AIDA Network

Research

New Study on the Dermatological Spectrum of Still’s Disease Published

A new international study has just been released, focusing on the wide range of skin manifestations in Still’s disease. The article, titled “Unfolding dermatological spectrum of Still’s disease: a cohort study from the International AIDA Network Still’s Disease Registry”, is based on real-world data from 518 patients enrolled in the AIDA Network Still’s Disease Registry.

Link to article

DOI: 10.1093/rheumatology/keaf512

Still’s disease, a rare systemic autoinflammatory condition, is typically characterized by the salmon-colored evanescent rash, but patients may also present with atypical and persistent cutaneous lesions that complicate diagnosis and management. This study aimed to investigate the prevalence of these manifestations and their relationship with age at onset, ethnicity, disease activity, and macrophage activation syndrome.


Key findings include:

  • The typical evanescent rash was the most common feature (63.9%), followed by macules (7.7%), urticarial eruptions (5.9%), erythema (5.2%), and persistent pruritic papules and plaques (4.8%).
  • Atypical lesions were found in 21.2% of patients.
  • The typical rash was more frequent in children, while pruritus occurred more often in adults.
  • Hispanic patients showed higher prevalence of atypical lesions, macules, persistent pruritic papules and plaques, and pruritus compared to Arab and White patients.
  • The typical rash correlated with higher disease activity, while no cutaneous manifestation correlated with macrophage activation syndrome.

This work provides the largest international overview to date of the dermatological spectrum of Still’s disease, highlighting important age-related and ethnic differences. These results emphasize the clinical value of recognizing both typical and atypical lesions to improve timely diagnosis and management.