Bullous Pemphigoid (BP)
Conditions
Keywords
bullous pemphigoid (BP), Periostin, Eosinophilic Catyonic Protein, BPDAI, IgE, Eosinophilia, Autoimmune Blistering Disease
Brief summary
Bullous pemphigoid is an autoimmune subepidermal blistering disease characterized by eosinophilic inflammation and variable disease severity. This retrospective observational study aims to evaluate the relationship between disease severity and histopathological features, serum IgE levels, peripheral eosinophil counts, and the expression of eosinophilic cationic protein (ECP) and periostin in skin biopsy specimens of patients with bullous pemphigoid. Immunohistochemical staining for ECP and periostin will be assessed semi-quantitatively and correlated with clinical and laboratory parameters, including BPDAI scores, eosinophil counts, serum IgE levels, hospitalization history, and disease relapse. The study seeks to identify potential tissue biomarkers associated with disease activity and severity in bullous pemphigoid.
Detailed description
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease and is characterized by autoantibodies directed against hemidesmosomal proteins. Eosinophils and eosinophil-associated mediators are believed to play a central role in disease pathogenesis. Periostin and eosinophilic cationic protein (ECP) have been implicated in eosinophilic inflammation; however, their relationship with clinical disease severity in BP remains incompletely understood. This single-center retrospective observational study will include patients diagnosed with bullous pemphigoid at the University of Health Sciences, Istanbul Training and Research Hospital. Clinical, laboratory, and histopathological data will be collected from medical records. The study will evaluate demographic characteristics, disease severity measured by BPDAI, serum IgE levels, peripheral eosinophil counts, hospitalization history, relapse frequency, and other clinicopathological parameters. Archived skin biopsy specimens will undergo immunohistochemical staining for periostin and ECP. Staining intensity will be assessed using semi-quantitative scoring methods and correlated with clinical and laboratory findings. The primary objective is to determine whether periostin and ECP expression levels are associated with disease severity. Secondary objectives include evaluating correlations between tissue biomarker expression and serum IgE levels, eosinophil counts, hospitalization, and disease recurrence.
Interventions
None listed
Sponsors
Istanbul Training and Research Hospital
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Age ≥18 years. * Diagnosis of bullous pemphigoid confirmed by compatible clinical, histopathological, and/or direct immunofluorescence findings. * Availability of archived skin biopsy specimens suitable for immunohistochemical evaluation of periostin and eosinophilic cationic protein (ECP). * Availability of clinical and laboratory data, including BPDAI score, serum IgE level, and peripheral eosinophil count.
Exclusion criteria
* Age \<18 years. * Diagnosis other than bullous pemphigoid. * Inadequate or unavailable biopsy specimens for immunohistochemical analysis. * Missing clinical, histopathological, or laboratory data required for study analyses.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Correlation Between BPDAI Score and Periostin Immunohistochemical Expression | Baseline (retrospective assessment of existing clinical and histopathological data) | Correlation between BPDAI disease activity score and periostin immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens from patients with bullous pemphigoid. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Correlation Between Periostin Immunohistochemical Expression and Serum Total IgE Levels | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data | Correlation between periostin immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and serum total IgE levels (IU/mL) in patients with bullous pemphigoid. |
| Correlation Between Eosinophilic Cationic Protein Immunohistochemical Expression and Peripheral Blood Eosinophil Count | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data) | Correlation between eosinophilic cationic protein (ECP) immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and peripheral blood eosinophil count (cells/µL) in patients with bullous pemphigoid. |
| Correlation Between Periostin Expression and Disease Relapse Frequency | Baseline (retrospective assessment of existing clinical and histopathological data | Correlation between periostin immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and disease relapse frequency (number of relapses) in patients with bullous pemphigoid. |
| Correlation Between Periostin Immunohistochemical Expression and Hospitalization History | Baseline (retrospective assessment of existing clinical and histopathological data) | Correlation between periostin immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and hospitalization history (yes/no) in patients with bullous pemphigoid. |
| Correlation Between Eosinophilic Cationic Protein Expression and Disease Relapse Frequency | Baseline (retrospective assessment of existing clinical and histopathological data) | Correlation between eosinophilic cationic protein (ECP) immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and disease relapse frequency (number of relapses) in patients with bullous pemphigoid. |
| Correlation Between Eosinophilic Cationic Protein Immunohistochemical Expression and Hospitalization History | Baseline (retrospective assessment of existing clinical and histopathological data) | Correlation between eosinophilic cationic protein (ECP) immunohistochemical expression measured using a semiquantitative 0-3 staining score in skin biopsy specimens and hospitalization history (yes/no) in patients with bullous pemphigoid. |
| Correlation Between BPDAI Score and Eosinophilic Cationic Protein Expression | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data) | Correlation between BPDAI disease activity score and tissue eosinophilic cationic protein (ECP) expression assessed by immunohistochemistry using a semiquantitative 0-3 staining score in skin biopsy specimens from patients with bullous pemphigoid. |
Countries
Turkey (Türkiye)
Contacts
STUDY_CHAIRAyse Esra Koku Aksu, Prof.Dr.
University of Health Sciences, Istanbul Training and Research Hospital
Outcome results
None listed