Classic Sweet Syndrome with Mucosal Involvement

Abstract

Sweet syndrome is an uncommon disorder of unknown etiology and characterized by skin and systemic inflammation. Classic Sweet syndrome is not associated with malignancy or drug exposure and typically involves elderly females. A 67-year-old female with a past medical history of multiple sclerosis and chronic kidney disease presented with worsening erythema of bilateral lower extremities, and diffuse, tender pustular lesions in her extremities, eye lids, nares, oral commissures, and tongue. A biopsy revealed marked neutrophilic inflammation suggestive of Sweet syndrome and she was started on high dose methylprednisolone which immediately improved her condition. As with most progressive inflammatory diseases, early recognition and early treatment improves the prognosis. This case is unusual because of mucosal involvement at presentation. Classic Sweet syndrome can often be diagnosed by exclusion after failed treatment for either infectious or autoimmune disorders.