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Published on: December 7, 2018
Cognitive function in patients with epidermolysis bullosa: social adjustment and emotional problems
R Feldmann1, J Weglage, M Frosch
1University Hospital, Pediatrics, Münster, Germany. feldrei@uni-muenster.de
Insights
Children with Epidermolysis bullosa (EB) may face cognitive and social challenges. Recessive dystrophic EB (RDEB) is linked to significant cognitive deficits, requiring targeted rehabilitation and support for all EB patients.
Area of Science:
- Pediatric Psychology
- Dermatology
- Neurodevelopmental Disorders
Background:
- Epidermolysis bullosa (EB) is a severe genetic skin disorder causing chronic wounds and significant distress in children.
- Beyond physical challenges, EB is associated with potential cognitive, social, and emotional difficulties.
Purpose of the Study:
- To evaluate cognitive abilities in children and adolescents with Epidermolysis bullosa.
- To assess social and emotional problems reported by parents of EB patients.
Main Methods:
- A cohort of 20 children and adolescents with EB (ages 6-17) and 24 healthy controls were assessed using Wechsler Intelligence Scales.
- Parents completed the Achenbach Child Behaviour Checklist (CBCL) to report on their child's emotional and behavioral symptoms.
Main Results:
- While overall cognitive scores did not significantly differ between EB patients and controls, severe generalized recessive dystrophic EB (RDEB) was associated with considerable cognitive deficits.
- The entire group of EB patients exhibited lower social competence and more social-emotional problems compared to controls.
Conclusions:
- Children with severe RDEB require functional rehabilitation to support cognitive development alongside physical healing.
- All children and adolescents with EB subtypes need therapeutic interventions to address social and emotional well-being.
Background:
Children with Epidermolysis bullosa (EB) suffer from an intractable, burdensome skin disease that may result in cognitive as well as social and emotional problems.
Patients:
To assess cognitive problems in patients with EB, we investigated 20 affected children and adolescents, 6-17 years of age (mean: 10.8 years; SD: 3.4 years), and 24 healthy controls (6-15 years, mean: 10.9 years; SD: 3.0 years) for cognitive abilities. Additionally, parents were asked to assess social and emotional problems of their children.
Methods:
Patients and controls were assessed using the Wechsler Intelligence Scales. Parents completed Achenbach's Child Behaviour Checklist (CBCL), a parent-report measure on emotional and behavioural symptoms in children.
Results:
The mean scores of the young patients tested with WISC-R and WAIS-R showed no significant left shift if compared to healthy controls. Analyzing the subtypes of EB, however, considerable cognitive deficits were associated with recessive dystrophic EB (RDEB), severe generalized. Less social competence and more social and emotional problems were reported for the entire patient group.
Conclusions:
Functional rehabilitation is required to increase not only the physical but the cognitive development of the severely affected children with RDEB. Children and adolescents with all subtypes of EB require therapeutic support regarding their social and emotional life.
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