Keywords

The Assessment

The local treatment of ulcers is based on a methodological approach that considers the type of lesion and any variables (infection, associated pain, localization, dimensions) that can be present at baseline, or it can occur subsequently. Therefore, a preliminary assessment is always necessary to develop an effective treatment.

The classification of upper and lower limb systemic sclerosis (SSc) ulcers [1, 2] defines the ulcer type and provides the fundamental elements to decide the type of local treatment (e.g. removal of calcinotic deposits in DU or the curettage of DPS) and also the information necessary to make a prognosis defining the risk of recurrences.

Ulcers and Pain

Ulcers are often a source of moderate/severe pain, which may cause functional impairment and deeply affect patient’s health-related quality of life (HRQoL) [3]. Ulcer-related pain must be always analysed and classified (WUWHS classification, 2004) [4] (see Table 18.1).

Table 18.1 DU-related pain, classification, definitions and evaluation tools
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Assessing skin ulcers’ related pain is crucial to verify the effectiveness of the therapeutic approach and to plan a correct local treatment.

At baseline, the clinician must evaluate pain to decide an antalgic therapy which should be changed if the previous therapy was ineffective. An increased amount of necrotic tissue, infection and a not suitable dressing may be potential sources of pain. Therefore, the local approach should start by the removal of the dead or infected tissue. Moreover, an adequate dressing is mandatory to maintain the wound bed moist and protect the tissue. It is also important to assess the onset of critical ischaemia which can cause pain and need a systemic therapeutic approach to prevent its evolution to necrosis and/or gangrene.

During the change of the dressing, the detersion and the debridement of the wound bed, some procedural problems may be encountered. First, if the dressing is adherent to the wound bed, it is useful to use warm sterile saline solution to moist the attached medication and remove it gently. The use of medications that provide a warm moist environment without sticking to the wound bed is always recommended.

The detersion must be performed using warm (37 °C) sterile saline solution to avoid tissue thermal shock using a 10 ml syringe to reduce rinsing pressure on DU.

At least 15 min before debridement, the application of lidocaine/prilocaine ointments (cream 2.5%/2.5%) or gauze with lidocaine solution (2–4%) to control pain and to perform a safe and effective debridement is fundamental.

Instructions to Control DU-Related Pain

Background Pain

• Initiate/change antalgic therapy.

• Assess new onset of infection.

• Assess new onset of critical ischaemia.

• Evaluate RP severity and intensity (Raynaud’s Condition Score – RCS) [5].

• Assess efficacy of local therapy (change type of medication/gauze).

Procedural Pain

During Dressing Change

• Remove gently the previous dressing using warm sterile saline solution.

• Use nonadhesive medications.

• Use hydrogel which hydrates viable tissue and protects cutaneous nerve endings.

• Assess efficacy of local therapy (change type of medication/gauze).

During DU Detersion

• Rinse and irrigate the ulcer with warm (37°C) sterile saline solution.

• Reduce rinsing pressure using 10 ml syringe.

Before Sharp Debridement

• Application of lidocaine/prilocaine ointments (cream 2.5%/2.5%)

• Application of gauze with lidocaine solution (2–4%)

Ulcer Dimension and Depth

The ulcer dimension should be monitored over time to assess the healing process. Experts agree on the fact that a reduction of wound area from 20% to 40% in 2–4 weeks of treatment is a sign of a good healing process [6].

However, due to the small DU dimensions, it is difficult to ensure a reliable measurement. Therefore, to assess the DU dimension, it is necessary to use photographic records with standard anatomical reference points and unit of measurement adequately defined. Photographic records can be stored and used for research purposes only with patient’s written consent. Figure 18.1 reports DU measurement.

Fig. 18.1
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DU measurement

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The following staging of ulcers has been proposed:

  • Superficial: partial thickness skin loss involving epidermis (Fig. 18.2).

  • Intermediate: full thickness skin loss involving damage to, or necrosis of, subcutaneous tissue that may extend down to, but not through, the underlying fascia (Fig. 18.3).

  • Deep: full thickness skin loss with extensive destruction or damage to muscle down over the fascia to the bone. All the structures, tendon, joint capsule and bone are usually involved (Fig. 18.4).

Fig. 18.2
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Superficial – partial thickness skin loss involving the epidermis only

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Fig. 18.3
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Intermediate – full thickness skin loss involving necrosis of subcutaneous tissue

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Fig. 18.4
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Deep – full thickness skin loss down to the bone

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All these types of ulcers must be considered as potentially critical due to the high risk of infection and the evolution to severe complications as necrosis and gangrene [1].

The Wound Bed Preparation (WBP)

In SSc, the local therapy of ulcers is based on the principles of WBP , which has gained international recognition as a structured approach to the management of chronic wounds (a chronic wound is defined as a wound which lasts more than 6 weeks) [7]. The definition of WBP is “the management of an ulcer in order to accelerate endogenous healing or to facilitate the effectiveness of other therapeutic measures”.

To explain the correct strategy to approach an ulcer, the acronym TIME was developed in 2002 by a group of experts as a practical guide to summarize the four main components of WBP [8]:

  • T: Tissue management

  • I: Control of infection and inflammation

  • M: Moisture imbalance

  • E: Advancement of the epithelial edges of the wound

The TIME approach is a strategical framework which is a useful and practical tool to identify the main elements that should be considered to achieve a steady healing and to carry out a plan of care apt to promote wound healing.

Tissue Management “T”

The assessment of the tissue consists in a careful observation of the characteristics of the ulcer considering its bed, edges and the perilesional skin. The primary goal of this step is the identification of the main barriers to a steady healing that are the biofilm, the bioburden, the slough and the nonviable or deficient tissue (Table 18.2).

Table 18.2 Barriers to healing in SSc cutaneous ulcers
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The treatment of tissue is carried out following two different steps:

1. The first step is detersion – the mechanical removal of dirt, cellular debris, necrotic tissue, remnants of previous dressings and other wastes present on the wound bed and on the surrounding skin [14, 15]. It can be performed by irrigating with a warm (37 °C) saline solution (NaCl 0.9%) and using a 35 ml syringe and 19 G needle for lower limb ulcers and a 10 ml syringe and a 19G needle cannula for all the other types of ulcers, modulating the strength applied on the plunger (Fig. 18.5).

Fig. 18.5
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Detersion of a fingertip digital ulcer in a SSc patient

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Fig. 18.6
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Sharp debridement in a DU

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Instructions for Detersion

• Detersion – irrigation of the wound with saline solution (NaCl 0.9%) and applying a pressure ranging from 8 to 15 psi (using a 35 ml syringe and a 19G needle). This procedure allows an efficient detersion without damaging the granulating tissue.

• The detersion may be extremely painful for SSc patients; in these situations it is mandatory to decrease remarkably the irrigating pressure. This is possible using a 10 ml syringe and a 19G needle cannula modulating the strength applied on the plunger.

• In order to avoid vasospastic attacks, the irrigating solution must be warmed (37°C).

2. The second step is debridement which is defined as follows: “The removal of necrotic material, eschar, nonviable tissue, infected tissue, slough, pus, foreign bodies, cellular debris, bone fragments or any other kind of bioburden from a wound in order to promote its healing” [8]. Thus, debridement mainly consists in the removal of nonviable material, foreign bodies and necrotic tissue from a wound. Surgical resection of viable tissue or surgical amputation is not included in the debridement procedure. Debridement procedure must be carried out also on wound edges and perilesional skin. There are five types of debridement:

  • Passive debridement – It is based on the enhancement of the physiological and endogenous processes of debridement naturally occurring in a healing wound.

  • Active debridement – It is carried out by a physician using specific surgical tools.

  • Selective debridement – It consists on the selective removal of nonviable tissue, preserving viable material and granulating tissue.

  • Nonselective debridement – It consists on the removal of healthy or/and nonviable tissue.

  • Maintenance debridement [7] – In chronic wounds, in which the normal process of healing has been disrupted, the necrotic burden continually accumulates on the ulcer surface. In these cases, it may be more appropriate to perform regular or even continuous debridement. In SSc, the maintenance debridement is mandatory on fingertip DU, due to the continuous and fast production of bioburden.

The debridement is recommended in all types of SSc wounds. The assessment of the clinical features of a wound (e.g. presence/absence of biofilm, slough, infection, etc.) is essential to choose the adequate type of debridement. In addition many other factors have to be taken into account such as the patient’s general health status, the ability of the caregiver and the presence and intensity of wound-related pain, patient’s age and HRQoL [8].

Principal Goals of Debridement in SSc Ulcers

• Removing all barriers to healing

• Decreasing the amount of exudate

• Decreasing wound smell

• Reducing the risk of infection

• Decreasing the pain intensity (necrotic/nonviable tissue produces algogenic toxins)

• Promoting the proliferation of viable and granulating tissue

• Improving patient’s HRQoL

Table 18.3 shows the most frequent methods of debridement in SSc. They are frequently performed in association. In Table 18.4, the instructions for an efficacious debridement are displayed.

Table 18.3 Methods of debridement in SSc ulcers
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Fig. 18.7
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Sharp debridement in a calcinosis

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Table 18.4 Instructions for debridement
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Fig. 18.8
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DU covered by slough

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Identification of Signs and Symptoms of Infection and Treatment “I”

In WBP, the correct approach to an inflamed or/an infected ulcer is pivotal to avoid the delay or even the block of the healing process.

Inflammation

It is a physiological response to tissue damage and it leads the way to wound healing. However, excessive or inappropriate inflammatory response – common in infection – can have serious consequences for the patient. Inflammation is not only related to physiological healing or to infection process. A persistent inflammation can lead to a stall of the healing process, favouring its chronicization and the block of wound healing. In chronic wounds, studies underline the fact that inflammation phase may become a disrupting event: in fact, fibroblasts from chronic wounds are dysfunctional; they show a premature senescence, and they are not responsive to growth factors. Fibrin on the wound bed seems to block the production of growth factors. The exudate of chronic wound exudate shows an increased activity of matrix metalloproteinases (MMPs), elastases and cytokines [19, 21, 22]. All these elements hinder the covering movement of the wound edges thus worsening the wound conditions.

Infection

Definition of “infection” is one of the most debated topics as there are many factors that take part in the development of infection where there is a critical bacterial wound colonization.

The infection process depends on the following:

  • Bacterial burden (number of microorganism on the wound bed)

  • Pathogen virulence (ability to produce toxins, invasiveness)

  • Host resistance (capability to resist to bacterial growth through an effective immune response)

The presence of microorganism on wound’s surface does not necessarily mean the presence of injury to the host. Contamination at wound site is common in any ulcer, and a constant number of bacteria are present on the wound bed usually not slowing the healing process. A real infection is a characterized by a critical bacterial colonization of the ulcer that can spread over the surrounding tissue: this is due to some concomitants, and usually this process follows a typical time continuum.

Biofilm

Most recent studies about the management of wound bed underlines the increasing importance of assessing and treating biofilms [23]. A biofilm is a complex microbial community, consisting of bacteria endowed in a protective matrix of sugars and proteins (glycocalyx). Biofilms are known to form on the surface of medical devices and are also found in wounds. Bacteria communities embedded within biofilm are partially protected from antimicrobials, environmental stresses and host’s immune responses. The interaction between those microorganisms and host tissue is parasitical: bacteria get stable attachment and nutrition. Biofilms are a major contributing factor to chronic inflammatory changes in the wound bed. The chronic inflammation benefits the organisms in the biofilm which gains a higher resistance against antimicrobial and immune activity (phagocytosis, immune complex). Biofilm removal is fundamental to improve wound condition and to lead towards healing: it can be eradicated through debridement, though it can be treated with less invasive techniques such combinations of surfactant and products with antimicrobial activity.

Diagnosis

Diagnosis of infection in chronic skin ulcer is based upon bacteriological examination after the removal of biological material from the wound. Culture is indicated to identify the microorganisms and to guide antibiotic therapy. Swab culture is the most frequently employed method for confirming wound infection.

In everyday clinical practice, when infection is suspected, a prompt action must be taken, without waiting for the culture response, in order to prevent progression. The clinical evaluation of the lesion and the general conditions of the patient are sufficient to set a therapeutic plan on empirical basis in order to quickly stop the infection process and the deterioration of the wound. Afterwards, the result of the culture will be useful to confirm the infection and to set up an antimicrobial therapy.

Local Treatment of Infected Ulcer

Infections in DU have to be prevented or treated as soon as possible because they can lead to gangrene, osteomyelitis or self-amputation [24]. For this reason, local treatment in chronic wounds can be undertaken also as prevention or when an infection is probable because of the presence of typical signs and symptoms but without the evidence of an infection in the culture. Local therapy is based on broad-spectrum antimicrobial dressings and does not cause microbial resistance [25]. Table 18.5 reports the most frequent antimicrobial local treatments.

Table 18.5 Main antimicrobial local treatments and their characteristics
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Fig. 18.9
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DU after several sessions of local therapy based on sharp debridement and application of adequate dressings

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In SSc, DUs are often infected because of their specific localization (touching people, objects, surfaces). Poor patient’s general conditions (malnutrition) and their hand disability increase the risk of infection. Moreover, the impairment of the immune response and sclerodactyly reduces the capacity to keep the hands clean [28]. In Table 18.6 the instructions for an efficacious management of infected ulcers are displayed.

Table 18.6 Instructions for proper management of infected ulcers
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Fig. 18.10
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Secondary dressing – application

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Moisture Balance: Maceration or Dryness “M”

Managing exudate in chronic wounds is fundamental: wound epithelialization is stimulated by a moist environment, but an excess of fluid can macerate the healthy skin and delay the healing for the high content of proinflammatory cytokines and metalloproteinases that decrease the healing progression. The increased proteolytic activity of chronic wound exudate is thought to inhibit healing by damaging the wound bed, degrading the extracellular matrix and aggravating the integrity of the peri-wound skin, while the high levels of cytokines promote and prolong the chronic inflammatory response seen in these wounds.

The quantity and quality of wound exudate are associated with several factors:

  • Ulcer surface

  • Type of wound

  • Stage in healing process

  • Infection

  • Oedema

  • Local treatments

High exudate volume is one of the problems of chronic wounds that, with smell too, highly affect the patient quality of life. Excessive exudate production brings to a loss of protein, worsening malnutrition and increasing oedema. Moreover it increases the risk of infection as it favours the conditions for the replication of microorganisms. Wound leakage causes high distress in patients because it makes the wound smelly and it can soil clothes. Wound bed dryness is a relevant problem which blocks wound healing and is frequent in SSc ulcers where it may be due to low blood provision typical of microcirculation pathologies. Table 18.7 shows the adequate management of dry wounds. Table 18.8 shows how to manage the fluid balance in SSc ulcers both for the location, for the microvascular damage and for the dysfunction of the connective tissue (Table 18.9).

Table 18.7 The most frequent medications to treat dry ulcers
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Fig. 18.11
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A periungual-infected ulcer

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Table 18.8 Instructions to balance fluid
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Table 18.9 Instructions to assess and treat the lesion edges and the perilesional skin in scleroderma ulcers
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Epidermis and Epithelial Edges “E”

The evolution of the healing process is shown by the growth of the epithelial edges. Assessing the characteristics of the epithelial growth allows understanding whether the ulcer is moving or is “blocked” into the state of chronic lesion. In a healing ulcer, actively proliferating keratinocytes form a line which progressively degrades in the bed made up of ripe granulation tissue. In chronic ulcers with a reduced tendency to recovery, epithelial cells often present phenotypic alterations and a reduced capacity of proliferation and migration [29]. Frequently, the chronic wound edge appears thickened with a typical “clifflike” outline. In addition, hyperproliferation of the edges interfering with the normal cell migration on the wound bed may be observed [30]. The hyperproliferation might be due to the inhibition of apoptosis (programmed cellular death) of keratinocytes and fibroblast [9].

The assessment of the outline of the edges has an important diagnostic value in particular when the edges do not adhere to the wound bed and the wound closure does not take place. The “undermining” of the edges should be always checked out following the “clock” method: the depth of the edges is probed clockwise with a sterile swab along the whole ulcer perimeter [31].

Digital pitting scars (DPS) are frequent and may manifest in the form of microareas of pinhole-sized depression and corneous deposit. The DU secondary to DPS often presents phenomena of undermining as well as a marked thickening and hardening of edges and perilesional skin.

The functionality of the edges also depends from the trophism and conditions of perilesional skin. For the perilesional skin, the following aspects are considered:

  • Colour: red/erythematous skin (infection/inflammation), pale skin (ischemia), yellowish skin (hyperkeratosis), cyanotic skin (hypoperfusion)

  • Temperature: warm skin (infection/inflammation), cold skin (hypoperfusion)

  • Dryness: callosity, hyperkeratosis, hardening

  • Maceration: white-greyish skin, softening, wrinkling

  • Integrity: epithelial stripping, microlesions (skin tear)

  • Pain and tenderness

Education of SSc Patients Affected by Ulcers

The optimal medical strategy for an SSc patient affected by ulcers includes a local and a systemic approach. This synergism promotes skin perfusion and trophism leading to a marked improvement of the patient’s condition and to the ulcers’ healing.

Nevertheless, to assure lasting results and to properly manage chronic wounds, it is necessary to achieve full patient participation and adherence to the treatments. This means that the clinicians must provide therapeutic educational interventions about dressing, protection of the extremities, self-management and measures to prevent the onset of new DUs. The therapeutic education process includes a thorough evaluation of the patient and its caregivers to assess their skills in self-management.

Basic instructions for skin care and DU prevention and treatment are listed below. These basic skills must be provided to patients and their caregivers.

Moreover the therapeutic education must be focused on the practical skills for DU self-management. Information materials (brochures, videos and educative sessions) could be extremely useful.

A direct helpline led by rheumatology nurses specialized in wound care allows home dweller patient to manage DU treatment and to recognize severe complications on time. Information and support about DU prevention and systemic therapy are given if necessary.

All those measures allow patients’ active involvement in DU care and prevention to reduce hospital admission costs.

Life-Style Modifications for SSc Patients

• Wear gloves to reduce the intensity and the frequency of RP attacks when the temperature is cold (below 20 °C).

• Use cotton gloves in warm season too if the temperature is below 20 °C or in air-conditioned rooms [32].

• Protect dressings and medications on DUs using PVC or latex gloves to keep them clean, dry and in the correct position.

• It is mandatory to keep the hands clean.

• Check your hands’ conditions daily in order to point out dyschromic areas or any signs of inflammation. Inform wound care nurses about:

  • RP frequency, duration and related pain

  • Itch, erythema or any other kind of cutaneous manifestation

• Don’t smoke and don’t assume vasoconstrictive agents (caffeine) [33].

• Keep the skin moisturized (with ointments/creams).

• Don’t use aggressive soaps or detergents.