What is Sweet’s syndrome?

Updated 17/09/17.

What is Sweet’s syndrome?

Sweet’s syndrome is a rare autoinflammatory condition and form of neutrophilic dermatosis that mainly affects adults, but can also affect children and babies. The main symptoms include fever and painful skin lesions that most often appear on the face, neck and upper extremities, but can appear on any part of the body. On rare occasions, there are no skin lesions. Other common symptoms include fatigue (a medical term that includes a number of different symptoms, and is not the same as simply feeling tired); muscle pain; joint pain (arthralgia); joint pain and swelling (arthritis); headaches; eye problems; sometimes, mouth ulcers. On rare occasions, the lesions can develop in internal joints and organs causing more serious symptoms. Very rarely, SS may be life-threatening. The cause of SS is poorly understood, but it is associated with infections, blood disorders and cancer (malignancy-associated or paraneoplastic), autoimmune conditions, inflammatory bowel disease, medications (drug-induced), pregnancy, skin damage, immunodeficiency, overexposure to sunlight or ultraviolet (UV) light, and vaccination. In regards to the latter, SS caused by vaccination is very rare – only 11 cases reported in medical literature in the past 42 years, globally – and a definite connection between the SS and vaccination was not established in some of those cases.


Patient information.

British Association of Dermatologists, 2015.

‘Sweet’s syndrome (also known as acute febrile neutrophilic dermatosis) is a rare skin disorder characterised by a fever and the appearance of tender solid red lumps on the skin. It is a reactive condition with a number of potential triggers. It is not contagious and is not skin cancer.’

DermNet NZ, 2015.

Acute febrile neutrophilic dermatosis is an uncommon skin condition characterised by fever and inflamed or blistered skin and mucosal lesions. Neutrophilic dermatoses are autoinflammatory conditions often associated with systemic disease.

Acute febrile neutrophilic dermatosis also has the eponymous name, Sweet syndrome or disease—named after Dr Robert Douglas Sweet from Plymouth, England, who first described it in 1964.’

Mayo Clinic (US), 2017.

‘Sweet’s syndrome is a rare skin condition. Its main signs include fever and painful skin lesions that appear mostly on your arms, neck, head and trunk.

The exact cause of Sweet’s syndrome isn’t known. In some people, it’s triggered by an infection, illness or certain medications. Sweet’s syndrome can also occur with some types of cancer.’

National Organization for Rare Diseases (US), 2015.

‘Sweet syndrome is a rare disorder characterized by fever and the sudden onset of a rash, which consists of multiple tender, red or bluish-red bumps or lesions. These lesions usually occur on the arms, legs, trunk, face or neck. In some cases, additional systems of the body can become involved including the musculoskeletal system such as inflammation of the joints (arthritis), the eyes such as inflammation of the conjunctiva or the membrane that lines the eyes (conjunctivitis), and the internal organs. In the majority of affected individuals, the disorder occurs by itself for no known reason (idiopathic Sweet syndrome); this is also known as classical Sweet syndrome. Less often, the disorder can be associated with an underlying cancer (malignancy), usually a blood (hematologic) cancer such as certain types of leukemia; this is known as malignancy-associated Sweet syndrome. The disorder can also result as a reaction to taking certain drugs, especially a drug known as granulocyte-colony stimulating factor; this is known as drug-induced Sweet syndrome. Sweet syndrome is treated with corticosteroids.’


Medical information.

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

‘Sweet’s syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Several hundreds cases of Sweet’s syndrome have been published. Sweet’s syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced.’

Cunliffe, T. (2015) Sweet’s syndrome (syn. acute febrile neutrophilic dermatosis; Gomm-Button disease). PCDS:  The Primary Care Dermatology Society (online).

‘Sweet’s syndrome is a neutrophilic dermatosis, characterised by fever, peripheral neutrophil leucocytosis and the acute onset of painful, erythematous papules, nodules or plaques.’

Medical Articles & Trials.

1 current trial: Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases (NEUTROGENE).


Media.

McColl, I. (2013) Sweet’s Syndrome: Clinical and Histopathological Variants (YouTube).


Other.

Orphanet (2017) Sweet’s syndrome (online).

Provides a summary and coding information. ICD-10: L98.2

© 2012-2017 Sweet’s Syndrome UK

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5 thoughts on “What is Sweet’s syndrome?

  1. I have had Sweets for 25 years-diagnosed by biopsy of one of the lesions accompanied by bloodwork. It seems to have altered in presentation over the years when it reoccurs. At the present time the outbreaks seem to be inclusive of fever, sharp pain in heels (used to be outer ankle bones) AND stiffness & pain in the entire foot & ankle upon rising in the morning which loosens up as the day goes on. I also have, for the past 9 years-been suffering with sporadic facial edema, also swelling of feet & hands. Upon my last outbreak the extreme swelling of eyelids & face coo included with the most severe painful stiffness & pain in all of both feet upon waking necessitating half an hour of ‘shuffling’ motions to get around until the ankle & toes loosened and normal walking motion could begin. I would like to know if perhaps I am dealing w more than one ‘disease’ or ‘syndrome’ OR if all of these symptoms ‘fit’ within the ‘normal’ presentation…for ME-since no two patients obviously have identical presentations. This all began o e year after, at 19, I had a Whipple Procedure for pancreatic cancer followed up at 20 by a total Pancreatectomy of the remaining pancreas for recurrence. I know much damage was done-and presents s little worse as the years pass-to my colon & digestive tract. I am assuming the cause of MY Sweets was a combination of the cancer, the colon loss in parts (removal of several different parts following Whipple due to impaction) which has left me a severe Celiac patient. ANYTHING SOMEONE MAY BE ABLE TO ADD WOULD BE GREATLY APPRECIATED- my doctors feel they have maxed out their knowledge & prescribe Medrol (prednisone) Dose Packs for outbreaks. THANK YOU….Rebecca Brookoff Fichtel

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  2. Hi Rebecca,

    It sounds like you’ve been having a really tough time.

    My apologies for not getting back to you sooner. For some reason, I didn’t receive an email notification to say that a comment on my blog had been made.

    The symptoms of Sweet’s syndrome (SS) can sometimes change, but as some of the symptoms of SS can be the same as the symptoms of other conditions, it would be impossible for me to say if your symptoms are all being caused by SS or not.

    Read more about the symptoms of SS @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-are-the-symptoms-of-sweets-syndrome/

    Common symptoms of SS include fever, joint pain and/or swelling which could include stiffness. Facial oedema or swelling of the hands and feet doesn’t tend to occur unless lesions are present. However, facial puffiness, and swelling of the hands and feet are common side-effects of prednisone.

    SS can develop secondary to cancer and autoimmune conditions, including coeliac disease. However, once the underlying condition is treated or managed, the SS often settles down. As you mention that your coeliac disease is severe, I’m wondering if you are having problems managing your coeliac disease, and this in turn could be triggering your SS.

    Read more about causes and triggers for SS @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-causes-sweets-syndrome/

    As your prednisone doesn’t seem to be working particularly well at the moment, your doctors may want to consider trying some other medications alongside your steroids. Many SS patients have to take more than one medication to bring their condition under control.

    Read more about treatment @ https://helpforsweetssyndromeuk.wordpress.com/key-information/what-is-the-treatment-for-sweets-syndrome/

    Rebecca, because you have SS, there are also certain things that you will have to be careful about.

    1. Overexposure to UV light or sunlight can trigger SS in some patients, so you may have to be careful about spending too much time in the sun and minimize or avoid using sunbeds. Sunburn must definitely be avoided.

    2. Some SS patients demonstrate pathergy, so you may have to be careful about skin damage and irritation. Read about pathergy @ https://helpforsweetssyndromeuk.wordpress.com/2014/10/06/skin-hypersensitivity-in-sweets-syndrome-koebner-phenomenon-pathergy/

    3. Infection, particularly upper respiratory tract infection, e.g. coughs, colds, flu, sore throats and flu-like illness, can trigger SS in some patients. This means that it’s a good idea to try to minimize exposure to infection or take very good care of yourself if you do pick up an infection.

    Take care,

    Michelle.

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    • OMG THANK YOU FOR REPLYING!!! And thank you for all the links I will research on right now!!! I never got your response in my email..rebecca0313brooks@gmail.com….it is now March 10th and as I was scanning the internet I came across a story and I was SHOCKED that someone else had their pancreas removed also at 19, also had a total Pancreatectomy AND THEN I saw…it was me !!! Lol…and then I saw your response!! Interesting update-I had bloodwork a month ago that was indicative of para-Thyroid cancer- I am waiting for follow up blood results-THIS would explain a lot as at times SS presents prior to a malignancy & para-Thyroid cancer causes FEVER & BONE PAIN-just as a described. I don’t feel panicked-I simply feel hopeful that the blood work comes back & if it is positive we can get a move on and get me some help. QUESTION…do you think diuretics could cause SS symptoms??? Due to the swelling about ten Yrs ago which made my blood pressure go thru the roof I was PERSCRIBED diuretics…I am wondering if the diuretics can be setting off the SS- would you email me??? You are the only person in this world who actually gets back to me-inclusive of my doctors who seem to have given up on me….💭Rebecca

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