Pustular Psoriasis (PP) Clinical Trial
— IRASPENOfficial title:
International Rare And Severe Psoriasis Expert Network (IRASPEN) - A Prospective Registry With Genotype-Phenotype Correlation
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.
| Status | Recruiting |
| Enrollment | 180 |
| Est. completion date | December 2030 |
| Est. primary completion date | December 2030 |
| Accepts healthy volunteers | No |
| Gender | All |
| Age group | 6 Months and older |
| Eligibility | 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 |
| Country | Name | City | State |
|---|---|---|---|
| Germany | Klinikum der Universität München | München | |
| Italy | Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS | Rome | |
| Portugal | Universitário do Porto | Porto | |
| Singapore | National Skin Centre | Singapore | |
| Switzerland | Dermatology, University Hospital Basel | Basel | |
| Switzerland | University Hospital Zürich | Zürich | |
| Turkey | Akdeniz University School of Medicine; Department of Dermatology and Venereology | Antalya | |
| Turkey | Trakya University, Faculty of Medicine; Department of Dermatology and Venereology | Edirne |
| Lead Sponsor | Collaborator |
|---|---|
| University Hospital, Basel, Switzerland | Boehringer Ingelheim |
Germany, Italy, Portugal, Singapore, Switzerland, Turkey,
| Type | Measure | Description | Time frame | Safety issue |
|---|---|---|---|---|
| Primary | 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 | Change in Generalized Pustular Psoriasis (GPP) Area and Severity Index (GPPASI) | Measure of severity and area of psoriatic lesions in patients with psoriasis. It is a tool that provides a numeric scoring for a patient's overall GPP disease state,ranging from 0 to 72. It is a linear combination of percent of surface area of skin that is affected by erythema, pustules and scaling and the severity of erythema, pustules, and scaling (desquamation) over 4 body regions. | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Dermatology Life Quality Index (DLQI) | The Dermatology Life Quality Index (DLQI) consists of 10 questions concerning patient's perception of the impact of skin diseases on different aspects of their health related QoL over the last week. The DLQI evaluates the impact of the patient's skin disease on daily activities, leisure, work and personal relationships. Each question is scored on a 4-point Likert scale. | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in EuroQol (EQ-5D) | The EuroQol is a generic questionnaire. It is a preference based health status and multi attribute utility scale that produces a single index score for each state of health. These score ranges from 0 to 1, where 1 is equivalent to full health and 0 equivalent to death | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Work Productivity and Activity Impairment Questionnaire-General Health (WPAI-GH) | The Work Productivity and Activity Impairment Questionnaire-General Health (WPAI-GH) consists of six questions: 1 = currently employed; 2 = hours missed due to health problems; 3 = hours missed other reasons; 4 = hours actually worked; 5 = degree health affected productivity while working (using a 0 to 10 Visual Analogue Scale (VAS)); 6 = degree health affected productivity in regular unpaid activities (VAS)[ | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in psoriasis symptom scale (PSS) | The psoriasis symptom scale (PSS) is a measure of the degree to which situations in one's life are appraised as stressful. Items were designed to assess how unpredictable, uncontrollable, and overloaded respondents find their lives to be. The scale also includes a number of direct queries about current levels of experienced stress. Moreover, the questions are of a general nature and hence are relatively free of content specific to any sub-population group. The questions in the PSS ask about feelings and thoughts during the last month. | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Disease activity Visual analogue Scale (VAS) | The disease VAS is a unidimensional measure of disease intensity. The VAS is a continuous scale comprised of a horizontal line, 10 centimeters (100 mm) in length, anchored by 2 verbal descriptors, one for each symptom extreme. To avoid clustering of scores around a preferred numeric value, numbers or verbal descriptors at intermediate points are not given. The disease intensity VAS is self-completed by the respondent. The respondent is asked to place a line perpendicular to the VAS line at the point that represents their current disease intensity. | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Pain Visual analogue Scale (VAS) | The pain VAS is a unidimensional measure of pain intensity, which has been widely used in diverse adult populations. The pain VAS is a continuous scale comprised of a horizontal line, 10 centimeters (100 mm) in length, anchored by 2 verbal descriptors, one for each symptom extreme. To avoid clustering of scores around a preferred numeric value, numbers or verbal descriptors at intermediate points are not given. The pain VAS is self-completed by the respondent. The respondent is asked to place a line perpendicular to the VAS line at the point that represents their pain intensity. | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Number of flares in the last 2 years | Number of flares in the last 2 years | at Baseline | |
| Secondary | Number of flares since the last visit | Number of flares since the last visit | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Palmoplantar Pustulosis (PPP) Area and Severity Index (PPPASI) | investigator assessment of the extent and severity of pustular and plaque lesions on the palms and soles presenting in PPP patients. This tool provides a numeric scoring for patients overall PPP disease state, ranging from 0 to 72. It is a linear combination of the percent of surface area of skin that is affected on the palms and soles of the body and the severity of erythema, pustules, and scaling (desquamation). | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 | |
| Secondary | Change in Psoriasis Area and Severity Index (PASI) | The Psoriasis Area and Severity Index (PASI) is a composite variable used to assess the severity of Psoriasis. The PASI evaluates the area of psoriatic involvement in 4 main areas (head, trunk, upper and lower extremities) and the severity of the psoriatic lesions with respect to three target symptoms: erythema, infiltration and desquamation (actual percentages of area involvement) . | At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260 |