Table 1 Recommendations summary for the Psychosocial care of individuals living with EB

A multidisciplinary approach in treating EB improves QoL for individuals with EB

 • Psychological support and close monitoring of EB improves QoL.

 • They facilitate participation in social activities.

 • Patients with all types of EB including EBS report a great impairment in QoL due to restrictions in physical and social activities.

✓

Adults, children (n = 12/185) EBS; JEB; DDEB; RDEB; KS

Unclear if adults, children or both (n = 43/134) EBS; JEB; DDEB; RDEB;

Adults, children (n = 120/248) EBS; JEB; DDEB; RDEB

Review of inherited & autoimmune blistering diseases

B

2++

1-

2+

2+

2-

1-

[29]

[30]

[31]

[28]

[32]

To promote self-efficacy and support around body image to aid psychological well-being

 • Having access to knowledge and resources about EB can help people have a greater role in managing their EB. This self-management can help improve well-being.

 • Improved self-efficacy and locus of control, as well as support around body-image could help to develop a more positive sense of well-being.

For support during transition periods in life (school transitions, transition into adulthood)

 • Communication and education about EB to improve people’s understanding.

 • Support from families, EB healthcare professionals and DEBRA.

Review of inherited &autoimmune blistering diseases

Adults (n = 87) RDEB, DEB, EBS

Children 10–14 years old (n = 11) EBS (autosomal recessive)

Young male adults (aged 21–35 years) with RDEB (n = 5) and EBS (n = 2)

Observational report

C

2+

1-

2+

2+

4

4

[32]

[33]

[34]

[35]

[36]

People diagnosed with EB should be referred for psychosocial support as early as possible in childhood or in adulthood, if the person with EB wishes

• To support the family unit.

Encourage supportive network for the family, for example:

 • Education about EB for others

 • Provide access to DEBRA (or other EB support groups)

✓

Children (n = 11/82) EBS; JEB; DDEB; RDEB

Children (n = 16) JEB Adult (n = 1) RDEB

C

2-

2-

2-

4

[37]

[38]

[39]^α

Pain is present for most children and adults with EB (all types) with profound psychosocial impact:

 • Activity related pain can significantly affect psychosocial well-being and QoL (e.g. fear of/actual pain restricting social activities, affecting relationships with family and friends).

 • Treatment related pain can make managing EB harder and link to procedural anxiety.

Adequate holistic pain management is essential as a focus for helping people with EB:

 • Following pain guidelines.

 • Offering approaches to help people with EB cope emotionally.

 • Help with managing the impact of pain and the interlinked cycle of pain and psychosocial challenges.

Adults, children(n = 374) EBS, JEB, DDEB, RDEB

Adults (n = 6) JEB, DDEB

Children; (n = 11) EBS, JEB, DDEB, RDEB,

Adults (n = 30) children (n = 27) EBS

Children/families (n = 70) type of EB unclear

Adults (n = 43) EBS, JEB, DDED, RDEB

Unclear if adult/child (n = 40)

EBS, JEB, DDEB, RDEB

Best practice guideline

Children (n = 11) EBS, JEB, DDEB, RDEB

Adult (n = 1) RDEB

C

2-

2+

2-

2-

2-

2-

2+

2+

⇒

2+

3

[3]

[40]

[37]

[42]

[41]

[30]

[44]

[7]^α

[17]

[43]

People with EB need support to cope with EB, and their ways of coping need to be supported by others: participation in social life needs to be supported

 • Such as at school, the community, friendships, employment.

 • Aid access to supportive networks.

 • Public education campaigns to help those around them to understand EB and their needs.

Promote a sense of self-management of their EB

 • This can help bring a sense of control over certain aspects of the disease/treatment and pain.

Build social skills and communication

 • Help in learning how to communicate about EB to others and within the family unit.

✓

Children (n = 27) DDEB; (n = 28) RDEB

Children 10–14 years old (n = 11) EBS (autosomal recessive)

Children (n = 11/82)

EBS; JEB; DDEB; RDEB

Children (n = 24)

EBS; JEB; DDEB; RDEB

C

2-

2+

2+

2-

2-

[45]

[34]

[37]

[46]

Encourage access to, and a collaborative ‘working together’ relationship with, an expert multi-disciplinary team of professionals.

 • Facilitate access to multidisciplinary professional support for medical and psychosocial care across the lifespan.

 • At both specialist centres and community services

✓

Adults, children (n = 15) RDEB

Children (n = 21) EBS; JEB; DDEB; RDEB

Children (n = 11/82) EBS; JEB; DDEB; RDEB

HCPs (n = 33) 30 stakeholders (HCPs, and 9 with EB RDEB, DDEB, EBS)

Adults (n = 6) JEB, DDEB

Children and Adults (n = 20) EBS, JEB, DEB

Children (n = 16) JEB

Children (n = 20) EBS, JEB, RDEB

C

2-

1-

2+

2+

2+

2-

2-

2-

2-

2-

2+

[29]

[48]

[51]

[17]

[46]

[49]

[40]

[38]

[48]

[47]

Grades

 B

 C

 1-

 2++

 2+

 2-

 3

 4