title: Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.
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.
authors.affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA. de.michailidou@gmail.com.
authors.forename: Despina
authors.initials: D
authors.lastname: Michailidou
authors.affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
authors.forename: Junghee
authors.initials: J
authors.lastname: Shin
authors.affiliation: Section of Pulmonary, Critical Care and Sleep Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
authors.forename: Inga
authors.initials: I
authors.lastname: Forde
authors.affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
authors.forename: Kavitha
authors.initials: K
authors.lastname: Gopalratnam
authors.affiliation: Department of Pathology, Bridgeport Hospital, Yale University School of Medicine, New Haven, CT, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: DeGirolamo
keywords: AOSD; Atypical cutaneous manifestations; Eosinophils; Systemic inflammatory response syndrome