Persistent pigmented plaques in adult-onset Still's disease

Background: Adult-onset Still's disease (AOSD) is a systemic affection characterized by intermittent fever, evanescent rash, polyarthralgia or arthritis and neutrophilic leucokytosis. We report a new case of AOSD with persistent pigmented plaques in a Vietnamese woman.

Methods: A 23 year-old woman had presented within several months: inflammatory polyarthralgia, intermittent fever, asthenia, myalgia, weigh loss, polyadenopathy and an evanescent cutaneous rash. In addition, she presented pigmented skin plaques on the trunk. The investigations noted: neutrophilia (10000/mm3) and a high level of serum ferritin (42,000 microg/l). The erythrocyte sedimentation rate was 55mm in the first hour. C reactive protein was normal. HIV and syphilis serologies, antinuclear antibodies and rheumatoid factor were negative. No growth of micro-organisms was observed in repeated blood and urine cultures. The histology of the persistent plaque and lymph node were not specific. It required a high dose of corticosteroid to obtain remission.

Conclusions: The existence of persistent plaques in AOSD is uncommon. Nevertheless, the exact relationship between the fixed cutaneous lesions and AOSD remains unclear. But their synchronous evolution with systemic symptoms and response to therapy suggest that they represent a specific manifestation of the disease.