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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Bullous Pemphigoid Patients | Patients with bullous pemphigoid included in a retrospective observational study evaluating the relationship between disease severity and periostin and ECP expression in skin biopsy specimens. |
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| Measure | Description | Time Frame |
|---|---|---|
| Correlation Between BPDAI Score and Periostin Immunohistochemical Expression | 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. | Baseline (retrospective assessment of existing clinical and histopathological data) |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation Between Periostin Immunohistochemical Expression and Serum Total IgE Levels | 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. | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data |
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Inclusion Criteria:
Exclusion Criteria:
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Adult patients diagnosed with bullous pemphigoid and followed at the University of Health Sciences, Istanbul Training and Research Hospital. Participants will be identified retrospectively from hospital records and pathology archives. Clinical, laboratory, and histopathological data, including BPDAI scores, serum IgE levels, peripheral eosinophil counts, and immunohistochemical expression of periostin and eosinophilic cationic protein, will be evaluated.
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| Name | Affiliation | Role |
|---|---|---|
| Ayse Esra Koku Aksu, Prof.Dr. | University of Health Sciences, Istanbul Training and Research Hospital | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| S.B.Ü. İstanbul Eğitim ve Araştırma Hastanesi | Istanbul | FATİH | 34096 | Turkey (Türkiye) |
Individual participant data (IPD) will not be shared. This retrospective, single-center study uses patient data collected as part of routine clinical care. Data sharing is restricted due to institutional policies, ethical considerations, and patient confidentiality requirements.
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Archived formalin-fixed paraffin-embedded (FFPE) skin biopsy specimens obtained from patients with bullous pemphigoid. Samples will be used for immunohistochemical evaluation of periostin and eosinophilic cationic protein (ECP). No DNA extraction will be performed.
| Correlation Between Eosinophilic Cationic Protein Immunohistochemical Expression and Peripheral Blood Eosinophil Count | 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. | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data) |
| Correlation Between Periostin Expression and Disease Relapse Frequency | 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. | Baseline (retrospective assessment of existing clinical and histopathological data |
| Correlation Between Periostin Immunohistochemical Expression and Hospitalization History | 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. | Baseline (retrospective assessment of existing clinical and histopathological data) |
| Correlation Between Eosinophilic Cationic Protein Expression and Disease Relapse Frequency | 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. | Baseline (retrospective assessment of existing clinical and histopathological data) |
| Correlation Between Eosinophilic Cationic Protein Immunohistochemical Expression and Hospitalization History | 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. | Baseline (retrospective assessment of existing clinical and histopathological data) |
| Correlation Between BPDAI Score and Eosinophilic Cationic Protein Expression | 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. | Baseline (retrospective assessment of existing clinical, laboratory, and histopathological data) |
| ID | Term |
|---|---|
| D010391 | Pemphigoid, Bullous |
| D004802 | Eosinophilia |
| ID | Term |
|---|---|
| D012872 | Skin Diseases, Vesiculobullous |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
| D007960 | Leukocyte Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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