Abstract
Adult onset Still’s disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still’s rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.
Highlights
Adult onset Still’s disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by intermittent spiking high fever, an evanescent, salmonpink or erythematous maculopapular skin rash, arthralgia or arthritis and leukocytosis with at least 80 % neutrophils [1]
AOSD can present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease
Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated
Summary
Adult onset Still’s disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by intermittent spiking high fever, an evanescent, salmonpink or erythematous maculopapular skin rash, arthralgia or arthritis and leukocytosis with at least 80 % neutrophils [1]. Ibuprofen has been used intermittently to alleviate back pain as well as the fever with minimal relief He denied any contacts with sick individuals, insect or animal bites and his last trip was Autoimmun Highlights (2015) 6:39–46. The clinical and laboratory findings were consistent with the diagnosis of AOSD according to Yamaguchi criteria [2] He was started on 50 mg of prednisone. After 1 week of treatment with Anakinra and while on 40 mg of prednisone the patient remained afebrile but new erythematous plaques appeared on lower abdominal quadrants and a skin biopsy was performed (Fig. 4).
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
