Pustular Psoriasis Pustular Psoriasis (PP), non-interventional, the phenotypes of PP, INTERNATIONAL RARE AND SEVERE PSORIASIS EXPERT NETWORK (IRASPEN) A Prospective Registry With Genotype-Phenotype Correlation
Conditions
Interventions
Diagnosis of PP confirmed by a dermatologist in the participant. The type of PP can be any one of PPP, GPP/AGEP, ACH or a mixed phenotype, according to the judgment of the investigator.
Treatment
Pustular Psoriasis
Sponsors
Prof. Dr. med. Dr. sc. nat. Alexander Navarini
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: Adult or pediatric patients eligible for inclusion in this registry have to fulfill all of the following criteria: 1. Age equal to or greater than 6 months 2. Written informed consent of the patient or legal proxy in the registry 3. Diagnosis of PP confirmed by a dermatologist in the participant. The type of PP can be any one of PPP, GPP/AGEP, ACH or a mixed phenotype, according to the judgment of the investigator. 4. At the timepoint of inclusion, the participant (or the patient referred to in criterion 2) must have had active pustulation with either white, yellow or brown pustules within 6 month before inclusion. 5. Sufficient language skills (in the languages which the patient information and the consent form is available) for the informed consent to participate 6. Patients of all ancestries and skin pigment type can be included. 7. If applicable: Direct non-affected adult (>18 years old) relatives of the participant (up to two, namely mother, father, sibling) with the purpose to provide DNA for family trio sequencing analysis. The patient is not excluded from the study if no relatives are included.
Exclusion criteria
Exclusion criteria: 1. Any medical or psychological condition in the opinion of the treating physician which may prevent the patient in registry participation for the next 5 years. 2. Lack of informed consent for registry participation. Due to the rarity of PP and the nature of this registry, minimal risks are associated with the participation to the registry. For those reasons, we also plan to include vulnerable participants like children > 6 months of age and pregnant women.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number of flares in the last 2 years (baseline) / since the last visit 5 Years A percentage will be calculated as well as the 95% confidence interval, using Blaker s method,PP area and severity score, namely GPPASI / PPP ASI / NAPSI 5 Years PP area and severity score,PGA (depending on PP subtype, further specified as GPP, PPP, ACH) 5 Years scale of measurement | — |
Secondary
| Measure | Time frame |
|---|---|
| the exploration of a range of endpoints including objective and subjective measures of psoriasis and the burden of the illness 5 Years Tables and graphical representation will be chosen to optimally display the development of these endpoints over the course of the illness. | — |
Countries
Thailand
Contacts
Public ContactAlexander Navarini
University Hospital Basel
Outcome results
None listed