biological sampling: 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.
Phenotypic description: Phenotypic characterization of the patient's clinical features
Photography: All affected areas will be photographed at each visit with 2-dimensional standardized photography
Study 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.
Eligibility
Sex
ALL
Min age
6 Months
Max age
—
Healthy volunteers
No
Inclusion Criteria:
* 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
* At the timepoint of inclusion, the participant must have had active pustulation with either white, yellow or brown pustules within six month before baseline. Active postulation at baseline is not mandatory for inclusion.
* 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.
Exclusion Criteria:
* 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
Primary outcome measure(s)
Change in Physician Global Assessment (PGA) — At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 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.
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
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