International Rare And Severe Psoriasis Expert Network

NCT04359394 | Recruiting

• 1 other identifier: 2020-00425; sp18Navarini2
• Study Type: observational
• Target: 180
• Locations: 6 countries
• Sites: 8

Brief Summary

This registry is a prospective observational study in order to describe primarily the natural course of PP subtypes and to gain detailed information about their phenotype.

Conditions

Pustular Psoriasis (PP)

Keywords

Palmoplantar Psoriasis (PPP); IRASPEN International Rare and Severe Psoriasis Expert Network; ERASPEN European Rare and Severe Psoriasis Expert Network; Psoriasis; Generalized pustular psoriasis (GPP); Acrodermatitis continua of Hallopeau (ACH); Interleukin 36 Receptor Antagonist Gene (IL36RN); Caspase Recruitment Domain-containing protein 14 (CARD14); Adaptor Related Protein Complex 1 Subunit Sigma 3 (AP1S3)

Outcome Measures

Primary Outcomes (1)

• Change in Physician Global Assessment (PGA)

  modified PGA (physician's assessment of psoriatic lesions) scoring the erythema, pustules, and scaling of all GPP or PPP lesions from 0 to 4. Each component is graded separately, the average is calculated, and the final PGA is determined from this composite score. A lower score indicates a lesser severity, with 0 being clear and 1 being almost clear.

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

Secondary Outcomes (11)

• Change in Generalized Pustular Psoriasis (GPP) Area and Severity Index (GPPASI)

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

• Change in Dermatology Life Quality Index (DLQI)

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

• Change in EuroQol (EQ-5D)

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

• Change in Work Productivity and Activity Impairment Questionnaire-General Health (WPAI-GH)

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

• Change in psoriasis symptom scale (PSS)

  At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260

Study Arms (1)

PP patients

patients with active PP

Other: biological sampling; Other: Phenotypic description; Other: Photography

Interventions

biological sampling OTHER

In order to investigate the level of molecular pathophysiology, blood and punch biopsies will be collected of each patient. In up to two relatives per patient, 30mL blood will be collected only once.

PP patients

Phenotypic description OTHER

Phenotypic characterization of the patient's clinical features

PP patients

Photography OTHER

All affected areas will be photographed at each visit with 2-dimensional standardized photography

PP patients

Eligibility Criteria

• Age: 6 Months+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

The project population encompasses all patients aged 6 months and over with active PP in the participating countries. Patients will be recruited by consecutive ongoing recruitment in the investigators' clinical practice during 5 years.

You may qualify if:

• Written informed consent of the patient or legal proxy in the registry
• Diagnosis of PP confirmed by a dermatologist in the participant. The type of PP can be any one of PPP, GPP/Acute Generalized Exanthematous Pustulosis (AGEP), ACH or a mixed phenotype, according to the judgment of the investigator
• GPP: Primary, sterile, macroscopically visible epidermal pustules on non-acral Skin with or without systemic Inflammation; with or without plaque psoriasis; either relapsing (\>1 episode) or persistent (\>3 months)
• PPP: Primary, persistent (\>3 months), sterile, macroscopically visible epidermal pustules on palms and/or soles with or without plaque psoriasis
• ACH: Primary, persistent (\>3 months), sterile, macroscopically visible epidermal pustules affecting the nail apparatus with or without plaque psoriasis
• Sufficient language skills (in the languages which the patient information and the consent form is available) for the informed consent to participate
• Patients of all ancestries and skin pigment type can be included
• 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.

You may not qualify if:

• Any medical or psychological condition in the treating physician's opinion which may prevent the patient in registry participation for the next 5 years
• Lack of informed consent for registry participation

Sponsors & Collaborators

• University Hospital, Basel, Switzerland: lead
• Boehringer Ingelheim: collaborator

Study Sites (8)

Klinikum der Universität München

München, 80337, Germany

RECRUITING

Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS

Rome, 00168, Italy

RECRUITING

Universitário do Porto

Porto, 4150-117, Portugal

RECRUITING

National Skin Centre

Singapore, 308205, Singapore

RECRUITING

Dermatology, University Hospital Basel

Basel, 4031, Switzerland

RECRUITING

University Hospital Zürich

Zurich, 8058, Switzerland

RECRUITING

Akdeniz University School of Medicine; Department of Dermatology and Venereology

Antalya, 07059, Turkey (Türkiye)

RECRUITING

Trakya University, Faculty of Medicine; Department of Dermatology and Venereology

Edirne, Turkey (Türkiye)

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

fresh blood (whole blood) and skin biopsies (formalin-fixation followed by embedding in paraffin and fixation in RNAlater) for isolation of RNA, DNA

MeSH Terms

Conditions

Psoriasis

Interventions

Photography

Condition Hierarchy (Ancestors)

Skin Diseases, Papulosquamous; Skin Diseases; Skin and Connective Tissue Diseases

Intervention Hierarchy (Ancestors)

Diagnostic Imaging; Diagnostic Techniques and Procedures; Diagnosis; Investigative Techniques

Study Officials

• Alexander Navarini, Prof. Dr. med. Dr. sc. nat.

  Dermatologie, Universitätsspital Basel

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Alexander Navarini, Prof. Dr. med. Dr. sc. nat.

CONTACT

+41 61 328 60 80; alexander.navarini@usb.ch

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 5 Years
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 21, 2020
• First Posted: April 24, 2020
• Study Start: September 22, 2021
• Primary Completion (Estimated): December 1, 2030
• Study Completion (Estimated): December 1, 2030
• Last Updated: May 9, 2024

Record last verified: 2024-05

Locations

SG (1); TU (2); PT (1); IT (1); DE (1); CH (2)