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 have been 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 is a more common trigger for Sweet’s syndrome than vaccination, and 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). It is most commonly triggered by upper respiratory tract infection, but can be triggered by other infections too.
  • 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 or contain dangerous chemicals?

No. Vaccinations do not trigger Sweet’s syndrome because they are toxic or contain dangerous chemicals, and anyone who tells you this is either lying to you, trying to scare you, or has no understanding of vaccinations and 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 – 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 – in a way that it shouldn’t, i.e. is hypersensitive and goes into overdrive, overreacting 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 unnecessarily activate or activate too many inflammatory cells, mainly 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, e.g. they are taking certain types of medication or have other health conditions. 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

What NOT to say to someone with Sweet’s syndrome!

Image: ‘You’ve got it backwards…’, someecards.

If  you know someone with Sweet’s syndrome (SS), please don’t say the following things to them. Unfortunately, they are things that people with SS hear all too often, and even though a few of them are well-meant, some are incredibly insulting.

What NOT to say to someone with Sweet’s syndrome!

1. ‘But you don’t look sick’ or ‘Are you faking it?’

The commonest symptom of SS is tender or painful skin lesions, but these are often covered up in some way, e.g. with make-up, by hair or clothing. Also, most of the symptoms of SS cannot be seen, but just because you can’t see them, doesn’t mean that someone with SS isn’t very sick or that they’re faking being ill.

2. ‘You should try this new diet or supplement. It can’t hurt to give it a try.’

SS is an autoinflammatory condition caused by errors in the innate system, and there is no special diet or supplement that can correct these errors. Sometimes, a change in diet or certain vitamins and supplements might help to improve overall health, but they are certainly not a replacement for proper medical treatment. Also, some diets and supplements can do more harm than good, and be very costly. For example, restrictive diets can lead to nutritional deficiency and health problems; certain supplements may not be safe to take or interact with medications; special diets and supplements can be something that many people with SS really can’t afford to buy, and their money could be better spent elsewhere.

3. ‘I’m too busy to get ill’ or ‘You need to keep busy and just get on with it!’

NEVER smugly say to someone with SS, ‘I’m too busy to get ill’. Being busy is not a protection against illness, and no matter how busy or in demand you are, illness can still affect you and stop you from doing the things that you want to do at any time. People with SS don’t choose to get ill, and even when they desperately want to just ‘get on with it!’, they can’t. Also, keeping busy isn’t going to make SS go away or lessen its impact, and doing too much can sometimes make symptoms worse.

 4. ‘I wish I had the luxury of being sick so that I could stay at home all day’.

Being sick is not a luxury, and most people with SS would give anything to be well again. They do not enjoy being ill (do you enjoy it?), and can get very frustrated and depressed because they can no longer work or do the things that they once did. In fact, many with long-term SS go through a grieving process where they mourn the loss of the person that they were and the life that they once had.

5. ‘If you learnt to cope better or didn’t get stressed then you wouldn’t be sick’.

As already mentioned, SS is caused by errors in the innate immune system, and at present, there is no evidence to show that it is directly caused by stress. Some people do find that their SS gets worse when they are stressed, but this might happen for a number of different reasons, including their steroid medication being reduced or stopped. However, in others, their condition flares-up when they are not feeling stressed at all. Also, it is ridiculous to expect those with SS to avoid stress completely. Living with SS can be very stressful in itself, and for all of us, stress is part of everyday life.

6. ‘Don’t give in’, ‘Stop complaining’ or ‘Why do you have to be so negative?’

People with SS are not giving in or being negative when they admit to having a bad day or struggling to cope with their condition. They have the right to have a bad day, just the same as anyone else. NO-ONE can be upbeat, positive and optimistic all of the time, and having a moan or rant or even a bit of a cry can sometimes be very healthy. It can help you to release your feelings and reduce emotional stress.

© 2012-2017 Sweet’s Syndrome UK

Can medication trigger Sweet’s syndrome?

Updated 03/03/17.

Can medication trigger Sweet’s syndrome?

Yes. In up to 5% of cases, Sweet’s syndrome is triggered by medication (Cohen, 2007). This is known as drug-induced Sweet’s syndrome.

How will you know if your Sweet’s syndrome has been triggered by medication?

In at least 95% of patients with Sweet’s syndrome, their condition is not triggered by medication. However, drug-induced Sweet’s syndrome should be considered if:

  • Your Sweet’s syndrome developed not long after a particular medication was started.
  • Your Sweet’s syndrome has continued to persist for many months or years, even after treatment.

What will happen if your doctor thinks you have drug-induced Sweet’s syndrome?

Unfortunately, there is no special test to tell you whether or not your Sweet’s syndrome is being triggered by medication. However, if it is suspected that your Sweet’s syndrome is drug-induced, your doctor will:

  • Stop the medication that is possibly causing your Sweet’s syndrome. Your Sweet’s syndrome should then start to settle down, but you may still need treatment.
  • Re-introduce the medication (rechallenge) to see if your Sweet’s syndrome flares-up again. Sometimes, your doctor will decide that this is not necessary.

Why does medication trigger Sweet’s syndrome in some people?

Drug-induced Sweet’s syndrome is often caused by hypersensitivity reaction, but it can sometimes happen for other reasons too, e.g. a treatment that increases white cell production, or causes hormonal changes. Read more here.

What is hypersensitivity reaction, and is it the same as allergic reaction?

No. Hypersensitivity reaction is not the same as allergic reaction.

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 – 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 – in a way that it shouldn’t, i.e. is hypersensitive and goes into overdrive, overreacting 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 unnecessarily activate or activate too many inflammatory cells, mainly white blood cells called neutrophils (Gosheger et al, 2002: 70). This then leads to the symptoms of Sweet’s syndrome. Read more here.

Allergic reaction.

The most common type of allergy is an IgE-mediated allergy. This is an adverse reaction that the body has to a particular substance that is foreign to the body, e.g. a food, pollen, or pet hair, that does not normally cause harm. This substance is known as an allergen (a type of antigen). Allergic reaction occurs when the immune system mistakes an allergen for a foreign invader such as a bacteria or virus. The adaptive immune system then quickly produces allergen-specific immunoglobulin E (IgE) antibodies in response to this, in order to fight the allergen off. Chemicals such as histamine are also produced, with the overall immune response causing the symptoms of allergy.

What medications have been reported to have triggered Sweet’s syndrome?

Medications that have been reported to trigger Sweet’s syndrome include:

Analgesics (non-opioids).

  • Paracetamol (triggered a Sweet’s syndrome-like condition) (Culla et al, 2014).

Antibiotics.

  • Amoxicillin (possibly) (Volpe, 2016).
  • Clindamycin (Cruz-Velasquez et al, 2016).
  • Tetracycline.
  • Doxycycline (Ibid).
  • Minocycline (Cohen, 2007).
  • Nitrofurantoin.
  • Norfloxacin.
  • Ofloxacin.
  • Trimethoprim/sulfamethoxazole.
  • Quinupristin/dalfopristin (Ibid).
  • Piperacillin/tazobactam (Cruz- Velasquez et al, 2016).

Anti-epileptics.

  • Carbamazepine (Cohen, 2007).
  • Diazepam.

Anti-hypertensives.

  • Hydralazine (Cohen, 2007).

Anti-malarials.

  • Chloroquine (Cruz-Velasquez et al, 2016).

Anti-manic agents.

  • Lithium (Xenophontos et al, 2016).

Anti-neoplastics.

  • Bortezomib (Llamas-Velasco et al, 2015).
  • Decitabine (Kasirye et al, 2011: 134).
  • Imatinib mesylate (Cohen, 2007).
  • Ipilimumab (Gormley et al, 2014).
  • Lenalidomide (Cohen, 2007).
  • Obinutuzumab (triggered a Sweet’s syndrome-like condition) (Korman et al, 2016).

Anti-viral drugs.

  • Abacavir (Cohen, 2007).
  • Acyclovir (Cruz-Velasquez et al, 2016).
  • Interferon-α.

Colony stimulating factors.

  • Granulocyte-colony stimulating factor (G-CSF). This is the most common treatment to trigger Sweet’s syndrome (Cohen, 2007).
  • Granulocyte-macrophage-colony stimulating factor (GM-CSF).
  • Pegfilgrastim (Ibid).

Contraceptives.

  • Levonorgestrel/ethinyl estradiol (Triphasil) (Cohen, 2007).
  • Levonorgestrel-releasing intrauterine system (Mirena).

Diuretics.

  • Furosemide (Cohen, 2007).

Immunosuppressants.

  • Azathioprine (Salem et al, 2015). Sometimes, azathioprine-induced Sweet’s syndrome can be confused with azathioprine hypersensitivity syndrome (AHS) (Aleissa et al, 2017). This is a rare adverse reaction occurring a few days to weeks after azathioprine has been given. AHS can sometimes mimic Sweet’s syndrome, and an azathioprine rechallenge is not advised, as it may lead to a severe adverse reaction or even death.

Nonsteroidal anti-inflammatory drugs (NSAIDs).

  • Celecoxib (Cohen, 2007; Oh et al, 2016).
  • Rofecoxib (Cruz-Velasquez et al, 2016).
  • Diclofenac (Cohen, 2007; Gupta et al, 2015).

Platelet aggregation inhibitors.

  • Ticagrelor (Ikram and Veerappan, 2016).

Proton-pump inhibitors.

  • Esomeprazole (Cohen, 2015).
  • Omeprazole.

Psychotropics.

  • Clozapine (Cohen, 2007).
  • Amoxapine (Cruz-Velasquez et al, 2016).
  • Diazepam.
  • Lormetazepam (Ibid).

Retinoids.

  • All-trans retinoic acid (Cohen, 2007; Tam and Ingraffea, 2015).
  • 13-cis-retinoic acid (isotretinoin) (Cohen, 2007).

Sulfa drugs.

  • Sulfasalazine (Romdhane et al, 2016).

Thyroid drugs.

  • Propylthiouracil (Cruz-Velasquez et al, 2016).

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.

Sweet’s syndrome triggered by vaccination is incredibly rare (only 10 cases reported in medical literature in the past 42 years), and a definite connection has not been established in all the listed cases. Also, as infection is a more common trigger for Sweet’s syndrome than vaccination, you may be more likely to develop Sweet’s syndrome as a result of not having your vaccinations than having them.

If someone tells you that Sweet’s syndrome is triggered by vaccination because they are toxic or poisonous or contain dangerous chemicals, then they either have no understanding of Sweet’s syndrome and vaccinations, are not telling you the truth, or are trying to scare you, and will have no evidence to support their claims. Sweet’s syndrome triggered by vaccination occurs because of errors in the innate immune system, and this part of the immune system responding to antigens in the way that it shouldn’t. It is nothing to do with vaccines containing dangerous chemicals.

Xanthine oxidase inhibitors.

  • Allopurinol (Polimeni et al, 2015).

Other.

  • X-ray contrast agents (Cruz-Velasquez et al, 2016).

References.

Aleissa, M., Nicol, P., Godeau, M., Tournier, E., de Bellissen, F., Robic, M., Livideanu, C., Mazereeuw-Hautier, J. and Paul, C. (2017) Azathioprine Hypersensitivity Syndrome: Two Cases of Febrile Neutrophilic Dermatosis Induced by Azathioprine. Case Reports in Dermatology, Jan 19;9(1):6-11 (0nline).

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).

Cohen, P. (2015) Proton pump inhibitor-induced Sweet’s syndrome: report of acute febrile neutrophilic dermatosis in a woman with recurrent breast cancer. Dermatology Practical & Conceptual, April; 5(2):113–119 (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.

Culla, T., Amayuelas, R., Diez-Canseco, M., Fernandez-Figueras, M., Giralt, C. and Vazquez, M. (2014) Neutrophilic dermatosis (Sweet’s syndrome-like) induced by paracetamol. Clinical and Translational Allergy, Jul; 4(Suppl 3): P83 (online).

Gormley, R., Wanat, K., Elenitsas, R., Giles, J., McGettingan, S., Schucher, L. and Takeshita, J. (2014) Ipilimumab-associated Sweet syndrome in a melanoma patient. Journal of the American Academy of Dermatology, Nov;71(5):e211-3 (online).

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).

Gupta, S., Bajpai, M. and Uraiya, D. (2015) Diclofenac-induced sweet’s syndrome. Indian Journal of Dermatology;60:424 (online).

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).

Ikram, S. and Veerappan, V. (2016) Ticagrelor-induced Sweet Syndrome: an unusual dermatologic complication after percutaneous coronary intervention. Cardiovascular Intervention and Therapeutics, May 4th (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).

Korman, S., Hastings, J. and Byrd, J. (2016) Sweet-Like Eruption Associated With Obinutuzumab Therapy for Chronic Lymphocytic Leukemia. JAMA Dermatology, Nov 23 (online).

Llamas-Velasco, M., Concha-Garcon, M., Fraga, J. and Arageus, M. (2015) Histiocytoid sweet syndrome related to bortezomib: A mimicker of cutaneous infiltration by myeloma. Indian Journal of Dermatology, Venereology and Leprology, May;81:305-6 (online).

Oh, E., Shin, J., Hong, J., Kim, J., Ro, Y. and Ko, J. (2016) Drug-induced bullous Sweet’s syndrome by celecoxib. The Journal of Dermatology, Apr 6 (PubMed).

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).

Polimeni. G., Cardillo, R., Garaffo, E., Giardina, C., Macrì, R., Sirna, V.,  Guarneri, C. and Arcoraci, V. (2015) Allopurinol-induced Sweet’s syndrome. International Journal of Immunopathology and Pharmacology, Dec 18th (PubMed).

Romdhane, H., Mokni, S., Fathallah, N., Ghariani, N., Sriha, B. and Salem, B. (2016) Sulfasalazine-induced Sweet’s syndrome. Therapie, Jun;71(3):345-347 (PubMed).

Salem, C., Larif, S., Fathallah, N., Slim, R., Aounallah, A. and Hmouda, J. (2015) A rare case of azathioprine-induced Sweet’s syndrome in a patient with Crohn’s disease. Current Drug Safety, July (PubMed 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). 

Volpe, M. (2016) Sweet Syndrome Associated with Upper Respiratory Infection and Amoxicillin Use. Cureus, Apr; 8(4): e568 (online).

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).

Xenophontos, E., Ioannou, A., Constantinides, T. and Papanicolaou. E. (2016) Sweet syndrome on a patient with autoimmune hepatitis on azathioprine and CMV infection. Oxford Medical Case Reports, Feb; (2): 24–27 (online).

Other information.

Cetin, G., Sayarlioglu, H., Erhan, C., Kahraman, H., Ciralik, H. and Sayarlioglu, M. (2014) A case of neutrophilic dermatosis who develop palpable purpura during the use of montelukast. European Journal of Dermatology,  Dec; 1(4): 170–171 (online).

© 2012-2017 Sweet’s Syndrome UK

10 myths about Sweet’s syndrome

Myth no.1: Sweet’s syndrome is a condition that is caused by eating too much sugar or is something to do with diabetes.

Fact: Sweet’s syndrome or acute febrile neutrophilic dermatosis is nothing to do with sugar or diabetes. It is a rare autoinflammatory condition and neutrophilic dermatosis. It is called Sweet’s syndrome because it was a Dr. Robert Sweet who first described the condition in 1964. Read more here.

Myth no.2: Sweet’s syndrome is an autoimmune condition.

Fact: Sweet’s syndrome is an autoinflammatory and not an autoimmune condition. These conditions are similar, but involve different parts of the immune system. Read more here.

Myth no.3: Sweet’s syndrome only affects the skin.

Fact: Skin lesions are usually one of the main symptoms of Sweet’s syndrome. However, Sweet’s syndrome doesn’t just affect the skin, but causes lots of other symptoms too. Sometimes, it can make a person seriously ill, and on rare occasions may be life-threatening. Read more here.

Myth no.4: People with Sweet’s syndrome only have skin problems because of poor hygiene.

Fact: In people with Sweet’s syndrome, the skin lesions are caused by the activation of inflammatory cells, mainly white blood cells called neutrophils. They are not caused by poor hygiene. Read more here.

Myth no.5: Sweet’s syndrome is contagious.

Fact: Sweet’s syndrome is not an infection, so cannot be ‘caught’. See Myth no.1 & 2.

Myth no.6: If people with Sweet’s syndrome spent more time in the sun then their skin problems would go away.

Fact: Even though exposure to sunlight might be beneficial to people with certain conditions affecting the skin, in some people with Sweet’s syndrome, overexposure to sunlight or ultraviolet (UV) light can trigger the development of new skin lesions. Read more here.

Myth no.7: Skin problems in people with Sweet’s syndrome can be treated with essential oils.

Fact: In some people with Sweet’s syndrome, trying to treat the skin lesions with essential oils could irritate the skin and make things worse. This is because of pathergy, i.e. when skin damage or irritation triggers the development of new lesions. Read more here.

Myth no.8: Sweet’s syndrome is caused by diet or dietary toxins, e.g. gluten or dairy.

Fact: Sweet’s syndrome is not caused by diet or dietary toxins, and cannot be treated or cured with a special diet. It is caused by errors in a part of the immune system called the innate system, and involves factors such as genetic susceptibility, cytokine dysregulation, and hypersensitivity reaction (not the same as allergic reaction). Read more here.

Myth no.9: Boosting the immune system will cure Sweet’s syndrome.

Fact: In people with Sweet’s syndrome, the innate immune system is overactive and not underactive. Boosting the immune system, i.e. increasing immune system activity, will not help, and might even make symptoms worse if the Sweet’s syndrome has developed secondary to an autoimmune condition. Read more here.

Myth no.10: You only have Sweet’s syndrome because you get stressed.

Fact: At present, there is no evidence to prove that Sweet’s syndrome is directly caused by stress. See Myth 1, 2, 4, 8 & 9. Some people find that their Sweet’s syndrome gets worse when they are stressed, but this could be happening for a number of different reasons, including steroid medication being reduced or stopped.

© 2012-2017 Sweet’s Syndrome UK

[BLOG] Periodic Fever Syndromes in Adults

Links checked 2/03/17.

SAID Support has posted a very informative blog article on autoinflammatory conditions in adults.

Periodic Fever Syndromes in Adults.

Posted on 28th October 2015 by Jennifer Tousseau (author) & Karen Durrant RN (editor).

‘Autoinflammatory diseases, also commonly known as periodic fever syndromes, are systemic inflammatory diseases caused by malfunctions of the innate immune system. Most autoinflammatory diseases are genetic and the first symptoms start in childhood, and most are chronic lifelong diseases.  However, due to the complex range of symptoms and their rarity, patients with these conditions may not get diagnosed or helpful treatment until they are adults.

In addition, some patients may be diagnosed with autoinflammatory disease symptoms, but the cause may not have been classified, or determined yet, and these patients are still seeking a diagnosis, often into adulthood.  But the following common symptoms and labs may be noted in these patients.  Experts on autoinflammatory diseases estimate that up to 2/3 of their patient population has a systemic undifferenciated autoinflammatory syndrome (SURFS), so it is important to consider systemic autoinflammatory diseases as a possible diagnosis in a patient that has the following symptoms, even if the genetic cause has not yet been identified.

Typical Autoinflammatory Symptoms and Lab Results.

Symptoms vary between the different autoinflammatory syndromes, but there are some common symptoms that many share:

  • recurrent fevers (without an infectious cause)
  • rashes
  • joint pain and/or arthritis
  • mouth ulcers
  • gastrointestinal symptoms including vomiting and/or diarrhoea
  • stomach or chest pain
  • red eyes (non-infectious conjunctivitis) – some may even get uveitis and/or iritis (inflammation in the eyes).

Many autoinflammatory diseases have similar lab results including:

  • high inflammatory markers (CRP, ESR, SAA)
  • high WBC
  • most have negative ANA and other autoantibody tests.

Some autoinflammatory diseases cause chronic inflammation that can affect any organ system in the body over time. This includes:

  • sensorineural hearing loss
  • enlarged liver and/or spleen
  • bone involvement
  • secondary amyloidosis
  • aseptic meningitis (either during flares or chronically, depending on the autoinflammatory disease).

Depending on the syndrome, many will have flares of symptoms that can last for a few days to several weeks, while some will have continuous inflammatory symptoms. Between flares, some patients may be asymptomatic with normal lab values. But for those with mild to moderate chronic symptoms, even when they do not have a fever, they may have elevated inflammatory markers, other abnormal labs and/or symptoms related to chronic inflammation.

It’s important to note that the above list represents the most common presentation of many autoinflammatory diseases, but there are some who do not have typical symptoms.

It is essential that physicians consider autoinflammatory diseases as a possible diagnosis when they are presented with a patient who has recurrent fevers, especially since childhood, along with other symptoms such as rash and/or arthritis.  Although these are rare diseases, they are often misdiagnosed for years, or patients go undiagnosed into adulthood. With genetic testing and resources to compare symptoms doctors should be able to diagnose, and treat more patients.’

Read the rest of the blog here.

N.B. The autoinflammatory condition Sweet’s syndrome (SS) rarely affects children. Unlike in most autoinflammatory conditions, a genetic cause for SS is extremely rare, but it is more common in those with the genetic marker HLA-B54.

© 2012-2017 Sweet’s Syndrome UK

[INFO] A brief description of Sweet’s Syndrome

Updated 3/03/17.

This is a brief description of Sweet’s syndrome that you can share with friends and family.

What is Sweet’s Syndrome?

Sweet’s syndrome (SS) is a rare autoinflammatory condition and form of neutrophilic dermatosis that causes fever, painful skin lesions, fatigue, headaches, muscle pain (myalgia), joint pain (arthralgia), joint pain and swelling (arthritis), eye problems, and sometimes mouth problems. You may experience it once, or have repeat flare-ups. Most lesions appear on the face, neck, and upper extremities, but can develop on other parts of the body. However, lesions are not always present. On rare occasions, the lesions can develop in internal joints and organs causing a variety of other symptoms. There are also several variant (unusual) forms of SS.

What causes it?

The cause of SS is not fully understood, but involves errors in the innate immune system. There are also potential triggers. These include infection, autoimmune conditions, inflammatory bowel disease, cancer and blood disorders, medications, pregnancy, skin damage, overexposure to sunlight or ultraviolet (UV) light, and vaccination. In regards to the latter, SS caused by vaccination is so rare (only 10 cases reported in medical literature in the past 42 years) that a definite connection has not been established in all cases. Also, as infection is a more common trigger for SS than vaccination, you may be more likely to develop SS as a result of not having your vaccinations than having them.

How is it diagnosed?

  •  A diagnosis of SS is made by taking your medical history to see if you have any health conditions or are taking any medications that could cause it.
  • Your temperature will be taken. SS patients normally have a fever of 38 degrees centigrade or above.
  • Your dermatologist will need to take a lesion sample (biopsy) for detailed microscopic examination to rule out any other conditions that might be confused with SS.
  • Blood tests will be needed to detect any inflammation or infection in the body, and some additional tests might be needed to rule out other conditions as a cause for SS.

How is it treated?

Steroid medication is the main form of treatment. Other treatments include colchicine, potassium iodide, dapsone, indomethacin, clofazimine, ciclosporin, and more.

© 2012-2017 Sweet’s Syndrome UK