This feasibility study forms the first part of work to study the epidemiology of Epidermolysis Bullosa (EB) in the UK, and how often patients with EB see their General Practitioners (GPs) and how they are managed, including what treatment they receive.
EB is a group of rare inherited skin disorders that cause the skin to become fragile and form blisters. It is usually diagnosed in babies and young children and in severe cases, can lead to early death. Little is known about how many people live with EB and how they are treated in the UK.
The work set out here proposes to examine the case definition of EB in primary care data using CPRD (Clinical Practice Research Datalink) Aurum and Gold, and in hospital level data using HES APC (Hospital Episode Statistics Admitted Patient Care). This preliminary work will establish a clear definition of EB which will be applied in a subsequent full CPRD application. Additionally, we will explore the definition of EB by key socioeconomic data including age, gender, ethnicity and Index of Multiple Deprivation (IMD). Once a clear definition is established, we will examine which comorbidities are most common in patients with an EB diagnosis.
The findings from this and the forthcoming full study are expected to enhance our understanding of the burden of EB in the UK, including case numbers, severity, treatments, and healthcare resources. This information will support future research, healthcare planning, and the development of new treatments for EB.
We aim to use CPRD Aurum and Gold, HES APC and IMD to explore the availability of data on definitions of EB according to age, sex, ethnicity, IMD quintile and common comorbidities.
Objectives are:
1. to examine counts of EB recorded by diagnosis in CPRD and/or HES APC, according to year, age group, sex, ethnicity and IMD quintile.
2. To help with the case definition, to examine the frequencies of patients with history of a related blistering diagnoses (e.g. Bullous pemphigoid, pemphigus, acquired EB)
3. To describe the frequency of the 10 most common comorbidities (from the Charlson Comorbidity index) and 3 comorbidities often associated with patients diagnosed with EB (depression, anxiety and squamous cell carcinoma (SCC)).
Only counts and percentages will be described.
HES APC linkage will allow us the fullest possible assessment of disease status and ethnicity.
The findings of this feasibility stage of the study will inform the development of a full CPRD protocol which we anticipate will contribute to the evidence of the burden of EB to the NHS and also to the development of new treatments for EB.
Epidermolysis Bullosa (EB), related blistering diagnoses (e.g. Bullous pemphigoid, pemphigus), and common EB comorbidities (as determined by the Charlson Comorbidity Index and SCC, anxiety and depression).
Zoe Venables - Chief Investigator - University of East Anglia
Charlotte Davies - Corresponding Applicant - University of East Anglia
Jane Skinner - Collaborator - University of East Anglia
Kathryn Richardson - Collaborator - University of East Anglia
Marta Kwiatkowska - Collaborator - University of East Anglia
HES Admitted Patient Care;Patient Level Index of Multiple Deprivation;Practice Level Index of Multiple Deprivation (Standard)