A Study of the Clinical Features of 15 Patients with Adult-onset Still’s Disease

Abstract

Fifteen patients with adult-onset Still’s disease (AOSD) who visited our department from January of 1995 to December of 2005 were assessed for their clinical features. One patient was male and the others were female. The mean onset age was 35.8 years (range 19–67 years). Although the typical skin rash arises as nonpruritic salmon pink macules that develop with febrile attacks, only 4 patients (27%) displayed an evanescent rash following defervescence. By contrast, the skin rash of 9 patients (60%) tended to disappear and reappear independently of fever. Six patients (40%) complained of pruritis. Therefore, we should recognize that urticarial rash with or without pruritis is also a common cutaneous feature of AOSD. Furthermore, we attempted to determine whether any characteristics present before treatment were of prognostic significance. Seven of the 14 patients that we followed up for more than 1 year exhibited a relapse of the symptoms that was dependent on the reduction of steroid dosage. None of the symptoms or laboratory data were predictive of relapse ; however, cases with a skin rash that had preceded fever and/or arthralgia showed a lower rate of recurrence. Although further large and longitudinal studies are needed, the subset of patients who have the skin rash as an initial symptom may show a better clinical course than do other patients.