Abstract

We present a unique case of paraneoplastic Sweet's syndrome associated with a primary CNS lymphoma.

A 69-year old gentleman presented with pyrexia of unknown origin (PUO), Sweet's syndrome and marked neutrophilia. 3 repeated bone marrow aspirates demonstrated a myelodysplastic-like syndrome. Initial treatment with hydroxycarbamide ameliorated the PUO and skin lesions.

He subsequently presented with a clinical syndrome mimicking motor neurone disease; developing dysphagia, weakness and weight loss. Videofluoroscopy demonstrated oropharyngeal dysphagia requiring gastrotomy insertion. Nerve conduction and CSF studies were unremarkable. Paraneoplastic screening revealed transient anti-NMDA antibody positivity. MRI showed inferior frontal and anterior temporal lobe injury. Following steroid treatment, neurological symptoms improved, he gained weight and mobilised unaided.

4 months later he was readmitted with pyrexia, confusion and pneumonia. Positron emission tomography showed multiple foci of moderate fluorodeoxyglucose uptake in the skin and infective changes in the right lung. The patient continued to deteriorate whilst on dapsone, antibiotics and oral steroids. He developed status epilepticus, remaining encephalopathic in spite of aggressive antiepileptic therapy. Further chest imaging identified worsening of cavitating lung lesions. Despite empirical treatment with antibiotics, anti-fungals and high dose methylprednisolone the patient died. Post mortem microscopic immunostaining revealed diffuse large B-cell lymphoma in temporal regions and brainstem.