Autoinflammatory & rare systemic diseases
Unfolding dermatological spectrum of Still’s disease: a cohort study from the International AutoInflammatory Disease Alliance (AIDA) Network Still’s Disease Registry
Authors' conclusion
The present study enhances dermatologists' awareness of the diverse cutaneous lesions that may represent heterogeneous manifestations of Still's disease, shedding new light on the difference related to the age at disease onset, the patients' ethnic origin and the severity of the disease.
Study context
- Population
- Patients with Still's disease in the International AIDA Network Still's Disease Registry41.3% males
- Participants
- 518 patients
- Outcome
- Cutaneous manifestations of Still's disease and their correlation with ethnic origin, age at onset, disease pattern, macrophage activation syndrome (MAS) and systemic activity score
Methodological detailsregistry-based, multicentre
- Registry-based
- Multicentre
Main finding
As reported in the abstract
Frequency of skin manifestations
- Salmon-coloured evanescent skin rash
- n = 304, 63.9%
- Macules
- n = 40, 7.7%
- Urticarial eruptions
- n = 31, 5.9%
- Erythema
- n = 27, 5.2%
- Persistent pruritic papules and plaques (PPPP)
- n = 25, 4.8%
- Atypical skin rash
- 110 (21.2%)
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Salmon-coloured evanescent skin rash and pruritus by age at onsetAge <16 years vs Age 16-60 | P = 0.002 |
| Salmon-coloured evanescent skin rash and pruritus by age at onsetAge <16 years vs Age 16-60 · pruritus more common in <16 years | P = 0.008 |
| Pruritus by ethnicity and disease courseWhite vs Arab · pruritus more frequent in White | P = 0.008 |
| Pruritus by ethnicity and disease coursePolycyclic course vs Monocyclic course · pruritus more frequent in polycyclic | P = 0.049 |
| Atypical skin manifestations by ethnicityHispanic vs Arab · higher rate in Hispanics | P = 0.036 |
| Atypical skin manifestations by ethnicityHispanic vs White · higher rate in Hispanics | P = 0.036 |
| Macules and PPPP by ethnicityHispanic vs White · macules more frequent in Hispanics | P = 0.027 |
| Macules and PPPP by ethnicityHispanic vs Arab · PPPP more frequent in Hispanics | P = 0.023 |
| Macules and PPPP by ethnicityHispanic vs White · PPPP more frequent in Hispanics | P = 0.002 |
| Salmon-coloured evanescent skin rash by systemic activity scoreSignificantly more frequent with systemic score ≥7 | P < 0.001 |
- Salmon-coloured evanescent skin rash and pruritus by age at onset: salmon-coloured evanescent skin rash more common in <16 years
Original publication
Unfolding dermatological spectrum of Still’s disease: a cohort study from the International AIDA Network Still’s Disease Registry. Rheumatology (Oxford). Published 30 September 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →