What is the treatment for Sweet’s syndrome?

Main form of treatment – steroid medication.

The main form of treatment for Sweet’s syndrome is corticosteroids, otherwise known as steroids (Cohen, 2007).

Steroids are most commonly given as tablets. The recommended dosage is prednisone 30-60mg daily. Sometimes, steroids are given in other ways, such as:

  • Intravenous infusion (via a drip) if the lesions and associated symptoms are more serious.
  • Cream (topical) applied to the skin if the lesions are small, in one place, or there are few associated symptoms. The strong steroid cream, clobetasol propionate, has been used to successfully treat lesions, but a milder cream may be needed in areas where the skin is thinner or more sensitive, e.g. the face or genital area (Ranpara and Yesudian, 2015).
  • Injection into the affected area if the lesions are in one place and there are few associated symptoms, or into a joint if the lesions have formed in an internal joint such as the knee.
  • Eye drops if Sweet’s syndrome has caused certain eye problems and there are few associated symptoms or if oral medication by itself is not effective.

Steroid medications are often given by themselves, but are sometimes given alongside other treatments for the following reasons:

  • Some patients find it more difficult to get off their steroids if they are not given another form of treatment alongside their steroids. This is because the Sweet’s syndrome can sometimes flare-up when the steroid dosage starts to be tapered (slowly reduced).
  • In some patients, the Sweet’s syndrome is more likely to flare-up once the steroid medication has been completely stopped, particularly if steroids were the only form of treatment.
  • Sweet’s syndrome is often more difficult-to-treat in patients who have developed it secondary to another health condition, e.g. infection, autoimmune condition, inflammatory bowel disease such as Crohn’s disease or ulcerative colitis, cancer or blood disorder. As a result, steroid medication alone may not be effective.
  • The patient has a variant of Sweet’s syndrome called chronic classical (idiopathic) Sweet’s syndrome.

Important!

Most patients with Sweet’s syndrome respond well to steroid medication, at least initially. If they do not this could be because:

  • They do not have Sweet’s syndrome, i.e. have been misdiagnosed.
  • Have developed their Sweet’s syndrome secondary to another health condition.
  • Their Sweet’s syndrome has been triggered by medication and they are still taking that medication.
  • They are taking herbal supplements that are interacting with their steroid medication and preventing it from working properly. This can include supplements that ‘boost’ the immune system, e.g. alfalfa, astragalus, chlorella, and echinacea.

Other treatments.

First and second-line treatments.

First-line treatments (treatments that are considered after steroid medication).

After steroids, first-line treatments include colchicine and potassium iodide (SSKI) (Cohen, 2007; Kaur et al, 2015). They can be given instead of or alongside steroid medication.

Second-line treatments (treatments that are considered after first-line treatments).

Second-line treatments include indomethacin, clofazimine, ciclosporin, and the sulfa drug, dapsone (Cohen, 2007). They can be given instead of or alongside steroid medication.

Less common treatments.

Less common treatments include:

  • Tetracycline antibiotics, e.g. minocycline* or doxycline* (Cohen, 2007; Liaw, 2017). These are a special a type of antibiotic that have the ability to reduce inflammation.
  • Methotrexate.
  • Mycophenolate mofetil.
  • Chlorambucil.
  • Cyclophosamide.
  • Interferon alpha* (Ibid).
  • Azathioprine* has been used to successfully treat histiocytoid Sweet’s syndrome (Miller et al, 2015).
  • Immunoglobulin and thalidomide are useful in patients who have developed Sweet’s syndrome secondary to myelodysplastic syndromes (MDS).
  • Azacitidine has been used to successfully treat Sweet’s syndrome in a patient with clonal hematopoiesis of indeterminate potential (CHIP) (Yaghmour et al, 2017). The term CHIP is used to describe individuals who have a clonal mutation associated with haematologic neoplasia (blood cancer), but do not meet the criteria for diagnosis of a haematologic neoplasm.
  • Biological therapies or biologics, including infliximab and etanercept (Cohen, 2007). See below for information on other biologics: anakinra, rituximab and adalimumab*.
  • Anakinra is a useful treatment in refractory (chronic or persistent, or difficult-to-treat) Sweet’s syndrome and other neutrophilic dermatosesand autoinflammation of unknown cause (Kluger et al, 2011; Passaro et al, 2013; Simon et al, 2014).
  • There are two reported cases of rituximab being used to successfully treat refractory Sweet’s syndrome in a man who was later diagnosed with interstitial lung disease and rheumatoid arthritis, and a man with chronic lymphocytic leukaemia (Hashemi et al, 2016; Seminario-Vidal et al, 2015).
  • Adalimumab* has been used to successfully treat refractory subcutaneous Sweet’s syndrome (Agarwal et al, 2016).
  • Sulfapyridine, a sulfa drug, has been used to to treat and manage the Sweet’s syndrome variant, chronic classical (idiopathic) Sweet’s syndrome (Gowda et al, 2016).
  • Granulocyte and monocyte adsorption apheresis (GMA) therapy has been used to successfully treat refractory Sweet’s syndrome in a 55-year-old woman (Fujii et al, 2017). Apheresis is a medical technology in which the blood of a person is passed through an apparatus that separates out one particular constituent and returns the remainder to the circulation. In GMA, the main action is to selectively adsorb and deplete activated neutrophils and monocytes, and may be useful in Sweet’s syndrome patients with high serum levels of G-CSF (granulocyte colony-stimulating factor).

* On rare occasions, these medications may trigger Sweet’s syndrome. Read more here.


Treatment during pregnancy.

The main form of treatment during pregnancy is steroids. There are other medications that can be considered, but some are not safe to use. Read more here.


Treating Sweet’s syndrome in children.

The main form of treatment for Sweet’s syndrome in children is steroid medication. There has been some success with mycophenolate mofetil, immunoglobulin and dapsone. Anakinra has been used to successfully treat hyper IgD syndrome (HIDS) presenting as Sweet’s syndrome in a 6-week-old girl (Payne et al, 2013: 118, 122). Other medications such as ciclosporin have also been tried with varying results.


Medical and surgical treatments that don’t work or should be avoided.

Sweet’s syndrome is a rare condition, and because of this, some doctors don’t know how to treat it. As a result, they will sometimes give treatments that don’t work or can make Sweet’s syndrome worse. This is a list of medical and surgical treatments that don’t work or should be avoided. These include:

  • Antibiotics: most patients don’t respond to antibiotics, but tetracyclines can be an exception (see ‘Less common treatments’). However, antibiotics are necessary if the skin lesions have become infected or another infection needs to be treated.
  • Antihistamines: these are not a treatment for Sweet’s syndrome. If a patient does respond well to antihistamines, it normally suggests that they have another condition, or an underlying condition that is triggering their Sweet’s syndrome. On occasion, antihistamines are prescribed for itchy lesions, but most of the time, they don’t work.
  • Wound debridement: if possible, wound debridement (removal of affected tissue), particularly surgical wound debridement (surgical removal of affected tissue) should be avoided. This is because it can potentially cause new skin lesions to develop. Read more here.
  • Ultraviolet (UV) light therapies: these should be avoided as overexposure to UV light is a proven trigger for Sweet’s syndrome. Read more here.

A warning about medications and scaremongering.

In recent years, there has been some scaremongering concerning certain medications used to treat Sweet’s syndrome, particularly the steroid, prednisone. It is very important that you take your medication to bring your Sweet’s syndrome under control, and if you are experiencing side-effects, please speak to your doctor. Unless a fairly serious adverse reaction has occurred, do not stop taking your medication without first seeking medical advice, and NEVER abruptly stop your steroid medication unless your doctor has instructed you to do so. This is potentially dangerous!


Other information.

Australia.

Australian Government, Department of Health (2015) A-Z Medicine Listing in The Pharmaceutical Benefits Scheme (online).

Australian Government, Therapeutic Goods Administration (2015) Product and Consumer Medicine Information (online). This is a list of medications available in AU and related product information. Click on an information sheet document link and accept the access terms to read the product information.

New Zealand.

Pharmac (2015) Online Pharmaceutical Schedule. This is a list of the approximately 2000 prescription medicines and therapeutic products subsidised by the New Zealand Government.

United Kingdom.

NHS Choices (2015) Get Help With Prescription Costs in Help With Health Costs (online). England only.

United States.

Needy Meds (2015) Find help with the cost of medicine (online). Eligibility requirements vary.


References.

Agarwal, A., Barrow, W., Selima, A. and Nicholas, M. (2016)  Refractory Subcutaneous Sweet Syndrome Treated With Adalimumab. JAMA Dermatology, Jul;152(7):842-844 (online). 

Cohen, P. (2007) Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis (online).

Fujii, A., Mizutani, Y., Hattori, Y., Takahashi. T., Ohnishi, H., Yoshida, S. and Seishima, M. (2017) Sweet’s Syndrome Successfully Treated with Granulocyte and Monocyte Adsorption Apheresis. Case Reports in Dermatology, May 22;9(2):13-1 (online).

Gowda, A., Rosenbach, M., Micheletti, R. and  James, W. (2016) Chronic idiopathic Sweet syndrome: A report of 2 cases. Journal of the American Academy of Dermatology, May;2(3):227–229 (online).

Hashemi, S., Fazeli , S., Vahedi, A. and Golabchifard, R. (2016) Rituximab for refractory subcutaneous Sweet’s syndrome in chronic lymphocytic leukemia: A case report. Molecular and Clinical Oncology, Mar;4(3):436-440 (online).

Kaur, S., Bery, A., Garg, B. and Sood, N. (2015) Table 2: Drugs used for treatment of malignancy associated Sweet’s syndrome in Sweet’s syndrome associated with chronic neutrophilic leukemia. Indian Journal of Dermatology, Venereology and Leprology, Mar-Apr;81:203-6 (online).

Kluger, N., Gil-Bistes, D., Guillot, B. and Bessis, D. (2011) Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret®) in a case of refractory Sweet’s syndrome. Dermatology (Basel, Switzerland), May;222(2):123-7 (PubMed).

Liaw, T. (2017) A case of Sweet syndrome successfully treated by anti-inflammatory property of doxycycline. Journal of the Formosan Medical Association, Jan 17 (online).

Miller, J., Lee, N. and Sami, N. (2015) Histiocytoid Sweet syndrome treated with azathioprine: a case report. Dermatology Online Journal, Jul; 21(7).

Passaro, G., Cerrito, L., Giovinale, M., Marinaro, A., Soriano, A,. Rigante, D. and Manna, R. (2013) P03-019 – Anakinra for sweet syndrome treatment. Pediatric Rheumatology Online Journal, Nov; 11(Suppl 1).

Payne, K., Keiser, P., Kaplan, M. and Jones, O. (2013) Hyper IgD Syndrome Presenting as Sweet’s Syndrome in a 6 Week Old Infant. Annals of Paediatric Rheumatology, Jun;2:118-123 (PDF).

Ranpara, M. and Yesudian, P. (2015) A perplexing rash. BMJ Case Reports, Sept 2nd (online).

Seminario-Vidal, L., Guerrero, C. and Sami, N. (2015) Refractory Sweet’s syndrome successfully treated with rituximab. Journal of the American Academy of Dermatology, May;1(3):123-125 (online).

Simon, A. et al (2014) Autoinflammation of Unknown Cause. AUTOINFLAMMATION.EU (online).

Yaghmour, G., Wiedower, E., Yaghmour, B., Nunnery, S., Duncavage, E. and Martin, M. (2017) Sweet’s syndrome associated with clonal hematopoiesis of indeterminate potential responsive to 5-azacitidine. Therapeutic Advances in Hematology, Feb;8(2):91-95 (online).

© 2012-2017 Sweet’s Syndrome UK

11 thoughts on “What is the treatment for Sweet’s syndrome?

  1. I have my friend who suffer from sweet syndrome and was told to have bee milk or bee venom to cure but not sure if is any benefits on it.

    Like

    • Hi Nora,

      I’m afraid that whoever told your friend this is either mistaken or making things up. Bee ‘milk’ or venom is not a treatment or cure for Sweet’s syndrome, and there is no medical evidence to support this claim.

      Like

  2. My son got Sweat syndrome. Here is our experience.
    You shall always know that steroid is a relief, not a cure.

    Several methods can be tried to relieve the suffering with less side effects.

    If one can get around without using steroid, try it.

    1. Blood Letting. Sterilize the surrounding area properly.
    Take a lancet, the one used for diabetes blood test,
    Pin a few times around a lesion and let a little bit of blood.

    2. Exercise and sweet.
    Drink sufficient water and mildly exercise in dry weather, e.g., hiking for a few hours.

    3. Light and balance diet. May be a low dose of multi vitamin daily.

    4. Enzymes.
    Take digestive enzymes to go with every meals.

    If works, 1 and 2 shall show good relief in 1-2 hours.
    3 and 4 are less obvious. But, they are healthy any way.

    Like

  3. About a year ago I developed bumps all over my leg, google had said they were flea bites but after a week or so began turning into craters. I saw layer after layer come off of these lesions and it got to the point where I could see the inside of my leg. I then decided to go to the hospital, only to find out no one knew what it was and took many guesses. Four thousand dollars later, a dermatologist diagnosed me with Sweets Syndrome. I was given prednisone 60 mg and a topical cream. Seemed to work but just recently came back spreading faster but only spots/ skin stains with pains. Next next they would prescribe me acticlate 150mg and cordran topical cream. The feeling of swelling and stiffness in my legs have gotten worse, and has spread from my calves to my feet and thighs. Any insite on what has been known to help? Or insite on long term medication uses? Ive read that the meds help slow it down but have bad effects on organs. I was told there was a ladder of types of treatments like heavier steriods, injections to affected spots, and creams.. When a blood test was done they told me I had strep in my blood? Aviod sick people as much as possible because of a low immune system cause of SS. Dont know how true that is.. Slowly learning about SS through my experiences..

    Like

    • Hi Jose,

      Sorry to hear that you’ve been struggling with your SS.

      Prednisone is usually the most effective form of treatment for SS, but there are others (see the main blog post above). Unfortunately, even though some people respond well to doxycycline (Acticlate), it doesn’t work for everyone. Therefore, your doctors will possibly need to prescribe you another medication.

      In regards to a weakened immune system, people with SS have an overactive not an underactive or weakened immune system. However, the immune system can be weakened by medication, e.g. immunosuppressants such as prednisone. Read more about how SS affects the immune system @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-kind-of-condition-is-sweets-syndrome/

      You say you have a streptococcal infection. Sometimes, infection can trigger SS in some people with SS, but not all. This is because of something called hypersensitivity reaction.

      – In SS, hypersensitivity reaction is when the innate immune system (the part of the immune system involved in SS, and a part of the immune system that doesn’t produce antibodies) is hypersensitive and goes into overdrive, overreacting to the presence of antigens such as infectious antigens, e.g. a bacteria or virus.
      – Antigens are mainly proteins or sugars on the surface of a cell, or a non-living substance that a part of your immune system called the adaptive immune system sees as a foreign invader and produces antibodies in response to.
      – The presence of these antigens can potentially trigger SS by causing the innate immune system to activate lots of inflammatory cells, particularly white blood cells called neutrophils. This then leads to the symptoms of SS.

      If you are someone whose SS does tend to be triggered by infection, it might be worth trying to reduce your exposure to people with infections if you possibly can.
      Read more about potential triggers for SS @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-causes-sweets-syndrome/

      In regards to your leg swelling and stiffness, joint or muscle pain (could include stiffness) are side-effects of doxycycline. However, muscle pain, joint pain, and arthritis (joint pain or stiffness and swelling) are fairly common symptoms of SS. If these symptoms are being caused by your SS, then your doctors need give you another SS medication, as your doxycycline doesn’t appear to be working well. If you are able to take them, non-steroidal anti-inflammatory drugs (NSAID’s), e.g. ibuprofen (Brufen, Advil, Motrin), can help to reduce pain and swelling. Read more about the symptoms of SS @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-are-the-symptoms-of-sweets-syndrome/

      If you need to take medication in the longer term, doctors generally try to give a medication other than prednisone because of the side-effects associated with long term steroid use. Dapsone is a fairly common treatment for SS, but does have side-effects and you will need to monitored while taking it. If you are very worried about side-effects and are able to take potassium iodide/SSKI (not everyone can), this treatment tends to have some of the fewest side-effects.

      – Systemic steroids, DermNet NZ: http://www.dermnetnz.org/topics/systemic-steroids/
      – Dapsone, DermNet NZ: https://www.dermnetnz.org/topics/dapsone
      – Potassium iodide, DermNet NZ: https://www.dermnetnz.org/topics/potassium-iodide

      Hope things improve soon, Jose.

      Like

  4. Jose,

    I’ve just re-read your post, and I’m not sure whether you’re still taking your prednisone or not, as some people take prednisone plus other medications. Prednisone normally brings the symptoms of SS under control, so as you are having problems with joint stiffness and swelling, then your doctors would need to rule out any underlying cause for your SS, e.g. an autoimmune condition that causes joint pain and swelling. This may have already been done.

    Like

    • I’m not on the prednisone at the moment, I was prescribed the acticlate. When I was taking prednisone I had minor fatigue, but now with the acticlate I do get a heavy fatigue feeling and dizziness. Loss of appetite and stomach cramps.. I do greatly appreciate all the information, I feel a little better knowing more about SS.

      Like

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s