Persistent plaques and linear pigmentation in adult-onset Still's disease

A 25-year-old Japanese man presented with high spiking fever, arthralgia an d a skin rash. A pruritic edematous erythema with persistent plaques was fo und mainly on the trunk; these lesions persisted even when the fever subsid ed, with prominent linear pigmentation. As marked neutrophilia and a high l evel of serum ferritin were detected, a diagnosis of adult-onset Still's di sease (AOSD) was made, even though the persistent eruption was not characte ristic of the disease. Oral prednisolone, together with low-dose methotrexa te, was given with good results. In the literature, a similar atypical rash has been reported in 11 cases in Japan. All of them required high-dose adm inistration of corticosteroids or other immunosuppressive agents. Severe sy stemic complications were seen in 3 patients, and 2 cases died of the disea se. Persistent plaques and linear pigmentation are some of the manifestatio ns of AOSD, which cannot be overlooked. This appearance could be an indicat ion that suggests an increased risk of systemic complications and a prolong ed time to clinical remission. Copyright (C) 2001 S. Karger AG, Basel.