Adult-onset Still's disease and the role of dermatological manifestations: A case report and literature review.

ABSTRACT

Adult-onset Still's disease (AOSD) is a rare inflammatory systemic disease with unknown etiology, characterized by spiking fever, evanescent rash, arthralgia and arthritis, leukocytosis and possible multi-organ involvement. Based on a case report of a 19-year-old man who was admitted to hospital for an influenza-like syndrome associated with a transient and recurrent pale-red disseminated non-specific rash, we performed a PubMed database search for cases and reviews of Adult's Onset Still's Disease published in English in the last 5 years. Due to its heterogeneous clinical manifestations, of which skin rash is an important sign, and the lack of a specific laboratory test, the diagnosis requires the exclusion of a wide range of mimicking disorders and the use of specific scoring systems. The high ferritin levels, major leukocytosis with neutrophilia, absence of typical antibodies for other systemic autoimmune diseases and other markers of infectious disease were the milestones that led to the positive diagnosis. The first line of treatment remains corticosteroid therapy in association with disease-modifying anti-rheumatic drugs.