Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Pemphigus and bullous pemphigoid (BP) are severe autoimmune blistering diseases (AIBD) that pose a critical need for new therapeutic approaches. Clinical trials in pemphigus and BP will require the availability of validated disease severity measures that can be used to define primary outcomes.
The disease severity instruments for pemphigus and BP, the Pemphigus Disease Area Index (PDAI) and the Bullous Pemphigoid Disease Area Index (BPDAI), respectively, have the potential to capture changes in all grades of disease activity, including mild disease, and therefore represent a substantial improvement over simple lesion counts or measurements of affected body surface area. However, full validation of the PDAI and BPDAI for use in clinical trials will require additional and more extensive measurements that will enable us to classify patients correctly by disease severity and be able to define the minimal change in score that is clinically significant.
This database study will compare the Pemphigus Disease Area and Severity Index (PDAI) and the Bullous Pemphigoid Disease Area Index (BPDAI) against other disease severity measures, including the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and a physician's global assessment. Additionally, patients will be asked to rate their overall disease severity using a visual analogue scale, the patient's global assessment. Participants will also have the option to donate blood samples to our AIBD blood bank for immunologic and pathophysiologic studies.
The purpose of this database study will also be to evaluate quality of life (QoL) measures and correlate these QoL measures with disease severity in patients with pemphigus and BP. QoL is an important and independent component of disease impact on patients. This database study will use the following QoL measures: the SF-36, which is a nonspecific QoL measure; the Skindez-29 and the Dermatology Life Quality Index (DLQI), which are dermatology-specific instruments; the Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires, which are AIBD-specific tools. The results will be utilized in planning future clinical studies.
The AIBD study will be conducted only at Penn at this time. Data from the study will be entered into a password protected, web-based database that is used only at Penn.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Questionnaires | Other |
| Measure | Description | Time Frame |
|---|---|---|
| Pemphigus Disease Area Index (PDAI) | one year | |
| Bullous Pemphigoid Disease Area Index (BPDAI) | one year |
| Measure | Description | Time Frame |
|---|---|---|
| Autoimmune Bullous Disease Quality of Life (ABQOL) | one year | |
| Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) | one year | |
| Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Adult patients over the age of 18 who have been diagnosed with pemphigus vulgaris, pemphigus foliaceus or bullous pemphigoid.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Victoria P Werth, MD | Contact | 215-615-2940 | werth@pennmedicine.upenn.edu | |
| Joyce Okawa, RN | Contact | 215-615-2960 | Joyce.Okawa@pennmedicine.upenn.edu |
Not provided
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital of the University of Pennsylvania, Department of Dermatology | Recruiting | Philadelphia | Pennsylvania | 19104 | United States |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D010392 | Pemphigus |
| D010391 | Pemphigoid, Bullous |
| ID | Term |
|---|---|
| D012872 | Skin Diseases, Vesiculobullous |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
Not provided
Not provided
| ID | Term |
|---|---|
| D011795 | Surveys and Questionnaires |
| ID | Term |
|---|---|
| D003625 | Data Collection |
| D004812 | Epidemiologic Methods |
| D008919 | Investigative Techniques |
| D017531 | Health Care Evaluation Mechanisms |
Not provided
Not provided
Not provided
Not provided
Not provided
Blood specimens will be collected during regularly scheduled clinic visits. The specimens will be processed and stored at the research labs belonging to the principal investigator at the University of Pennsylvania and the Philadelphia VA Medical Center. The specimens will not be publicly available and will be coded and stored in a locked facility equipped with a freezer.
| one year |
| Hospital of the University of Pennsylvania | Recruiting | Philadelphia | Pennsylvania | 19104 | United States |
|
| University of Pennsylvania, Department of Dermatology | Recruiting | Philadelphia | Pennsylvania | 19104 | United States |
|
| D007154 | Immune System Diseases |
| D011787 | Quality of Health Care |
| D017530 | Health Care Quality, Access, and Evaluation |
| D011634 | Public Health |
| D004778 | Environment and Public Health |