These include inflammatory bowel disease (ulcerative colitis or Crohn disease), rheumatoid arthritis, systemic lupus erythematosus, Sjogren syndrome, Hashimoto thyroiditis, Behcet disease, and dermatomyositis. Sweet syndrome can also occur in association with several inflammatory and autoimmune diseases. Pathological features with the histiocytoid sweet syndrome are more likely to be associated with malignancy, especially myelodysplastic syndrome. When associated with malignancy, it is more likely to be associated with older age of onset and cytopenias. Sweet syndrome may occur before, at the time of or after the diagnosis of malignancy. Several malignancies have reported correlations with Sweet syndrome, including myeloproliferative disorders (myelodysplasia, acute myelogenous leukemia, chronic myelogenous leukemia, multiple myeloma, monoclonal gammopathy, lymphoma, and rarely with solid tumors. Sweet syndrome may be idiopathic, which is most common, or may be associated with an underlying condition. If corticosteroids are contraindicated, anti-inflammatory medications such as colchicine or dapsone are available options. The best first-line option is systemic or topical corticosteroids if the lesions are limited. It may also be associated with other extracutaneous systemic manifestations, which are, however, rare. The goal of pharmacotherapy in acute febrile neutrophilic dermatosis (Sweet syndrome) is to reduce morbidity and complications. 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. Sweet syndrome belongs to the non-vasculitic group of neutrophilic dermatoses disorders, with others including pyoderma gangrenosum, pustular psoriasis, reactive arthritis (Keratoderma blennorrhagicum), Bowed-associated dermatosis-arthritis syndrome (BADAS), rheumatoid neutrophilic dermatosis, Behcet's disease, acne fulminans, Familial Mediterranean Fever, SAPHO syndrome, etc. Neutrophilic dermatoses can be idiopathic or secondary to an underlying disorder, localized or generalized, and may or may not have extracutaneous manifestations. Neutrophilic dermatoses consist of a group of non-infectious disorders that are characterized by neutrophilic infiltration of the skin (epidermis, dermis, or hypodermis) with or without true vasculitis. Sweet syndrome, first described in 1964 by Robert Douglas Sweet, is an acute febrile neutrophilic dermatosis.
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