role in the treatment of lymphedema.
Antimicrobial drugs should be administered if any sign of infection is present. The drugs of choice are oral penicillin, cephalexin, and azithromycin from 7 to 14 days. In case of recurrence—more than two to four episodes per year—prophylactic treatment is required with oral penicillin 7 days once a month or benzatin penicillin once a month .
It is reserved for the most severe and refractory cases. The approach may be reconstructive or debulking [1, 12–14]. The reconstructive approach aims to restore the lymphatic function through the making of lymph-venous shunts or transpositions of autologous vessels .
Erysipelas is a skin and mucosal infection caused by the Streptococcus pyogenes beta-hemolytic group A of Lancefield classification. It is the most frequent among all lymphangitis .
The erysipelas is a common condition in clinical daily practice, with an estimated incidence of 10–100 cases per 100,000 inhabitants/year. The female is the most affected gender and it primarily affects adults between 40 and 60 years . It is more common among patients with some chronic diseases .
More than 40 % of affected people have some other health condition . The lower limbs are affected in more than 80 % of cases. The main risk factors are the presence of (1) lymphedema and (2) obesity [16, 19], but also (3) low socioeconomic conditions, (4) immune-deficiencies states, (5) alcoholism, (6) diabetes mellitus , and (7) chronic venous insufficiency are associated [20, 21].
The bacteriological agent needs an inoculation point or a gateway. The most common site of entrance is interdigital mycosis. However, other pathways could be active chronic venous ulcers, skin injuries caused by shoes, insect sting, scarification by scratch, or other injuries of any kind [22, 23].
The evolution depends on the host resistance. It can regress spontaneously without sequel or cause serious systemic infections with hemodynamic instability. It is known that untreated erysipelas have a higher rate of recurrence and leave more sequels in the lymphatic system, with residual and progressive lymphedema than the treated ones [16, 17].
A painful lymph node enlargement may be the first sign. Usually the symptoms precede in a few days the skin manifestations. Initial symptoms are local pain, malaise, asthenia, headache, nausea and vomiting, chills and high fever. Systemic manifestations are usually attributed to the high potential lesion of the toxin produced by Streptococcus sp. Among the signs it is observed edema and local erythema which give the skin the appearance of an orange peel (Fig. 18.1). Later appear spots and erythematous papules that evolve into vesicles, blisters, and bubbles, which coalesce revealing its citrus liquid, blood, or pus content. It may also lead to scabs and skin necrosis (Fig. 18.2). Vesicles and blisters usually appear late in the course and often after systemic and laboratory improvement [15, 24].
Pacient presenting edema and erythema of the right leg, and an orange peel skin, suggestive of erysipela
Advanced erysipela showing erythematous papules, vesicles and blisters containing purulent secretion