Neurological Sweet's syndrome: a rare but treatable manifestation of an autoinflammatory disease

Sweet's syndrome is an autoinflammatory disease characterised by systemic symptoms and a cutaneous neutrophilic dermatosis. Neurological involvement is rare but important to recognise. Patients may have headache, confusion, seizures, and focal neurological deficits; MR brain scanning may show widespread T2-hyperintense lesions, with a CSF pleocytosis. Clinicians should suspect neurological Sweet's syndrome in patients with central nervous system dysfunction, who have unexplained fever or systemic inflammation, and a pustular neutrophilic dermatosis. The condition responds well to corticosteroids, which can prevent long-term neurological sequalae.