Sweet Syndrome

Sweet syndrome, first described in 1964 by Robert Douglas Sweet, is an acute febrile neutrophilic dermatosis. Neutrophilic dermatoses consist of a group of noninfectious disorders that are characterized by neutrophilic infiltration of the skin (epidermis, dermis, or hypodermis) with or without true vasculitis. Neutrophilic dermatoses can be idiopathic or secondary to an underlying disorder, localized or generalized, and may or may not have extracutaneous manifestations. Sweet syndrome belongs to the nonvasculitic group of neutrophilic dermatosis disorders, with others including pyoderma gangrenosum, pustular psoriasis, reactive arthritis (Keratoderma blennorrhagicum), Bowed-associated dermatosis-arthritis syndrome, rheumatoid neutrophilic dermatosis, Behcet disease, acne fulminans, familial Mediterranean fever, and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteomyelitis). Sweet syndrome characteristically demonstrates the sudden onset of well-defined tender plaques or nodules accompanied by fever, arthralgias, ocular inflammation, headaches, and, rarely, oral or genital lesions. This condition may also be associated with other extracutaneous systemic manifestations, which are, however, rare. The goal of pharmacotherapy in acute febrile neutrophilic dermatosis (eg, Sweet syndrome) is to reduce morbidity and complications. The best first-line option is systemic or topical corticosteroids if the lesions are limited. If corticosteroids are contraindicated, anti-inflammatory medications such as colchicine or dapsone are available options.