international rare and severe psoriasis expert network

Conditions: Pustular Psoriasis (PP)

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.

this project is to describe the natural course of disease in different subtypes of pp. the network builds on a static registry that was based on a one-time clinical characterization of pp patients in europe (eraspen). the international rare and severe psoriasis expert network (iraspen) already has multiple clinicians involved who have successfully characterized and included their patients in eraspen. iraspen addresses the question of temporal evolution of clinical features and is actually a non-interventional prospective registry that aims to describe the clinical course and responses to already established treatments of a large number of pp patients over a period of 5 years. the data collection with this registry will give insight on the natural course of pp disease revealing the burden of disease including frequency and severity of flares and the role of therapeutic interventions.

Eligibility criteria:
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

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)