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( Helena Lobo Martins ),( Tomas Abrantes Da Fonseca ),( Teresa Sequeira ),( Isabel Almeida ),( Antonio Marinho ),( Carlos Vasconcelos ) 대한내과학회 2014 대한내과학회 추계학술발표논문집 Vol.2014 No.1
Sweet`s syndrome (SS) is the most common acute neutrophilic dermatosis (ND), often idiopathic although it is associated with a wide range of diseases, especially neoplastic, infi ammatory and infectious. We report the case of SS occurring on a 43-year-old woman with a known seronegative rheumatoid arthritis (RA) since 20 year-old. After diagnosis, was treated by NSAIDs, methotrexate (MTX) and gold salts with complete remission. Medication was suspended. In 2002, develops disabling symmetric polyarthritis, unresponsive to corticosteroids, NSAIDs and sulfasalazine. MTX was avoided due to chronic cholestasis (autoantibodies to primary biliary cirrhosis were negative, IRM excluded primary sclerosing cholangitis and liver biopsy without cirrhosis or interface hepatitis). Initiated Anti-TNF alpha monotherapy (Adalimumab) in 2004 with complete remission. Suspended due to pregnancy in 2007 and stayed in remission without drugs until 2014. In 2012, she presented widespread painful mucocutaneous lesions involving the neck, trunk and upper limbs, with fever, arthralgia and deterioration of general condition, compatible with SS. Biopsy confi rmed diagnosis. Systematically excluded tuberculosis, lympho/myeloproliferative and other autoimmune diseases. Executed corticotherapy unsuccessfully, having developed iatrogenic diabetes. Improvement of lesions with dapsone, maintaining tolerable oral lesions. In 2014, worsening cutaneous lesions and initiates cyclosporine without any benefit. Under cyclosporine, progression to disabling oral lesions, and severe polyarthritis with DAS 28 5. 02 (high activity disease). The authors assumed SS secondary to RA and started anti-TNF alpha etanercept. Skin lesions improved within few days, with complete regression of all clinical and laboratory abnormalities. The authors argue the importance to consider SS in differential diagnosis of new mucocutaneus lesions in patients with known autoimmune diseases, including RA. The diagnosis should be based on clinical,laboratory and histological fi ndings of an acute febril neutrophilic dermatosis without vasculitis. In these cases, the treatment with anti-TNF alpha is an option.
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