Can vaccination trigger Sweet’s syndrome?

Sweet’s syndrome triggered by vaccination.

There is some medical evidence to show that certain vaccinations can potentially trigger Sweet’s syndrome, but this is incredibly rare, and it is important to take the following information into consideration:

  • Sweet’s syndrome is rare, probably affecting no more than 3 people per 10,000 (Zamanian and Ameri, 2007).
  • It mainly affects adults not children, and only 5% to 8% of cases will be in children (Sharma et al, 2015).
  • In some people, something is needed to trigger the onset of Sweet’s syndrome, but in up to 71% of people with Sweet’s syndrome there is no known trigger (Tam and Ingraffea, 2015).
  • Infection, mainly upper respiratory tract infection, is a more common trigger for Sweet’s syndrome than vaccination. As a result, Sweet’s syndrome tends to be more common in countries where people are more likely to develop infections (Ginarte and Toribio, 2011:120).
  • There have only been 10 cases of Sweet’s syndrome triggered by vaccination reported in medical literature in the past 42 years – globally! In some of these cases, a definite connection between the vaccination and Sweet’s syndrome was not established.
  • Sweet’s syndrome has only been associated with certain vaccinations and not others (see below).

Which vaccinations have been associated with Sweet’s syndrome?

Sweet’s syndrome has been associated with the following vaccinations:

  • Bacillus Calmette-Guerin (BCG or tuberculosis) (Carpentier et al, 2002: 82; Cruz-Velasquez et al, 2016). Two cases. One in 1986, occurring 15 days after vaccination, but the authors of the medical article that reported this did not control the tuberculin (Mantoux) test. One reported in 2002, occurring 10 days after vaccination.
  • Streptococcus pneumonia (Carpentier et al, 2002: 82; Cruz-Velasquez et al, 2016; Pedrosa et al, 2013). Two cases. One reported in 1990, occurring 4 days after vaccination following a splenectomy. One reported in 2013, and the first with the 13-valent conjugate vaccine.
  • Smallpox (Carpentier et al, 2002: 82; Cruz-Velasquez et al, 2016). Two cases reported in 1975, occurring 3 days after vaccination.
  • Influenza (Cruz-Velasquez et al, 2016; Hali et al, 2010, Jovanovic et al, 2005; Tan el al. 2006; Wolf et al. 2009). Four cases. One reported in 2005; in 2006, one case of bullous Sweet’s syndrome following vaccination in a HIV-infected patient; in 2009, neutrophilic dermatosis of the hands occurring 12 hours after vaccination; in 2010, one case of Sweet’s syndrome after H1N1 influenza (swine fluvaccination.

Do vaccinations trigger Sweet’s syndrome because they are toxic, contain toxins or dangerous chemicals?

No, vaccinations do not trigger Sweet’s syndrome because they are toxic, contain toxins or dangerous chemicals, and anyone who tells you this is either lying to you, trying to scare you, or has no understanding of vaccinations or Sweet’s syndrome.

Why do vaccinations trigger Sweet’s syndrome?

Vaccination can trigger Sweet’s syndrome because of hypersensitivity reaction. This is not the same as allergic reaction.

What is hypersensitivity reaction?

Sweet’s syndrome is caused by errors in the innate immune system (the body’s most primitive, ‘hard-wired’ immune system, and a part of the immune system that doesn’t produce antibodies). Because of these errors, in some people with Sweet’s syndrome, their innate immune system responds to antigens (proteins that a part of your immune system called the adaptive immune system sees as a foreign invader and produces antibodies in response to) in a way that it shouldn’t, i.e. is hypersensitive and over-reacts to the presence of infectious, inflammatory, drug, or tumour cell antigens (Bhat et al, 2015: 257; Kasirye et al, 2011: 135). This means that the presence of antigens associated with certain health conditions, medications and vaccinations can potentially trigger Sweet’s syndrome by causing the innate immune system to activate inflammatory cells, particularly white blood cells called neutrophils (Gosheger et al, 2002: 70). This then leads to the symptoms of Sweet’s syndrome.

If I have Sweet’s syndrome should I avoid having vaccinations?

No, most people with Sweet’s syndrome don’t need to avoid having their vaccinations unless they can’t be vaccinated for other medical reasons. However, if the Sweet’s syndrome was initially triggered by a particular vaccination, e.g. influenza, then it would not be advisable to have the same kind of vaccination again.

How do I know if vaccination has triggered my Sweet’s syndrome?

Remember, Sweet’s syndrome triggered by vaccination is incredibly rare, but if it does happen, then symptoms usually develop within hours, days or less commonly, a few weeks after vaccination. Skin lesions sometimes appear at the vaccination site, but this can also happen because of the skin damage caused by having the vaccination (puncture wound from the needle) rather than the vaccine itself. This response is known as pathergy.

Are there other triggers for Sweet’s syndrome?

Yes, and aside from the triggers that have already been mentioned (infection, skin damage, and vaccination), other triggers for Sweet’s syndrome include:

  • Cancer and blood disorders in 15-20% of cases, e.g. solid tumours, and myelodysplastic syndrome which may progress to acute myeloid leukaemia (Chen et al, 2016).
  • Inflammatory bowel disease, e.g. Crohn’s disease and ulcerative colitis (Cohen, 2007).
  • Autoimmune conditions, e.g. rheumatoid arthritis and systemic lupus erythematosus.
  • Medications in up to 5% of cases.
  • Pregnancy in up to 2% of cases. This is probably associated with hormonal changes, but further research is required.
  • Overexposure to sunlight or ultraviolet (UV) light. This can sometimes trigger Sweet’s syndrome, but we are not entirely sure why this happens.

References.

Bhat, Y., Hassan, I., Sajad, P., Akhtar, S. and Sheikh, S. (2015) Sweet’s Syndrome: An Evidence-Based Report. Journal of the College of Physicians and Surgeons – Pakistan, Jul;25(7):525-7 (PubMed).

Carpentier, O., Piette, F. and Delaporte, E. (2002) Sweet’s syndrome after BCG vaccination. Acta Dermato-Venereologica;82(3):221 (PubMed).

Chen, S., Kuo, Y., Liu, Y., Chen, B., Lu, Y. and Miser, J. (2016) Acute Myeloid Leukemia Presenting with Sweet Syndrome: A Case Report and Review of the Literature. Pediatrics and Neonatology (online).

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

Cruz-Velásquez, G., Pac Sha, J., Simal Gil, E. and Gazulla, J. (2016). Aseptic meningitis and anti-β2-glycoprotein 1 antibodies in Sweet syndrome. Neurologia (Barcelona, Spain), Jul 21 (0nline). Article in Spanish, use translate

Ginarte, M. and Toribio, J. (2011) Sweet Syndrome. In Dr. Fang-Ping (Ed.) Autoimmune Disorders – Current Concepts and Advances from Bedside to Mechanistic Insights. Croatia or China: Intech, pp. 119-132 (PDF). 

Gosheger, G., Hillman, A., Ozaki, T., Buerger, H. and Winklemann, W. (2002) Sweet’s Syndrome Associated With Pigmented Villonodular Synovitis. Acta Orthopædica Belgica, Feb;68(1):68-71 (PubMed).

Hali, F., Sbai, M., Benchikhi, H., Ouakadi, A. and Zamiati, S. (2010) [Sweet’s syndrome after H1N1 influenza vaccination]. Annales de Dermatologie et de Venereologie,  Nov;137(11):740-1 (PubMed).

Jovanovic, M., Poljacki, M., Vujanovic, L. and Duran, V. (2005) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) after influenza vaccination. Journal of the American Academy of Dermatology, Feb;52(2):367-9 (PubMed).

Kasirye, Y., Danhof, R., Epperla, N. and Garcia-Montilla, R. (2011) Sweet’s Syndrome: One Disease, Multiple Faces. Clinical Medicine & Research, Nov;9(3-4):134-136 (online).

Pedrosa, A., Morais, P., Nogueira, A., Pardal, J. and Azevedo, F. (2013) Sweet’s syndrome triggered by pneumococcal vaccination. Cutaneous and Ocular Toxicology, Sep;32(3):260-1 (PubMed).

Sharma, A., Rattan, R., Shankar, V., Tegta, G. and Verma, G. (2015) Sweet’s syndrome in a 1-year-old child. Indian Journal of  Paediatric Dermatology;16:29-31 (online).

Tam, C. and Ingraffea, A. (2015) Case Letter: Sweet Syndrome Presenting With an Unusual Morphology. Cutis, Aug;96(2):E9-E10 (online).

Tan, A., Tan. H., and Lim, P. (2006) Bullous Sweet’s syndrome following influenza vaccination in a HIV-infected patient. International Journal of Dermatology, Oct;45(10):1254-5 (PubMed). 

Zamanian, A. and Ameri, A. (2007) Acute febrile neutrophilic dermatosis (Sweet’s syndrome): a study of 15 cases in Iran. International Journal of Dermatology, Jun;46(6):571-4 (PubMed).

Wolf, R., Barzilai, A. and Davidovici, B. (2009) Neutrophilic dermatosis of the hands after influenza vaccination. International Journal of Dermatology, Jan;48(1):66-8 (PubMed).

© 2012-2017 Sweet’s Syndrome UK

28th Feb 2017 is Rare Disease Day: what can you do to spread awareness of Sweet’s syndrome?

The 28th February 2017 is Rare Disease Day, and it marks the tenth international Rare Disease Day coordinated by EURORDIS (the European Organization for Rare Diseases). On and around this day patient organizations from countries all over the world will be promoting awareness of rare diseases and holding awareness-raising activities, many of which will be based on the Rare Disease Day 2017 theme of research.

What can you do to spread awareness of Sweet’s syndrome?

  1. Like our Facebook page.
  2. Join our HealthUnlocked forum and community. It’s free!
  3. Follow this blog.
  4. Follow on twitter @sweetsfiend
  5. Follow on Google +.
  6. Talk about Sweet’s syndrome: share some posts; comment; blog about your experiences; tag a friend; tweet for Sweet’s.
  7. Make a donation to the Autoinflammatory Alliance. This is a US-based non-profit organization that helps children and adults with autoinflammatory conditions, including Sweet’s syndrome.
  8. Make a donation to Skin Conditions Campaign Scotland. Sweet’s Syndrome UK is a patient organization member of this charity. This gives us a greater ability to spread awareness of Sweet’s syndrome throughout the UK via Scotland.
  9. Visit the Rare Disease Day website, and sign up for their updates, download free materials, follow on social media, and read about how research can bring hope to those with a rare disease.

© 2012-2017 Sweet’s Syndrome UK

Mindfulness can reduce psychosocial distress in patients with conditions affecting the skin

What is mindfulness?

Taken from the UK mental health charity, MIND (MIND, 2016).

MIND describes mindfulness as:

‘ A technique which can help people manage their mental health or simply gain more enjoyment from life. It involves making a special effort to give your full attention to what is happening in the present moment – to what’s happening in your body, your mind or your surroundings, for example – in a non-judgemental way. Mindfulness describes a way of approaching our thoughts and feelings so that we become more aware of them and react differently to them.’

Can mindfulness help those with conditions affecting the skin to cope better?

Yes. A study by Montgomery et al has shown that mindfulness can help people ‘to reduce the distress associated with social anxiety and avoidance found in many skin conditions’ (Montgomery et al, 2016). This is very important, as those with conditions affecting the skin are at increased risk of developing anxiety and depression, often avoid social situations as a result of the distress that they cause, and can experience disability levels that are the same as those with other long term diseases.

Can anyone be mindful, and are there different ways to be mindful or practice mindfulness?

Yes. Anyone can be mindful, and there are many different ways in which you can practice mindfulness, e.g. by stopping to notice the small everyday things, by practising meditation or yoga, and by watching your thoughts or learning to view them in a different way (NHS Choices, 2016). For more information on how to be mindful, see ‘References’ below and click on the links.

References.

MIND (2016) Mindfulness (online).

Montgomery, K., Norman, P., Messenger, A. and Thompson, A. (2016) The importance of mindfulness in psychosocial distress and quality of life in dermatology patients. British Journal of Dermatology, Nov;175(5):930-936 (online).

NHS Choices (2016) Stress, Anxiety and Depression: Mindfulness (online). Includes information on the different ways in which you can be mindful.

Skin Support (2017) Support Materials. British Association Dermatologists (online). Includes ‘Meditations and Mindfulness’.

© 2012-2017 Sweet’s Syndrome UK

Alternative and nutritional therapies that don’t work, should be used with caution, or avoided completely in patients with Sweet’s syndrome

Reposted on 13/10/16, updated on 29/01/17, and links checked on 30/03/17.

Can alternative or nutritional therapies be used to treat or cure Sweet’s syndrome?

No. There is absolutely no medical evidence to show that Sweet’s syndrome can be successfully treated or cured with alternative or nutritional therapies. However, some of these therapies may be helpful in promoting overall good health and well-being.

Are you sure that alternative or nutritional therapies can’t be used to treat or cure Sweet’s syndrome, or is this simply a lie that ‘Big Pharma’ wants us to believe?

Yes. At present, we are sure that there is no alternative or nutritional therapy that can be used to treat or cure Sweet’s syndrome. However, some people are being lied to, and told that there are natural and alternative cures for Sweet’s syndrome, but that ‘Big Pharma’ doesn’t want them to know about it.

Who or what is ‘Big Pharma’?

‘Big Pharma’ is a term that’s used to refer to the pharmaceutical industry. Some conspiracy theorists believe that doctors, the pharmaceutical industry and the government, are trying to keep us sick and prevent or discourage us from accessing alternative ‘miracle cures’. This is supposedly being done so that we’ll be forced to buy and use the medications that ‘Big Pharma’ produce and sell, which will continue to make them very rich. There is no evidence to support this theory, but it is not unusual for those selling bogus treatments to resort to the ‘Big Pharma’ conspiracy simply to try and back up their false claims. This is done to try and convince you that their treatments really do work, but that ‘Big Pharma’ never wants you to discover this secret truth, because it will negatively affect their profits. If someone does resort to the ‘Big Pharma’ conspiracy to back up their claims, then it’s a red flag and a strong indication that they probably can’t be trusted.

Are alternative and nutritional therapies always safe to use?

Alternative and nutritional therapies are sometimes safe to use, but not always. Some of these therapies are potentially harmful, or could make Sweet’s syndrome worse. Herbal supplements in particular, can cause lots of problems. One real concern is that they can interact with medications or reduce their effectiveness, and sometimes, any interactions that do occur can be dangerous. Before trying an alternative or nutritional therapy, please check with your doctor first.

Alternative and nutritional therapies that don’t work, should be used with caution, or avoided completely in patients with Sweet’s syndrome.

This is list of alternative and nutritional therapies that don’t work, should be used with caution, or avoided completely. Despite this fact, they are still being advocated or sold as treatments or cures for Sweet’s syndrome by alternative therapists, other individuals, and businesses. These treatments include:

  • Acupuncture: no evidence to show that it works, and should be used with caution. This is because the skin damage caused by the treatment, i.e. the skin being punctured by the needles, may trigger the development of new lesions (pathergy). Read more here.
  • Baking soda (sodium bicarbonate): this is being advocated as a treatment for Sweet’s syndrome, myelodysplastic syndromes and leukaemia, and other cancers (in 15-20% of patients, Sweet’s syndrome develops secondary to some form of cancer). This is an incredibly dangerous pseudoscientific claim, i.e. a false or made-up claim that appears to be scientifically-based, but is not. Anyone who makes such claims should not be believed, and treated with caution. Read more here.
  • Change of diet or elimination of dietary toxins: there is no evidence to show that Sweet’s syndrome is caused by diet or dietary toxins, or that a change in diet can directly improve or cure it. Sweet’s syndrome is caused by errors in the innate immune system and involves factors such as hypersensitivity reaction, cytokine dysregulation and genetic susceptibility. Special diets, e.g. alkaline, anti-inflammatory, detox, gluten-free, Palaeolithic, dairy-free, and vegan are not treatments for Sweet’s syndrome, and could increase the risk of nutritional deficiency in some people. If possible, try to avoid a dairy-free diet, particularly a vegan diet, if you have been taking systemic steroid medication for more than 3 months. This kind of diet may be lower in calcium, and if you are taking steroids, it is very important that you meet your daily calcium requirements (Clarys et al, 2014: 1319, 1321, 1324, 1327). This is because you will be at increased risk of developing steroid-induced osteoporosis. You may also need to be careful about nutritional deficiency if you have other types of health condition, are pregnant, or on a low income. In regards to the latter, you might not have that much money to spend on food and may struggle to meet your nutritional needs as a result.
  • Chiropracty and osteopathy: Sweet’s syndrome does often cause joint pain (arthralgia) or joint pain and swelling (arthritis), and can sometimes develop secondary to autoimmune conditions that affect the joints, e.g. ankylosing spondylitis, rheumatoid arthritis, systemic lupus erythematosus, or Sjögren’s syndrome. However, there is no evidence to show that chiropracty or osteopathy can be used to treat or cure Sweet’s syndrome, and if the joints are painful and swollen, osteopathy and chiropracty should be avoided (NHS Choices, 2014; NHS Choices, 2015b). This is because joint manipulation could make symptoms worse. Physiotherapy is completely safe.
  • EAV or bioenergetics: these are tests that involve using electrodiagnostic devices to supposedly determine the cause of a disease by detecting the ‘energy imbalance’ causing the problem, or even cure a condition by correcting this imbalance. These tests and treatments are a scam, and there is absolutely no medical evidence to show that they work. In the United States (US), the importation of EAV devices has been banned. If you are in the US and someone offers you, or refers you for EAV testing, please report them to the relevant authorities.
  • Essential oils: no evidence to show that they work, and should be used with caution when applied to the skin. This is because the oils might cause irritation, potentially triggering the development of new skin lesions. Read more here.
  • Homeopathy: this is being advocated as a treatment for Sweet’s syndrome by some alternative therapists. This is a pseudoscientific claim, and there is no research to support this claim. In fact, in 2010, the House of Commons Science and Technology on Homeopathy stated that homeopathic remedies perform no better than placebos, and that the principles on which homeopathy is based are ‘scientifically implausible’ (NHS Choices, 2015a). Please take this into consideration before choosing to try homeopathy. However, if you do choose to try it, then it is probably safe.
  • Red root (blood root, bloodwort): this is being advocated as a treatment for Sweet’s syndrome by some alternative therapists. There is no evidence to support this claim, and red root should be used with caution, as it may not always be safe to use either orally or topically. When applied to the skin, it could potentially cause the development of new lesions, and should be completely avoided by those with certain health conditions. Read more here.
  • Some other herbs and supplements to be used with caution: there is no evidence to show that the following supplements can be used to treat Sweet’s syndrome. The algae chlorella is not suitable for those taking certain medications and could make the symptoms of autoimmune conditions, and possibly Sweet’s syndrome worse, particularly if the Sweet’s syndrome has developed secondary to an autoimmune condition. Alfalfa, astragalus, echinacea, and oral zinc should also be used with caution. Read more here.

References.

Clarys, P., Deliens, T., Huybrechts, I., Deriemaeker, P., Vanaelst, B., De Keyzer, W., Hebbelinck, M. and Mullie, P. (2014) Comparison of nutritional quality of the vegan, vegetarian, semi-vegetarian, pesco-vegetarian and omnivorous diet. Nutrients, Mar 24;6(3):1318-32 (online).

NHS Choices (2014) Chiropractic (online). Reviewed 20/08/15, and accessed 30/03/17.

NHS Choices (2015a) Homeopathy (online). Reviewed 15/02/15, and accessed 30/03/17.

NHS Choices (2015b) Osteopathy (online). Reviewed 10/06/15, and accessed 30/03/17.

Other information.

A warning about Polly Heil-Mealey! Sweet’s syndrome cannot be cured with herbs or homeopathic remedies.

What is the treatment for Sweet’s syndrome?

Additional note.

Sweet’s Syndrome UK does not promote the use of alternative or nutritional therapies. This is because there is no medical evidence to show that these therapies are effective, or sometimes even safe to use in those with Sweet’s syndrome. If anyone does have information that proves that alternative or nutritional therapies can be used to treat Sweet’s syndrome, I will be more than happy to read it. However, only peer-reviewed medical articles and case-studies will be accepted as evidence. The following will not be accepted as evidence: anecdotal evidence and personal stories; testimonials; YouTube videos; information on blogs or websites where there are no references or links to peer-reviewed medical articles or case-studies, or where the author is not willing to provide this information; blogs or websites where someone tries to pass off their feelings or instincts, beliefs or opinions as facts or evidence.

Thank you.

Michelle Holder.

© 2012-2017 Sweet’s Syndrome UK

Two neutrophilic dermatoses captured simultaneously on histology (Sweet’s syndrome and neutrophilic eccrine hidradenitis)

Links checked on 2/03/17.

This is the second reported case of Sweet’s syndrome and neutrophilic eccrine hidradenitis occurring in a patient with acute myeloid leukaemia at the same time (Wlodek et al, 2016).

Key points.

  • Sweet’s syndrome (SS) is a rare autoinflammatory (not autoimmune) condition and form of neutrophilic dermatosis (ND), and in 15-20% of patients can be triggered by cancer, including blood cancers.
  • Other forms of ND include neutrophilic dermatosis of the dorsal hands, Behcet’s syndrome, pyoderma gangrenosum, neutrophilic eccrine hidradenitis (NEH), erythema elevatum diutinum, and bowel-associated dermatitis-arthritis syndrome.
  • ND are skin conditions that occur as a result of lots of white blood cells called neutrophils infiltrating the tissues.
  • A number of ND are associated with cancer and their treatment, but more than one kind of ND rarely occurs together in the same patient at the same time.

Case-study.

This is a case of a 72-year-old man who was being treated for acute myeloid leukaemia (AML) with chemotherapy – daunorubicin and cytarabine. Within 48 hours of starting treatment he developed a fever, and two days later, wide-spread non-tender pink plaques (skin lesions that appear in the form of large raised areas) on the limbs and trunk. A skin biopsy showed lots of white blood cells in the tissues – lymphocytes and histiocytoid cells, but mainly neutrophils. Neutrophils had also infiltrated the fatty tissue under the skin, and this is known as panniculitis. All of these finding were consistent with SS. In addition, neutrophils and lymphocytes were also present around the sweat glands, and this is consistent with NEH. NEH is commonly caused by chemotherapy, including cytarabine, but can sometimes occur for other reasons.

The authors of this study have determined that the neutrophilic infiltrate that is found in a patient with SS has the potential to extend around the sweat glands, thus leading to NEH.

References.

Wlodek, C., Bhatt, N. and Kennedy, C. (2016) Two neutrophilic dermatoses captured simultaneously on histology. Dermatology Practical & Conceptual, Jul; 6(3): 55–57 (online).

Other information.

Copaescu, A., Castilloux, J., Chababi-Atallah, M., Sinave, C. and Bertand, J. (2013) A Classic Clinical Case: Neutrophilic Eccrine Hidradenitis. Case Reports in Dermatology, Sep-Dec; 5(3): 340–346 (online).

Tan, E. (2007) Skin toxicity of chemotherapy drugs. DermNet NZ (online). Accessed 2/03/17.

© 2012-2017 Sweet’s Syndrome UK

Mouth Ulcers and Sweet’s Syndrome

Updated 31/03/17.

Does Sweet’s syndrome cause mouth ulcers?

Yes. Occasionally, Sweet’s syndrome can cause mouth/oral ulcers (aphthous-like ulcers), but this is a symptom that is more commonly associated with the similar condition, Behcet’s syndrome.

Can Sweet’s syndrome cause other mouth problems?

Yes. On rare occasions, Sweet’s syndrome can cause other mouth problems, and also affect the throat. Symptoms include:

  • Cracks or fissures on the corners of the mouth (Contrucci and Martin, 2015).
  • Lesions on the inside of the lips (haemorrhagic bullae and vesicles, and necrotic nodules) (Cohen, 2007).
  • Lesions on the gums (haemorrhagic bullae and vesicles).
  • Necrotizing ulcerative periodontitis.
  • Enlargement of the gums (gingival hyperplasia) (Ibid).
  • Lesions on the tongue (aphthous-like ulcers, ulcers, and macerated papules) (Cohen, 2007; Kasirye et al, 2011: 135) .
  • Tongue pain, and swollen or enlarged tongue in association with lesions (Cohen, 2007; Contrucci and Martin, 2015; Kasirye et al, 2011: 135).
  • Lesions on the roof of the mouth (macerated papules, individual and grouped pustules, ulcers, and bullae) (Cohen, 2007; Contrucci and Martin, 2015).
  • Lesions affecting the pharynx (individual and grouped pustules) (Cohen, 2007).
  • Lesions on the inside of the cheeks (aphthous-like ulcers, and ulcers).
  • Inflammation of the saliva glands in the cheeks (parotitis) and associated cheek swelling (Jo et al, 2012).
  • Throat pain, painful swallowing, and hoarseness (Contrucci and Martin, 2015).

Read more about the symptoms of Sweet’s syndrome here.

Can mouth ulcers be treated or managed?

Yes. Mouth ulcers can be treated or managed, and the UK charity, the Behcet’s Syndrome Society (BSS), has put together a patient-information-leaflet to show you how. The information in this leaflet has been written for those with Behcet’s syndrome, but is also relevant to those with Sweet’s syndrome.


Information taken from the Behcet’s Syndrome Society leaflet – Behcet’s Disease and Mouth Ulcers (Birmingham Centre of Excellence, 2013).

Treatment.

General good oral hygiene is important with Behcet’s disease (*and Sweet’s syndrome), even when the mouth or gums are painful. Rinsing with mouthwash alone will not remove the dental plaque and is no substitute for brushing the teeth and flossing.

Typical adult toothpastes include detergents such as sodium lauryl sulphate (SLS) and flavouring agents that can exacerbate pain associated with oral ulceration. Toothpastes without SLS or prepared specifically for the ‘sore mouth’ are available. An alternative option is to use a children’s toothpaste. Toothpastes used by adults should include 1450 ppm fluoride.

Before considering the options listed below, you should discuss topical solutions with your specialist or doctor. Some of the remedies listed below are not licensed specifically for use in Behcet’s disease (*doxycycline and colchicine are recognised treatments for Sweet’s syndrome) so will need consideration by your medical professional. Many are prescription-only and will require ongoing monitoring.

Relief of pain.

Topical analgesia for oral ulceration is available as mouthwash and spray (Difflam). The mouthwash potentially allows more parts of the mouth to be reached than the spray but is less portable. A normal dose would be to rinse or gargle with 15 ml of the mouthwash every 1–3 hours as needed and spit it out. The preparation contains 10% alcohol, which can cause stinging when used with a sore mouth. Dilution with an equal volume of water can help. For the spray preparation, 4–8 sprays should be directed onto the affected area every 1–3 hours as needed.

Relief of inflammation and reduction in ulceration.

Topical corticosteroids reduce inflammation and are the mainstay of topical treatment. All topical corticosteroid therapies are best applied as soon as the ulcer starts to develop and should be continued until the ulcer has completely disappeared. A wide range of topical corticosteroids may be considered and food and drink should be avoided for at least 30 minutes following application.

For the treatment of a single or low number of ulcers.

Mucoadhesive buccal tablets (previously known as Corlan pellets) can be placed on the ulcers and allowed to dissolve. This should be done up to four times daily. However, some patients may find the tablets difficult to position in the correct place.

Alternatively, an aerosol preparation such as a steroid inhaler used in the management of asthma or allergic rhinitis (e.g. hay fever) may be considered. The aerosol can be sprayed directly onto ulcers. Suitable inhalers are beclometasone metered-dose inhaler 50–100 micrograms sprayed twice daily onto the affected area or fluticasone propionate aqueous spray 50 micrograms, 2 puffs sprayed on to the ulcers three times daily.

For treating several ulcers.

Corticosteroid mouthwashes can be used where there is widespread development of crops of ulcers. Betamethasone soluble 500 microgram tablets are licensed for the management of oral ulcers. One tablet should be dissolved in 10–15 ml of warm water and then gargled ensuring affected parts of the mouth are covered for up to 4 minutes. The solution should not be swallowed. The mouthwash should be used up to three times a day. Alternatively, soluble prednisolone 5 mg tablets dissolved in 10–15 ml of warm water can be used up to three times a day.

Protective barriers.

Mucosal coating agents are used to physically cover ulcerated areas to reduce unpleasant symptoms associated with activities such as speaking, smiling swallowing or yawning.

Pastes.

Carmellose sodium (Orabase) can be used to protect the sore areas of the mouth. It should be applied sparingly directly onto the ulcer when required. Application can be difficult to the tongue and the back of the mouth.

Topical gels.

Gelclair is a viscous gel specifically formulated to aid the management of inflammation of the oral mucosa. The gel can be used as a mouthwash up to three times daily after dilution or applied directly to the affected site using a clean finger or swab such as a cotton bud. The mouthwash is prepared by diluting the contents of one sachet with 3 tablespoons of water. The solution is then rinsed around the mouth for 1 minute and provides a protective coat over the mucosa.

Anti-microbial agents.

Anti-microbial agents are used to control pain by reducing the secondary infection associated with mucosal ulceration.

Chlorhexidine mouthwash, gel or spray.

Chlorhexidine has a broad anti-microbial spectrum. Preparations are licensed for the management of aphthous ulcers.

For chlorhexidine 0.2% mouthwash, 10 ml of solution should be rinsed around the mouth for 1 minute twice daily and then spat out. Alternatively, an oral spray – Corsodyl (chlorhexidine 0.2%) – may be used with up to 12 applications of spray used twice daily. Chlorhexidine gel preparations can be applied directly to the ulcer or brushed on the teeth once or twice daily. Preparations available include Corsodyl gel (chlorhexidine 1%) and Curasept gel (chlorhexidine 5%).

A number of chlorhexidine preparations contain alcohol, which can irritate the oral mucosa. However, alcohol-free mouthwashes can be found, including Corsodyl, Curasept and Periogard. The Curasept gel formulation is also alcohol-free.

Doxycycline.

Doxycycline has antibacterial and anti-inflammatory properties and can be used when the use of chlorhexidine has failed. Its main value in treating mouth ulcers comes from its anti-inflammatory action. The contents of one doxycycline 100 mg capsule should be dissolved in 10–15 ml water. Again, the solution should be held in the mouth for up to 4 minutes, ensuring that the solution comes into contact with the affected parts of the mouth. This should be done at least four times a day for 3 days. The solution should not be swallowed, and food and drink should be avoided for 30 minutes after use of the preparation. Prolonged use should be avoided, as this can increase the risk of oral infections such as candidiasis (thrush).

Colchicine.

For recurrent oral ulceration that has failed to respond to topical treatments alone, oral colchicine may be prescribed by your doctor.

* Additional notes added to the text – Michelle Holder, Sweet’s Syndrome UK.


For patients in the United States with an autoinflammatory condition, including Sweet’s syndrome.

Information taken from the SAID Support blog – Mouth Ulcer Treatment and Prevention (Tousseau, 2013).

Prescription Magic Mouthwash.

Magic mouthwash (*not available in the UK) is a prescription mouthwash that you use to rinse and then spit. It primarily contains lidocaine, which numbs the mouth. There are different brands available that may also contain hydrocortisone to reduce inflammation, the antifungal medication nystatin, an antibiotic, and/or the antacid magnesium hydroxide to coat the mucus membranes inside the mouth.

Milk of Magnesia and Liquid Benadryl.

This is a liquid mouthwash you can make at home, but discuss this with your doctor before you begin using this treatment. Mix 1/2 teaspoon each of milk of magnesium and liquid Benadryl. Swish it in the mouth and then spit it out. Do not drink it. Some people mix it up, and squirt it into the mouth with an oral medicine syringe to coat the mouth, then spit it out.

Hydrogen Peroxide and Milk of Magnesia.

Dab a drop of hydrogen peroxide mixed with water directly on the ulcer with a cotton swab. The National Institutes of Health (NIH) recommends mixing one part hydrogen peroxide to one part water followed by a dab of Milk of Magnesia directly onto the sore. You can do this three to four times a day.

B Vitamins.

Folic acid and vitamin B12 deficiencies can cause mouth ulcers. A daily B vitamin supplement may help reduce or prevent mouth sores.

*Sweet’s syndrome-related mouth ulcers are not caused by vitamin deficiency, but a daily B vitamin may help to reduce your overall risk of developing non-Sweet’s syndrome-related mouth ulcers.

A Benzocaine Warning.

In 2011, the FDA released a warning that using products containing benzocaine could lead to methemoglobinemia, a rare blood disorder. Most of the cases reported were in children under that age of two who were treated with benzocaine gel products for teething pain. Adult cases have also been reported. The FDA recommends that benzocaine products not be used on children younger than two without medical supervision.

*In the UK, the National Institute for Health and Care Excellence (NICE) does not recommend the use of benzocaine or any other topical anaesthetic in children under the age of two, unless on the advice of a health professional or under medical supervision (CKS, 2014).

* Additional notes added to the text – Michelle Holder, Sweet’s Syndrome UK.


New research.

Anakinra.

Anakinra (Kineret) 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: Simon et al, 2014). The results of a recent trial have also shown that anakinra at an optimal dose of 200mg daily is partially effective in the treatment of resistant oral and genital ulcers in Behcet’s syndrome (Grayson et al, 2017).


References.

Birmingham Centre of Excellence (2013) Behcet’s Disease and Mouth Ulcers. Behcet’s Syndrome Society (PDF).

CKS: Clinical Knowledge Summaries (2014) Teething – Topical Anaesthestics. NICE: National Institute for Health and Care Excellence (online).

Cohen, P. (2007) Extracutaneous Manifestations: Table 4. In Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis, Orphanet Journal of Rare Diseases (online).

Contrucci, R. and Martin, D. (2015) Sweet syndrome: A case report and review of the literature. ENT Journal, July;94(7):282-284 (online). Sign-up to the ENT Journal for free to access the full article.

Grayson, P. Yazici, Y., Merideth, M., Sen, H., Davis, M., Novakovich, E., Joyal, E., Goldbach-Mansky, R. and Sibley, C. (2017) Treatment of mucocutaneous manifestations in Behçet’s disease with anakinra: a pilot open-label study. Arthritis Research & Therapy, Mar 24;19(1):69 (online).

Jo, M., Lim, Y., Shin, H., Choe, J., Seul, J. and Jang T. (2012) A Case Report of Sweet’s Syndrome with Parotitis. Archives of Plastic Surgery, Jan;39(1):59-62 (online).

Kasirye, Y., Danhof, R., Epperla, N. and Garcia-Montilla, R. (2011) Sweet’s Syndrome: One Disease, Multiple Faces. Clinical Medicine & Research, Nov;9(3-4):134-136 (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).

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

Tousseau, J. (2013) Mouth Ulcer Treatment and Prevention. SAID Support (online).

Other information.

Ngan, V. and Oakley, A. (2016) Aphthous Ulcers (online). Initially published in 2003, and updated by Professor A. Oakley, Jan 2016. Accessed 30/03/17.

© 2012-2017 Sweet’s Syndrome UK