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Sweet syndrome (SS) is a neutrophilic dermatosis characterised by fever, neutrophilia, tender erythematous skin lesions and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis.1 The scarcity of the disease does not allow evaluation of drug responses in randomised controlled trials. This report describes for the first time the dramatic efficacy of a treatment with the interleukin 1 (IL-1) receptor antagonist in a patient presenting with refractory SS.
A 55-year-old woman had, since 1998, recurrent fever, pulmonary involvement (alveolar lung condensations), elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) without evidence of an infectious or inflammatory disease. Skin lesions of the right flank were occasionally …