Necrotizing Sweet’s Syndrome

Updated on 22/09/2017.

Sometimes, Sweet’s syndrome can occur in an unusual form. This is known as a disease variant.

Necrotizing Sweet’s syndrome.

Necrotizing Sweet’s syndrome is a rare variant of Sweet’s syndrome that mimics necrotizing fasciitis.

What is necrotizing fasciitis?

Necrotizing fasciitis (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.

What is necrotizing Sweet’s syndrome?

Necrotizing Sweet’s syndrome 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.

How many people have developed necrotizing Sweet’s syndrome?

By September 2017, at least seven cases of necrotizing Sweet’s syndrome had been reported in medical literature.

  • In three patients with very weakened immune systems (Kroshinsky et al, 2012).
  • In a 73-year-old male with chronic myelogenous leukaemia (Nakanishi and Kinjo, 2016).
  • In a 24-year-old transsexual male with recurrent myonecrosis of the neck and upper chest (Otero et al, 2017).
  • In a patient where the necrotizing Sweet’s syndrome was initially mistaken for NF (Kim et al, 2017).
  • In a patient after elective hand surgery (Lipof et al , 2017).

How is it diagnosed?

Necrotizing Sweet’s syndrome is mainly diagnosed via biopsy. Tissue samples show white blood cells called neutrophils in the deep tissues, but unlike in Sweet’s syndrome, soft-tissue necrosis also occurs. Read more here.

How is it treated?

The treatment for necrotizing Sweet’s syndrome and NF is very different, so it’s important that the two conditions are not confused.

The main form of treatment for necrotizing Sweet’s syndrome is high-dose steroids, whereas NF is treated with antibiotics and wound debridement (surgical removal of the infected tissue). Antibiotics do not work in the treatment of necrotizing Sweet’s syndrome, and wound debridement must be avoided as it can lead to the development of new lesions. Read more here.

Other.

Can patients with Sweet’s syndrome, but not necrotizing Sweet’s syndrome, sometimes develop symptoms that mimic necrotizing fasciitis?

Yes. Paraneoplastic fasciitis can be a symptom of Sweet’s syndrome, and is sometimes confused with NF (Chaudhry et al, 2015: 128-129).

What is paraneoplastic fasciitis?

Paraneoplastic fasciitis is a type of fasciitis that develops alongside cancer. It causes inflammation and thickening of the fascia, but unlike in NF, soft-tissue necrosis does not occur. In patients with Sweet’s syndrome who develop paraneoplastic fasciitis, skin lesions are normally present, but not always.

On occasion, inflammation of the fascia can be a symptom of Sweet’s syndrome, even when someone does not have cancer.

References.

Chaudhry, A., Baker, K., Gould, E. and Gupta, R. (2015) Necrotizing fasciitis and its mimics: what radiologists need to know. American Journal of Roentgenology, Jan;204(1):128-39 (online).

Kim, B.,  Chun, D., Jeen, Y. and Kim, H. (2017) Necrotizing Sweet’s syndrome presenting as necrotizing fasciitis with a good response to immune suppressant treatment. International Journal of Rheumatic Diseases, Aug 16 (PubMed).

Kroshinsky, D., Alloo, A., Rothschild, B., Cummins, J., Tan, J., Montecino, R., Hoang, M., Duncan, L., Mihm, M. and Sepehr, A. (2012) Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis, Journal of the American Academy of Dermatology, Nov;67(5):945-54 (PubMed).

Lipof, J., Beck, L., Reddy, S., Southgate, R., Carney-Young, K. and Hammert, W. (2017) Necrotizing Sweet Syndrome of the Upper Extremity After Elective Hand Surgery. The Journal of Hand Surgery, Aug 19 (American Society for Surgery of the Hand).

Nakanishi, K. and Kinjo, M. (2016) Mimicker of necrotising fasciitis with systemic inflammatory response syndrome: recurrent necrotising Sweet’s syndrome associated with chronic myelogenous leukaemia. BMJ Case Reports, Mar 31 (PubMed).

Otero, T., Barber, S., Yeh, D. and Quraishi, S. (2017) Necrotizing Soft Tissue Infection or Sweet Syndrome: Surgery Versus No Surgery? A Case Report. A&A Case Reports. Feb 1 (PubMed).

© 2012-2017 Sweet’s Syndrome UK

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3 thoughts on “Necrotizing Sweet’s Syndrome

  1. My daughter was just diagnosed with sweet syndrome. She is 23 years old . They thought she had flesh eating in her arm. She had nine surgeries on her arm. Then attacked her left breast and then attacked her and left a Buttock. She had 15 surgeries of debridment in 7.5 months before someone figured out that she has sweet syndrome . We have been to hell and back since May 17, 2015 I would love to send her medical file to someone because she is a very unique case she was misdiagnosed by many centers here in Canada

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  2. Hi Laura,

    I’m sorry to hear that your daughter has been so ill. Necrotizing SS can be a very nasty variant.

    If you would like others to learn about your daughter’s condition, the best thing that you can do is contact your daughter’s doctors and see if any of them are planning to/would consider writing a case-study detailing your daughter’s experiences.

    You could also try contacting the Canadian Organization for Rare Disorders (CORD) or the National Organization for Rare Disorders (NORD) to see if they can help. Rare Disease Day is approaching (Feb 29th 2016), and around this time, rare disease organizations are often looking for those who are willing to blog or share their experiences of having a rare disease.

    1. CORD: http://www.raredisorders.ca/

    2. NORD: http://rarediseases.org/

    If you would like to share your experiences with those with Sweet’s syndrome or their families, you can also join our groups.

    1. Sweet’s Syndrome UK Facebook Group. This is a closed group, so you have to send a request to join. Also, you do not have to be from the UK to join. Link: https://www.facebook.com/groups/sweetssyndromeawareness/

    2. Sweet’s Syndrome UK HealthUnlocked. You don’t have to be from the UK to join. Link: https://healthunlocked.com/sweetsyndrome

    Take care,

    Michelle.

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