The aim is to investigate the differences in pathogenesis of nonbullous and bullous pemphigoid by comparing gene expression, and the presence of activated and apoptotic eosinophils and IL-31 expression in skin, blood and blister fluid of both…
ID
Source
Brief title
Condition
- Autoimmune disorders
- Epidermal and dermal conditions
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
1. Differences in gene expression levels in the skin of healthy persons,
nonbullous pemphigoid patients and bullous pemphigoid patients by RNA
sequencing.
2. Differences in the number of activated and apoptotic eosinophils and IL-31
expression in the skin and blood between healthy controls, nonbullous
pemphigoid and bullous pemphigoid by immunofluorescence staining and
flowcytometry .
Secondary outcome
not applicable
Background summary
Pemphigoid is the most common autoimmune bullous disease and typically presents
with severe itch and bullae on the skin (bullous pemphigoid). Pemphigoid most
commonly affects the older patients and is associated with an increased risk of
mortality, as well as a significant decline in quality of life and
psychological well-being. Besides the typical bullous presentation, pemphigoid
can also present without blisters, named nonbullous pemphigoid. Nonbullous
pemphigoid can be difficult to recognize for doctors and results in a delay of
treatment. To date the exact pathogenesis of pemphigoid is still not completely
unravelled, and it is unknown what causes the differences in phenotype.
Study objective
The aim is to investigate the differences in pathogenesis of nonbullous and
bullous pemphigoid by comparing gene expression, and the presence of activated
and apoptotic eosinophils and IL-31 expression in skin, blood and blister fluid
of both disease phenotypes.
Study design
Observational study
Study burden and risks
Three skin biopsies of nonbullous pemphigoid and bullous pemphigoid will be
obtained by means of a 4mm punch biopsy under local anesthesia. It is a
generally safe procedure with minimal burden to the patient. Possible
complications of bruising, bleeding, infection and scarring rarely occur. Skin
samples of healthy controls will be obtained from left over skin of breast
reduction surgery. Furthermore two extra blood samples will be derived from
nonbullous and bullous pemphigoid patients during a venapunction for drawn for
standardcare purposes. Blood from healthy subjects for control is available in
our laboratory. In the patients with bullous lesions we will extract blister
fluid by using a seringe. This is a painless procedure and there are no
expected complications as blisters are punctured more often in pemphigoid
patients for pain relief.
Hanzeplein 1
Groningen 9700RB
NL
Hanzeplein 1
Groningen 9700RB
NL
Listed location countries
Age
Inclusion criteria
1. Written informed consent.
2. >=18 years old.
3. Patients that are recently diagnosed with pemphigoid (bullous or nonbullous) or pemphigoid patients that were in complete remission without therapy and experience a relapse can be included.
[The following diagnostic criteria are used for cutaneous pemphigoid: a positive DIF with linear IgG and/or C3c along the BMZ, and/or positive IIF on SSS or monkey esophagus, in combination with compatible clinical presentation, histopathological findings, or other immunoserological tests.]
If the criteria are fulfilled, patients will be categorized into the nonbullous phenotype (no history and no current blistering on the skin) or the bullous phenotype.
4. Active disease with skin lesions.
Exclusion criteria
1. The use of systemic immunosuppressive medication, such as prednisolone (>0.3mg/kg/day), methotrexate, azathioprine or dapsone (see guideline Feliciani et al)5 within the last 4 weeks before the sample collection. Prednisolone in a dosage <= 0.3 mg/kg/day is allowed.*
2. Application of topical potent corticosteroids on the skin within the last week.
3. Incapacitated (psycho)geriatric patients;* the use of a topical or oral corticosteroid treatment by the general practitioner in patients presenting with an acute pruriginous skin eruption, before the diagnosis of bullous pemphigoid is unfortunately quite frequent. Furthermore, 0.3mg/kg/d of oral prednisone has been previously demonstrated to be ineffective in controlling BP (Guillaume JC et al, 1993) and therefore doses upon to 0.3mg/kg/day are allowed.
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL63814.042.17 |