Abstract
Sweet’s Syndrome, also known as acute febrile neutrophilic dermatosis is a rare disease of unknown etiology, but has been associated with autoimmune processes, malignancies, infections, GI disorders and drug reactions. It is more prevalent in women than men, except in case of malignancy associated origin. Clinical features of this disorder include acute onset of painful plaques on skin, neutrophilic leukocytosis, fever, dermal infiltrations by neutrophils. Usually, sweet’s syndrome is presented in three main clinical settings which includes: the classical (idiopathic), malignancy-associated, and drug induced. Classical Sweet’s Syndrome (CSS) is usually seen in women between the age of 30 to 50 years. It is frequently preceded by an upper respiratory illness and could be linked to pregnancy, inflammatory bowel disease, and both. Recurrence of the dermatosis is usually seen in one third of the patients experiencing classical sweet’s syndrome. Malignancy associated Sweet’s Syndrome (MASS) is usually present in patients with confirmed cancer and manifested as a paraneoplastic syndrome. Also occurs in those whose solid tumor or hematologic dyscrasia associated with sweet’s syndrome was previously undetected. Drug induced Sweet’s Syndrome (DISS) occurs in patients being treated with granulocyte-colony stimulating factor. Various other drugs including antibiotics like Minocycline, Trimethoprim Sulfamethoxazole, nitrofurantoin, antiepileptics like Carbamazepine, Diazepam, NSAIDS, Diuretics and Retinoids can also cause DISS. Diagnosis of sweet syndrome is usually done by two major criteria (histopathology and acute cutaneous lesion), and four minor criteria’s (infection, malignancy, medications, fever, and extra cutaneous manifestations like leukocytosis and corticosteroid response. Systemic corticosteroids are the golden standard of treatment for Sweet’s Syndrome. Systemic corticosteroids (0.5mg to 1mg/kg/day) for 4-6 weeks improves the systemic symptoms and reduce the skin lesions. For treating localized lesions, topical application of high potency corticosteroids or intralesional corticosteroids are effective. Other first line agents including potassium iodide and colchicine are also used. Second line agents include Indomethacin, Cyclosporine, Dapsone and Clofazimine. Keywords: Sweet’s Syndrome, Corticosteroids, Malignancy, Leukocytosis
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