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 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 eye problems and there are few associated symptoms or if oral medication by itself isn’t 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 aren’t 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 can be 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, or cancer. 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.
Most patients with Sweet’s syndrome respond well to steroid medication, at least initially. If they don’t this could be because:
- They don’t have Sweet’s syndrome and have been misdiagnosed.
- The steroid dosage is too low.
- They 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.
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.
- Mycophenolate mofetil.
- Interferon alpha* (Ibid).
- Azathioprine* to treat histiocytoid Sweet’s syndrome (Miller et al, 2015).
- Immunoglobulin and thalidomide 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, adalimumab*, vedolizumab, and canakinumab.
- Infliximab where Sweet’s syndrome has developed secondary to ulcerative colitis and steroid treatment has been ineffective (Moreno et al, 2018).
- Vedolizumab where severe Sweet’s syndrome had developed secondary to ulcerative colitis that could only be managed with daily steroid treatment (Belvis et al, 2018).
- Anakinra in refractory (chronic or persistent, or difficult-to-treat) Sweet’s syndrome and other neutrophilic dermatoses, and 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* to treat refractory subcutaneous Sweet’s syndrome (Agarwal et al, 2016).
- Canakinumab to treat refractory Sweet’s syndrome (taken from the Sweet’s Syndrome UK Facebook group, 27/05/18).
- 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 to 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).
- Treatment with hydroxychloroquine, or retinoids such as isotretinoin* with varying success. When used to directly treat Sweet’s syndrome, hydroxychloroquine may be useful when there are significant problems with joint pain and swelling (taken from the Sweet’s Syndrome UK Facebook group, 2/04/18).
* 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 aren’t 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.
There has been some scaremongering concerning certain medications used to treat Sweet’s syndrome, particularly the steroid, prednisone. It’s 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!
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.
Pharmac (2015) Online Pharmaceutical Schedule. This is a list of the approximately 2000 prescription medicines and therapeutic products subsidized by the New Zealand Government.
Needy Meds (2015) Find help with the cost of medicine (online). Eligibility requirements vary.
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 (PubMed).
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 (PMC).
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).
Moreno Marquez, C., Maldonado Perez, B. and Castro Laria, L. (2018) Infliximab as rescue treatment in a Sweet’s Syndrome related to corticodependent ulcerative colitis. Journal of Crohn’s and Colitis, Feb 21 (Oxford Academic).
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).
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 (PMC).
2012-2018 Sweet’s Syndrome UK