Periorbital necrotizing sweet syndrome

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Periorbital necrotizing sweet syndrome: A report of two cases mimicking necrotizing soft tissue infections, Mar 2024. pubmed.ncbi.nlm.nih.gov/384...

2 patients with necrotizing Sweet's syndrome - a 74-year-old female with myelodysplastic syndrome, and a 40-year-old female with acute myeloid leukaemia. Initially, both were thought to have necrotizing soft tissue infections of the eyelids. Both demonstrated pathergy (read notes below).

WHAT IS NECROTIZING SWEET'S SYNDROME?

Necrotizing Sweet’s syndrome (NSS) is a rare variant of Sweet’s syndrome that mimics both the skin and systemic symptoms of necrotizing fasciitis (NF).

NF is a rare and serious bacterial infection that affects the superficial fascia – a layer of connective tissue that lies beneath the skin and between the muscles and organs in the body. NF not only causes inflammation and damage to the fascia, but also the tissues next to it. Toxins made by the bacteria then destroy soft-tissue, causing it to die. This is known as soft-tissue necrosis.

NSS is an acute necrotizing form of neutrophilic dermatosis that not only causes skin lesions, the most common symptom of Sweet’s syndrome, but also affects the deeper tissues and causes soft-tissue necrosis.

NSS symptoms include: fever; low blood pressure; skin lesions; pathergy – when the skin is damaged and it triggers the development of new skin lesions or makes existing ones worse; severe pain.

NSS is mainly diagnosed via biopsy. A sample of a lesion shows white blood cells called neutrophils in the deep tissues, but unlike in Sweet’s syndrome, soft-tissue necrosis also occurs. Fever and low blood pressure in NSS can be misdiagnosed as sepsis – a serious complication of infection – with NF.

Treatment for NSS and NF is very different, so it’s important that the two conditions aren’t confused, misdiagnosis increasing the likelihood of unnecessary surgical procedures and prolonged illness, at the very least. The main form of treatment for NSS is intravenous (IV) steroids, whereas NF is treated with IV antibiotics and wound debridement – surgical removal of the infected tissue. Antibiotics for infection don’t work in the treatment of NSS, and wound debridement must be avoided due to a pathergy response.