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Sweet syndrome in acute myeloid leukaemia

A 43-year-old male with acute myeloid leukaemia (AML) following a myelodysplastic syndrome, who was on azacitidine therapy but not in remission, presented with a 24-h history of high-grade fever and the acute eruption of painful reddish-brown elevated skin lesions. On examination, there were multiple red-brown plaques and nodules involving the face, scalp, neck and the extensor surfaces of the upper extremities, with some ulceration (top panels). His temperature was 38°C and C-reactive protein was 235 mg/l. The patient was commenced on empirical piperacillin-tazobactam, clindamycin, aciclovir and isavocunazole. A punch skin biopsy demonstrated a nodular neutrophilic infiltrate in the dermis, classical for Sweet syndrome, and 30 mg daily of oral prednisolone was then commenced. The skin lesions progressed and a 3-day pulse of methylprednisolone 500 mg intravenously was given, resulting in significant improvement (bottom panels). Following this, oral steroids were re-commenced. Sweet syndrome is a reactive dermatosis, which more commonly affects females (up to a 4:1 preponderance has been reported). It is characterised by pyrexia with painful, erythematous lesions that may ulcerate. The aetiology is unknown in approximately 50% of cases, although common triggers are respiratory or gastrointestinal tract infections and the use of non-steroidal anti-inflammatory drugs. Rarely, an autoimmune condition or a haematological malignancy is the culprit. Management involves the treatment of any underlying cause, along with topical and/or systemic corticosteroids. Occasionally, immunosuppressive agents are required. This case represents a rare manifestation of Sweet syndrome secondary to AML in a male patient.