Difference in phenotype between nonbullous and bullous pemphigoid can be explained by gene expression and/or eosinophillic activity in the skin.
ID
Bron
Aandoening
bullous pemphigoid, nonbullous pemphigoid, bulleus pemfigoïd, niet-bulleus pemfigoïd.
Ondersteuning
Onderzoeksproduct en/of interventie
Uitkomstmaten
Primaire uitkomstmaten
The difference in gene expression in nonbullous and bullous pemphigoid patients will be analyzed using principle component analysis. Furthermore, the expression levels of surface markers and proteins on eosinophils will be compared between the two pemphigoid variants.
Achtergrond van het onderzoek
Rationale: Pemphigoid is the most common autoimmune bullous disease and typically
presents with severe itch and bullae on the skin (bullous pemphigoid). However, 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 unraveled, and it is unknown what causes
the differences in phenotype.
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: prospective observational study
Method: 5 participants with nonbullous pemphigoid ≥ 18 years old and 5 participants with
bullous pemphigoid ≥ 18 years old will be included. Gene expression will be assessed by
RNA sequencing of one lesional and one healthy skin biopsy of the participants. Left over
skin after surgery will be used as control skin of 4 persons ≥ 18 years old that do not suffer
from pemphigoid. Moreover, a third biopsy will be taken of lesional skin for
immunofluorescent staining to assess eosinophilic activity and IL-31 in the skin. Moreover,
blister fluids will be collected in the patients with bullous pemphigoid, and a cytospin will be
stained for IL-31 expression
Doel van het onderzoek
Difference in phenotype between nonbullous and bullous pemphigoid can be explained by gene expression and/or eosinophillic activity in the skin.
Onderzoeksopzet
There is only one point of observation, at the moment that we take biopsies. There is no follow-up.
Onderzoeksproduct en/of interventie
no interventions will be used. we will collect biopsies from patients that are included.
Publiek
Wetenschappelijk
Belangrijkste voorwaarden om deel te mogen nemen (Inclusiecriteria)
1. Written informed consent.
2. ≥18 years old.
3. Patients that are diagnosed with pemphigoid (bullous or nonbullous) ¡Ü1 month ago, or pemphigoid patients that were in complete remission without therapy and experience a relapse can be included.
[The following diagnostic criteria are used for 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. DIF results of the healthy skin biopsy (taken as normal procedure for diagnosis) must be positive in all patients.
5. Active disease with skin lesions.
Belangrijkste redenen om niet deel te kunnen nemen (Exclusiecriteria)
potential subject who meets any of the following criteria will be excluded from participation in this study:
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 ¡Ü 0.3 mg/kg/day is allowed.
2. Application of topical potent corticosteroids on the skin within the last week.
3. cognitively incompetent (psycho)geriatric patients
Opzet
Deelname
Opgevolgd door onderstaande (mogelijk meer actuele) registratie
Geen registraties gevonden.
Andere (mogelijk minder actuele) registraties in dit register
Geen registraties gevonden.
In overige registers
| Register | ID |
|---|---|
| NTR-new | NL6971 |
| NTR-old | NTR7159 |
| Ander register | METc : 2017/653 |