What is the treatment for Sweet’s syndrome?

Links checked 02/11/20.

Main form of treatment – steroid medication.

The main form of treatment for Sweet’s syndrome is steroid medication, e.g. prednisone.

Steroids are most commonly given as tablets, and 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 there’s significant joint pain and swelling.
• Eye drops if Sweet’s syndrome has affected the eyes 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. cancer, autoimmune condition, or inflammatory bowel disease. As a result, steroid medication alone may not be effective.
• The patient has a long-term form of Sweet’s syndrome. See chronic classical (idiopathic) Sweet’s syndrome.

Important!

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 (increase immune system activity), e.g. alfalfa, astragalus, chlorella, and echinacea.

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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* 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/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.
• 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 (remove) 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.

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

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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.

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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.

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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 some sulfa drugs and tetracyclines are an exception. 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: surgical wound debridement (surgical removal of affected tissue) should be avoided. This is because it can potentially cause new skin lesions to develop or make existing ones worse (pathergy).
• Ultraviolet (UV) light therapies: these should be avoided as overexposure to UV light is a proven trigger for Sweet’s syndrome.

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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).

Belvis, M., Maldanado, B. and Arguelles-Arias, F. (2018) Using Vedolizumab to treat Severe Sweet Syndrome in a Patient with Ulcerative Colitis. Journal of Crohn’s and Colitis, May 19 (Oxford Academic).

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

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 (PMC).

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 (PMC).

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).

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

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

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) (PMC).

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 (ScopeMed).

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). Currently unavailable.

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-present, Sweet’s Syndrome UK