A challenging landscape

2 April 2019

Atypical wounds include a broad spectrum of conditions caused by inflammation, infection, malignancy, chronic illnesses or genetic disorders. An atypical wound may be suspected if the wound has an abnormal presentation or location and does not heal following a good treatment plan. The European Wound Management Association (EWMA) discusses its work to increase awareness about the clinical picture, diagnosis and treatment of these wounds.

An author group, chaired by document editor Dr Kirsi Isoherranen, is currently working on a new EWMA document that will be published and launched at the EWMA 2019 conference in Gothenburg, Sweden. The document focuses on atypical wounds – those wounds that create the most challenging situations for clinicians and/or patients from prevention, treatment and organisational perspectives. The author group includes prominent and well-respected clinicians who have all volunteered to use their expertise to describe the aetiologies and treatment strategies of different types of atypical wounds.

The prevalence of atypical wounds can be as high as 10% of all wounds, and it is probable that many of these wounds are underdiagnosed. Typical challenges include considerable diagnostic delays and prolonged healing times – for example, inflammatory and vasculopathy wounds (such as pyoderma gangrenosum, an inflammatory neutrophilic disorder, and cutaneous vasculitis). In addition, many atypical wounds have an enormous impact on the quality of life in the affected individuals, and a multidisciplinary team approach is necessary to ensure patients receive high-quality treatment in a timely fashion.

Risk factors and diagnosis

The risk for atypical wounds is usually higher in elderly people with weaker immune systems and is associated with pre-existing chronic medical illness, infections, inflammations or tumours. Taking a number of prescribed medications and leading an unhealthy lifestyle can also increase the risk for developing these wounds.

Managing any type of wound successfully demands an accurate patient assessment using a multidisciplinary approach that moves beyond standard care. Comorbidities, medical history and social support networks should all be evaluated during this process. It is essential that the clinician is able to recognise common wound types a well as atypical characteristics in order to identify the best course of treatment. Problems with the assessment can result in failing to recognise the early signs of infection or wound deterioration, which can result in more expensive treatment, the use of antibiotics or hospital readmissions.

A systematic approach needs to be taken to determine wound aetiology and underlying causes, and thus obtain an accurate diagnosis. This often entails multiple steps, including a biopsy, which provides a histopathologic diagnosis and identifies a skin disorder that is not responding to current treatment.

Research published in Wounds recommends a biopsy for diagnosing inflammatory, microthrombotic and bullous disorders such as non-atherosclerotic ischemic ulcers, inflammatory conditions, malignancies, infections, autoimmune bullous disorders, venous ulcers, neuropathic ulcers, medication-induced wounds, pressure ulcers and traumatic wounds.

If a punch biopsy performed in an outpatient setting cannot confirm a suspected diagnosis in a wound that has failed other treatment measures, a surgical biopsy that can sample a larger area of tissue may be needed. In cases where the biopsy does not help diagnose the wound aetiology, the clinician should review the patient’s medical history again.

Some newer oncology drugs can also trigger skin reactions. Tracking the timing of chemotherapy is therefore essential, as it can be the cycle of the medication, not just the medication itself, causing the wound.

Atypical wound treatment

Normal wound care therapies tend not to be effective and when treating atypical wounds it is also essential to control the underlying disease process. This includes evaluating and managing wound tunnels. During treatment, it is important to understand proteases and inflammatory processes. Proteases are enzymes that break down proteins into peptides and amino acids. In wound healing, the major proteases are the matrix metalloproteinases (MMPs) and the serine proteases, such as elastase.

Types of atypical wounds

There are six main types of atypical wounds – inflammatory, vasculopathy, infection, malignancy, hereditary and genetic, and wounds of external cause. In terms of inflammatory wounds, pyoderma gangernosum is a condition characterised by skin cell death and destruction resulting from an unknown cause. It causes large painful ulcers to form, mainly on the legs, but they can also occur anywhere on the body as a secondary complication of any skin cut or trauma. An example is Bullous pemphigoid, a rare autoimmune inflammatory condition of unknown cause. This is where the immune system creates antibodies against its own tissue.

“Many atypical wounds have an enormous impact on the quality of life in the affected individuals, and a multidisciplinary team approach is necessary to ensure patients receive high-quality treatment in a timely fashion.”

These are formed against the junction between the upper and lower skin layers, leading to large clear fluid-filled blisters formation that are difficult to rupture. If this does occur, the blisters can become painful and sensitive. These are most frequent on the abdomen, groin, inner thighs and arms.

The amount of all wounds that can be classified as atypical wounds.
European Wound Management Association

The two key vasculopathy wounds are cryoglobulinemia and vasculitis. Cryoglobulinemia is a systemic inflammation primarily affecting the kidneys, joints and skin and is caused by deposits of immune complexes containing cryoglobulin. The condition leads to itchy, small red skin lesions and ulcers, particularly on the legs, and causes joint pain in fingers, hands, knees and ankles, bloody urine, general weakness, and decreased sensation in the extremities, as well as abdominal pain. Vasculitis is an inflammatory condition of the blood vessels due to unknown origin. It can be occurred throughout the body (known as systemic) or in one area (referred to as localised) and can impact all types of blood vessels. The presentation varies from mild redness and irritation to occlusion of blood vessels and ischemia of the affected area.

“Managing any type of wound successfully demands an accurate patient assessment using a multidisciplinary approach that moves beyond standard care. Comorbidities, medical history and social support networks should all be evaluated during this process.”

Infected wounds are where bacteria or other microorganisms have colonised, causing either a delay in wound healing or deterioration of the wound. These occur when the body’s immune defences are overwhelmed or cannot cope with normal bacterial growth. Most infected wounds are caused by bacteria, originating either from the skin, other parts of the body or the outside environment. Infection of wounds can also be caused by surgery, which represents a serious health risk. The vast majority of deaths of patients who have undergone surgery are caused by surgical site infections.

A malignant wound is also known as tumour necrosis, a fungating wound, ulcerating cancerous wound, or malignant cutaneous wound. These occur when cancerous cells invade the epithelium, infiltrate the supporting blood and lymph vessels, and penetrate the epidermis. This results in a loss of vascularity and nourishment to the skin, leading to tissue death and necrosis. Malignant wounds may take the form of a cavity, an open area on the surface of the skin, skin nodules or a nodular growth extending from the surface of the skin. They can present with odour, exudate, bleeding, pruritus and pain.

Genetic or hereditary wounds can be the result of single or multiple causes, including psychological factors. Examples include Dermatitis artefacta, a condition that presents with multiple superficial, self-inflicted skin lesions of variable shape, size and depth on accessible areas like the face, arms and abdomen. In such cases the patient tends to have a history of chronic skin conditions and either a personal or familial history of psychiatric conditions.

Wounds of external cause can be either primary or secondary. An example of the former is brown recluse spider wounds, which can initially go unnoticed but within a few hours progress to severe pain, itchiness and a clear fluid-filled cyst with a red surrounding border. The wound can also be accompanied by a number of other symptoms, such as nausea, vomiting, diarrhoea, fever and even seizures. Conditions such as radiation necrosis are usually secondary to treatment for existing central nervous system tumours, which are being treated using radiotherapy. The existing tumour makes the surrounding tissue more vulnerable to radiation, leading to the destruction of cells, causing further damage.

It is clearly highly challenging to treat atypical wounds, as they have diverse causes and do not respond to conventional wound therapies. The EWMA document will thus provide a hugely valuable resource in helping to optimise care for patients with these wounds.

Atypical wounds, as with other types of wounds, also require treatment by clinicians from a range of disciplines.
Kirsi Isoherranen speaks at the Atypical Wounds Session at EWMA2018.

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