Overview

Pyoderma Gangrenosum (PG) is a rare, long-lasting (chronic) skin disease. It is characterised by intensely painful sores (ulcers) that are difficult to diagnose and treat.¹ These ulcers often lead to permanent scarring, which affects physical appearance and self-image. The disease progression is also unpredictable, varying from causing very little pain to rapidly spreading.²

The chronic and unpredictable nature of the disease can have a negative effect on the emotional and mental well-being of people with PG. The rates of anxiety and depression are higher in people with PG compared to the general population.² Despite this, the psychological effects are often overlooked, with emphasis put on the physical treatments. However, a more holistic approach is needed, one that addresses the physical, emotional, and mental well-being of people with PG.³

Understanding pyoderma gangrenosum

PG is a chronic skin condition. It can be classified into several subtypes, including ulcerative, bullous, and pustular.⁴ The ulcerative subtype is the most common form and causes large, deep ulcers with dead tissue at the centre. The disease initially starts with painful, raised spots (papules) or pus-filled spots (pustules) that progress into deep ulcers.³ The painful skin ulcers typically affect the lower legs.² When these ulcers heal, they leave behind scars that have a characteristic criss-cross pattern called a cribriform scar.⁵ The scarring is permanent and can be disfiguring. People with PG can also have an exaggerated response to minor skin trauma. This phenomenon is known as pathergy.⁵ Skin injuries that are insignificant for most people can cause distress for some people with PG.

PG is described as a relapsing and remitting disease, meaning it has periods of flare-ups (relapse) and periods where the disease is controlled (remission).² This makes the condition unpredictable, causing distress to affected individuals. The wounds in PG are slow to heal, taking an average of 5 months after diagnosis.⁶ This can contribute to ongoing pain and disability, lead to the use of more treatment interventions, and impact daily functioning.

Emotional and psychological effects

Pain is an important factor that influences the quality of life (QoL) and psychological well-being of people with PG.² Pain can cause poor sleep quality, problems with mobility, and limitations in completing daily activities. It may also impair the person's sense of self-control. Pain may exacerbate low mood and anxiety, and can affect the ability to function socially.²

Affected individuals can also experience anxiety.² This can be related to delays in diagnosis, disease progression, and admissions to hospital. Even when the ulcer is not causing symptoms, in the remission phase, individuals may worry that the condition will recur.² Previous traumatic experiences caused by their condition, such as significant bleeding and life-threatening infections, can cause individuals to worry about death. Individuals can also have feelings of loss of control and worries that the healing of their ulcer will not occur.²

Depression is a common associated condition in adults with PG, with estimated rates up to 23% across studies.⁷ A depressed mood has been attributed to intense pain, reduced physical and social functioning, and concerns about recovery.² Individuals affected by these factors can experience suicidal thoughts, despite never having a mental health condition before they were diagnosed with PG.² Individuals may experience feelings of low mood, helplessness, and hopelessness. As PG can significantly impact a person's ability to carry out daily activities, people with PG may feel "useless" as they are unable to be physically active and complete tasks they used to do.²

Impact on self-image and identity

PG causes chronic wounds and visible skin lesions such as skin ulcers and scars. These skin manifestations can be disfiguring, impacting the person's physical appearance. This can result in reduced self-esteem, poor body image, and low self-confidence.² It may also lead individuals to isolate themselves from social contact, and they may experience stigma. This further perpetuates their poor mental health state. The wounds can have an offensive smell or may leak fluid. This can exacerbate or trigger anxiety, depression, and social isolation.¹

Social and interpersonal challenges

PG can also affect social functioning. It can put a strain on relationships with partners.² The physical, mental, and social impairments can negatively impact their relationship. Individuals may feel self-conscious due to their visible skin symptoms. This can impact sexual intimacy.

It can also put a strain on relationships with family.² Persistent pain and reduced sleep can lead individuals to have emotional outbursts towards their family members.

PG can cause challenges in work, particularly in jobs requiring high physical demands.² This can be an issue, specifically during flare-ups of the condition, where pain can limit their work activities. Affected individuals may also take prolonged absence from work due to hospitalisations.²

Coping strategies and psychological support

As PG can significantly impact an individual's emotional and mental well-being, there is a need for a holistic approach when managing PG that includes psychological support.² The high rates of anxiety and depression in people with PG reinforce the importance of treatments that not only target physical symptoms but also psychological symptoms of the condition.³

Coping strategies are a way for affected individuals to take control of their condition. They can help reduce emotional distress and aid adaptation to current disease challenges.⁸ Individuals with PG may find the following coping strategies useful when dealing with PG:²

• Positive mind framing: Thinking about their current situation in a more positive way
• Distraction: Shifting attention away from distressing thoughts or symptoms
• Social connection: Actively connecting with their social network

Another strategy that has been helpful for people with other chronic skin diseases is mindful attention. This involves focusing on the present moment, which can aid in dealing with distressing situations.⁸

Role of healthcare professionals

Healthcare professionals play an important role in helping people with PG understand their condition and feel supported during their care.

Patient education is essential in PG management. This can be achieved by providing information leaflets for pain management and wound care.² It is also important for healthcare providers to increase their knowledge of PG. As PG is a rare condition, individuals may have to explain the condition to multiple healthcare professionals. This can cause stress for people with PG during consultations.²

People with PG should also be made aware of patient-to-patient support groups.² These groups allow affected individuals to connect with others who have similar experiences. This can help with coping strategies and emotional support.

People with PG may not be open about their emotional struggles with healthcare professionals.² They may instead only focus on their physical symptoms when presenting to healthcare settings. It is therefore important to ask questions about mental health sensitively and empathetically, and to create a comfortable environment for individuals to express their feelings.

Improving quality of life

Studies have demonstrated that in comparison to healthy individuals, people with PG experience a lower QoL.⁴ This is influenced by whether individuals feel satisfied with the treatment provided. Pain and depression both have an impact on patients' treatment satisfaction, emphasising the need to target these factors in PG management.⁹ Improvements in QoL are associated with effective control of symptoms and the ability to balance treatment with daily life. If a treatment needs more monitoring or causes excessive side effects, it may negatively affect the individual's ability to perform daily activities.⁴ Therefore, treatment should be tailored to the affected individual and should be effective and manageable.

Setting realistic goals and expectations is important. Healthcare professionals should clearly communicate how they expect the disease to progress and set realistic outcomes with patients.² This can help to reduce stress and actively involve individuals in their own care. This can improve how well individuals cooperate with healthcare services and adhere to their treatment plan.

Summary

PG is a chronic skin disease that causes significant physical and mental effects. The chronic and unpredictable nature of the condition leads to distress, affecting daily functioning. Living with chronic wounds and visible skin lesions causes psychological manifestations, most commonly anxiety and depression. The visibility of the lesions can impact self-image, resulting in low self-esteem, poor body image, and low self-confidence. PG can have social implications, resulting in social isolation and affecting employment. PG can also take a toll on romantic and family relationships. This can further perpetuate psychological effects. Pain is one contributing factor to reduced QoL and negative psychological effects. Therefore, appropriate physical treatment is paramount to improve QoL. A comprehensive approach to PG management is important: one that addresses physical, psychological, and social effects. Healthcare professionals play a key role in patient education through information leaflets, directing patients to support groups, and assessing for mental health conditions in patients with PG. In addition, individuals should be actively involved in their own care, such as setting realistic goals and expectations with their clinician. Coping strategies can help individuals take control of their condition and improve their day-to-day lives. With appropriate physical and psychological treatment, QoL can be improved through bettering mood and daily functioning.