Challenges of Living with Sweet’s Syndrome

Sweet’s syndrome (SS) is a rare autoinflammatory condition and neutrophilic dermatosis. The most common symptom is skin lesions, but what some people don’t know is that it can cause lots of other symptoms too. On rare occasions, it can even be life-threatening, and approximately 15-20% of patients will develop SS secondary to some form of cancer, most commonly, a group of blood disorders called myelodysplastic syndromes (MDS).

Amongst the SS community, those with SS are typically referred to as ‘Sweeties’, and for them, living with SS can be incredibly difficult. This is not only due to the symptoms of SS, but to the many obstacles and unexpected challenges that they can face along the way. For example, problems associated with a change in appearance, depression, debilitating fatigue, isolation, pain, mobility issues, job loss and financial difficulties, to name but a few.

Living with Sweet’s syndrome. How can it affect a Sweetie?

Change in appearance.

  • Visible skin lesions.
  • Permanent skin discoloration or scarring.
  • Weight gain due to reduced mobility or side-effects of medication.
  • Rarely, hair loss due to the side-effects of medication.

Change in mood, depression or other changes in mental health.

  • Increased risk of anxiety or depression due to having to live or deal with certain difficulties or symptoms, e.g. not well enough to socialise, debilitating fatigue, or frequent or chronic pain.
  • Changes in mood due to the side-effects of medication.
  • Mental changes associated with the rare neurological variant, neuro-Sweet’s disease.

Loss of confidence.

  • Due to change in appearance.
  • Due to being insulted or verbally abused because of a change in appearance.

Isolation.

  • Not wanting to go out due to change in appearance.
  • Being too ill or in too much pain to work or socialise.
  • Symptoms of SS or the side-effects of medication making it difficult or impossible to do certain activities.
  • Others not wanting to associate with Sweeties because they think that their skin lesions can be ‘caught’.

Pain.

  • Painful skin lesions.
  • Headaches, less commonly, migraine.
  • Muscle pain.
  • Joint pain.
  • Eye pain associated with certain eye symptoms.
  • Occasionally, painful mouth ulcers or other types of oral lesion.
  • Rarely, pain as a result of other parts of the body being affected, including internal organs.

Mobility issues.

  • Difficulty walking and moving about because of joint pain and swelling.
  • Painful skin lesions affecting or restricting movement.
  • Neuro-Sweet’s disease causing limb weakness, or problems with co-ordination and balance.
  • Fatigue causing limb heaviness.

Lack of sleep.

  • Pain or other symptoms of SS making it difficult to sleep.
  • Side-effects of medication negatively affecting sleep, e.g. insomnia or nightmares.

Difficulty accessing information and treatment.

  • Struggling to find a doctor who has any understanding of SS.
  • Struggling to access patient information, on or off-line.
  • Health care staff unable to answer any questions that a Sweetie might have, and not able to provide information when requested.
  • Health care staff not taking SS seriously enough, or insulting a Sweetie because they don’t understand SS.
  • Having to wait a long time for the right treatment, or being denied access to certain treatments.

Difficulties associated with work.

  • Sweeties being bullied by work colleagues or customers because of their appearance.
  • Being bullied by an employer because they have to take time off, and are therefore viewed as a malingerer or a costly drain on resources. For example, having to take sick-leave when ill, being admitted to hospital, or having to attend doctor or hospital appointments on a regular basis.
  • Sweeties having to reduce their hours, even if they can’t afford to.
  • Difficultly gaining promotion, demotion or job loss.

Financial difficulties.

  • Struggling to afford to pay for care if a Sweetie lives in a country where health care is privatised.
  • Financial difficulties due to having to reduce hours at work, leave a job, being bullied out of a job or being sacked.
  • Struggling to access benefits due to the assessors or doctors having a poor understanding of SS and it not being recognised by the benefits system. This can happen even when a Sweetie is seriously ill.

Unsupportive family and friends.

  • Family and friends refusing to take SS seriously enough, and telling Sweeties to ‘just get on with it!’
  • Family and friends giving unhelpful advice and telling Sweeties that they are choosing to be ill (‘patient blaming’). For example, ‘ You wouldn’t be ill if you just ate more healthily, cut out gluten, didn’t get stressed, tried this herbal supplement, spent time in the sun, put essential oils on your lesions, or tried homeopathy.’ Some of these things can even make SS worse.
  • Getting annoyed and angry with a Sweetie because they can’t do what they once did, or are no longer in a position to help out others in the way that they did before.

There are many more challenges, obstacles and difficulties that could be added to this list, so please feel free to mention your own in the comments section below this post.

Today is Sweet’s Syndrome UK Day! Please share this post to help raise awareness of SS and the difficulties that those with this condition have to deal with, often on a daily basis. Also, a big thank you to all the health care staff, employers and work colleagues, family members and friends who DO support Sweeties across the globe. You do an amazing job, and frequently have to face your own challenges when it comes to helping and supporting those with SS. 💛

© 2012-2017 Sweet’s Syndrome UK

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Sweet’s Syndrome in Children

Updated 06/09/17.

Sweet’s syndrome in children is very rare, so the information available is limited.

What is Sweet’s syndrome?

Sweet’s syndrome (SS) is a rare autoinflammatory condition and form of neutrophilic dermatosis that mainly affects adults, particularly women. 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 (medical term that includes a number of different symptoms, and is not the same as simply feeling tired); muscle pain; joint pain (arthralgia) or joint pain and swelling (arthritis); headaches; eye problems; sometimes, mouth ulcers. Read more here.

Can Sweet’s syndrome affect children?

Yes. SS most commonly affects adults, but on very rare occasions – only in 5% to 8% of cases – can affect babies, children and teenagers (Doinita et al, 2016; Sharma et al, 2015).

By August 2017, at least 82 cases of SS in children had been documented in medical literature.

A list of documented cases of Sweet’s syndrome in children.

1. By 2012, only 68 cases of SS in children had been documented in medical literature, and 58% of these cases were associated with underlying chronic disease (Gray et al, 2012). Out of the 68 cases, SS occurred in association with:

  • Aortitis (Gray et al, 2012).
  • Multifocal osteomyelitis.
  • Vasculitis.
  • Acute myeloid leukaemia.
  • Acute lymphoblastic leukaemia.
  • Juvenile chronic myelomonocytic (myelogenous) leukaemia.
  • Fanconi anaemia.
  • Aplastic anaemia.
  • Viral infection.
  • Medication (drug-induced).
  • Neonatal lupus erythematosus.
  • Primary immunodeficiency (PI) – PIs are a group of more than 300 rare hereditary or genetic chronic disorders, in which part of the body’s immune system is missing or doesn’t work in the right way.
  • Immunodeficiency secondary to HIV (human immunodeficiency virus) infection (Ibid).
  • 2 brothers with Majeed syndrome (congenital dyserythropoietic anaemia, chronic recurrent multifocal osteomyelitis and neutrophilic dermatosis) – a very rare genetic autoinflammatory condition that has only been reported in four families from the Middle East (El-Shanti and Ferguson, 2014; Majeed et al, 1989).
  • 3 children had probable CANDLE syndrome (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) – a rare genetic autoinflammatory condition (Gray et al, 2012).

2. In 2013, the first known case of hyper IgD (hyperimmunoglobulinemia) syndrome or HIDS presenting as SS in a 6-week-old girl (Payne et al, 2013). HIDS, a milder form of Mevalonate Kinase Deficiency (MKD), is a genetic autoinflammatory condition.

3. In 2014, three cases of SS were documented:

  • A case of drug-induced SS in a 20-month-old boy with congenital neutropenia who was being treated with granulocyte colony-stimulating factor (G-CSF) (Akilov et al, 2014).
  • In a 5-year-old child with erythema elevatum diutinum (Wang et al, 2014).
  • In a 14-year-old boy with Crohn’s disease (Fernandez-Torres et al, 2014). This is the first reported case of subcutaneous histiocytoid Sweet’s syndrome in a paediatric patient.

4. In 2015, five cases of SS were documented:

  • In a 1-year-old boy with a 15 day history of fever, and a sore throat (Sharma et al, 2015).
  • In a child with systemic lupus erythematosus (Quinn et al, 2015).
  • A case of histiocytoid SS (Kim et al, 2015).
  • In a girl aged 1 year and 11 months who had a urine infection (Santos et al, 2015).
  • In a 1.5 month girl with a perineal infection associated with rectovestibular fistula (abnormal connection between the rectum and the vulval vestibule) (Shinozuka et al, 2015). In this case, the SS was initially mistaken for chickenpox.

A Sweet’s-like syndrome was also reported in a 5-week-old girl with HIDS (Pace et al, 2015).

5. In 2016, four cases of SS were documented:

  • In a 6-year-old child whose SS caused an aseptic splenic abscess (Johnson and Sadik, 2016) . The child was also demonstrating pathergy and an ulcerated skin lesion developed at the site of a splenic drainage tube after it had been removed.
  • In a 2-month-old girl demonstrating neurological problems – impaired awareness and eye fixation (Aoki et al, 2016). She was also diagnosed with encephalitis.
  • In a 15-year-old girl with acute myeloid leukaemia who had initially been misdiagnosed with severe cellulitis (Chen et al, 2016).
  • In an 11-year-old boy with myelodysplastic syndrome who had been previously diagnosed with immune thrombocytopenic purpura (Doinita et al, 2016). His SS went into remission, and he was then given a bone marrow transplant, but later died after developing graft-versus-host disease.

6. In August 2017, the first case of SS developing secondary to Mycoplasma infection in a child (Hsieh et al, 2017).


Key points about Sweet’s syndrome in children.

  • SS in children and adolescents is very rare – only 5% to 8% of cases. The average age of a child with SS is 5 years, but the youngest to be affected was 10 days old. In children under the age of 3 years it is more common in boys, but over the age of 3 years affects girls and boys equally (Doinita et al, 2016; Sharma et al, 2015).
  • In children, signs and symptoms of a respiratory infection normally appear 1 to 3 weeks before skin lesions develop (Santos et al, 2015).
  • SS that occurs in children under the age of 6 weeks normally suggests serious underlying illness (Gray et al, 2012).
  • SS in children over the age of 3 to 6 months tends to be less severe and outcomes are better.
  • Most cases of childhood SS are not associated with underlying malignancy, i.e. cancer. When cancer does occur it tends to be a type of blood cancer, e.g. acute myeloid leukaemia, and in children over the age of 3 years (Sharma et al, 2015).
  • Unlike most autoinflammatory conditions, SS is not a genetic condition, and a genetic cause for SS, i.e. SS as a symptom of a genetic condition, is very rare. By 2013, only 6 cases of SS with a possible genetic cause had been reported in children under the age of 6 months (Gray et al, 2012; Payne et al, 2013). As already mentioned, in 2015, a Sweet’s-like syndrome was also reported in a 5-week-old girl with HIDS (Pace et al, 2015).

Additional note: The Gray et al article (see ‘References’) discusses genetic causes for SS in children under 6 months of age. In 1989, two brothers developed SS secondary to Majeed syndrome, but I am unaware of their ages (Majeed et al, 1989). However, symptoms of Majeed syndrome appear no later than 2 years of age – Michelle Holder, Sweet’s syndrome UK.

  • SS is not hereditary, and is extremely rare in families. In 2003, two brothers were reported to have developed Sweet’s syndrome at ages 10 days and 15 days (Parsapour et al, 2003). Hereditary SS was considered but has not been proven.
  • In children, SS rarely affects areas other than the skin, e.g. internal joints and organs (Gray et al, 2012).
  • Children with SS often demonstrate pathergy.
  • Children are more likely to develop atypical SS skin lesions than adults. Atypical lesions are lesions that occur in a less common form.
  • Children are more likely to experience permanent skin changes than adults (30% of cases). Skin changes include scarring, colour changes (darkening or redness) to the areas of skin that have been affected by skin lesions, and occasionally, cutis laxa (loosely hanging skin that lacks any elasticity).

Diagnosis in children and teenagers, and additional diagnostic recommendations in early infancy.

1. Diagnosis.

How is Sweet’s syndrome diagnosed? Find out here. 

2. Additional diagnostic recommendations in early infancy.

Additional diagnostic recommendations in early infancy include:

  • Haematological investigations (Gray et al , 2012).
  • A broad immunodeficiency screen, including neutrophil function and antibody testing.
  • Performing an extensive viral screen which could possibly include HIV testing if there is multisystem involvement.
  • As SS has been triggered by perineal infection associated with rectovestibular fistula, it has been recommended that the perineal region should be screened for changes following SS diagnosis in infants (Shinozuka et al, 2015).

Underlying conditions to be considered include:

  • Malignancy.
  • Neonatal lupus erythematosus. On average, neonatal lupus erythematosus appears at 6 weeks of age, but should not be discounted outside of the immediate neonatal period (Gray et al, 2012).
  • CANDLE syndrome, particularly if there is multisystem involvement (Gray et al, 2012). Violaceous ring-shaped lesions can be present in both SS and CANDLE syndrome (Torrelo, 2017).
  • Other, e.g. primary immunodeficiency, HIDS, and Majeed syndrome (see ‘A list of documented cases of Sweet’s syndrome in children’).

Also see Britton and Stratman in ‘Further information’.


How is Sweet’s syndrome treated in children and teenagers?

Corticosteroid (steroid) therapy is usually the most effective form of treatment for SS in children and teenagers, but sometimes it is necessary to try other medications (Boatman et al, 1994; Gray et al, 2012). These may be given alongside steroids or by themselves.

Other medications include:

  • Immunoglobulin and dapsone were used to successfully manage symptoms in a child with SS and primary immunodeficiency (Haliasos et al, 2005).
  • Mycophenolate mofetil (Gray et al, 2012).
  • Anakinra has been used to successfully treat HIDS presenting as SS (Payne et al, 2013: 118, 122).
  • Tacrolimus ointment alongside oral ciclosporin (Johnson and Sadik, 2016).
  • Ciclosporin, dapsone, indomethacin suppository, and later, oral indomethacin in a boy with myelodysplastic syndrome (Doinita et al, 2016)
  • Metronidazole has been recommended to treat underlying Crohn’s disease (Fernandez-Torres et al, 2014).

Support for parents of children with Sweet’s syndrome or other autoinflammatory conditions.

Autoinflammatory Alliance. This is a US-based non-profit organization (NPO) that provides advice and support to those with a wide range of autoinflammatory conditions, both inside and outside of the US. As most autoinflammatory conditions develop during childhood, this NPO is particularly useful for parents of children with these conditions.

Rare Autoinflammatory Conditions Community UK (RACC-UK).


Further information.

A wheat or gluten-free diet is not a treatment for Sweet’s syndrome.

Assari, R., Ziaee, V., Parvaneh, N. and Moradinejad, M. (2014) Periodic Fever and Neutrophilic Dermatosis: Is It Sweet’s Syndrome? Case Reports in Immunlogy, Dec (online). 

Autoinflammatory Alliance (2015) Autoinflammatory Disease Comparison Chart (online). Accessed 12/02/17.

Autoinflammatory Alliance (2012) Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE) Syndrome (online).

Britton, K. and Stratman, E. (2017) Decision Support in Medicine: Sweet Syndrome. Cleveland Clinic Journal of Medicine (online). Accessed 12/02/17.

Genetics Home Reference (2017) Majeed Syndrome. NIH: US National Library of Medicine (online). Originally published Aug 2009, and reviewed Feb 7th 2017.

Ngan, V. (2006) Cutis Laxa. DermNet NZ (online). Accessed 12/02/17.

Tellez, J. (2015) He Has What? In SAID Support Blog. Autoflammatory Alliance; Jan 8th (online). Accessed 12/02/17.

The Britton and Stratman article provides information on paediatric Sweet’s syndrome, e.g. findings, causes, investigations and diagnosis, treatment and adverse side-effects e.t.c.


References.

Akilov, O., Desai, N., Jaffe, R. and Gehris, R. (2014) Bullous Sweet’s Syndrome After Granulocyte Colony-Stimulating Factor Therapy in a Child with Congenital Neutropenia. Pediatric Dermatology, Mar;31(2):e61-2 (PubMed).

Aoki, T., Yamashita, Y., Minamitani. K., Ota, S., and Hayakawa. K. (2016) Erythematous Plaques in a Child with Sweet Syndrome. The Journal of Pediatrics, Jun 15 (PubMed).

Boatman, B., Taylor, R., Klein, L. and Cohen, B. (1994) Sweet’s Syndrome in Children. Southern Medical Journal, Feb;87(2):193-6 (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, Aug 5th (online).

Doinita, S., Simina-Maria, V., Bianca, D., Sabina, Z. and Cristian, S. (2016) A Case of Sweet’s Syndrome Secondary to Myelodysplastic Syndrome – Diagnostic and Treatment Challenges. Maedica, Jun;11(2):154-157 (online).

El-Shanti, H. and Ferguson, P. (2014) Majeed Syndrome. Gene Reviews [Internet].

Fernandez-Torres, R., Castro, S., Moreno, A., Alvarez, R. and Fonseca, E. (2014) Subcutaneous histiocytoid Sweet syndrome associated with Crohn’s disease in an adolescent. Case Reports in Dermatological Medicine, Mar 26th (online).

Gray, P., Bock, V., Ziegler, D. and Wargon, O. (2012) Neonatal Sweet syndrome: a potential marker of serious systemic illness. Pediatrics, May;129(5):1353-9 (AAP).

Haliasos, E., Soder, B., Rubenstein, D., Henderson, W. and Morrell, D. (2005) Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin. Pediatric Dermatology, Nov-Dec;22(6):530-5 (PubMed).

Hsieh, J., Yalcindag, A. and Coghlin, D. (2017) A Sweet Case of Mycoplasma. Pediatrics, Aug 18 (PubMed).

Johnson, K. and Sadik, K. (2016) Aseptic Splenic Abscess and Sweet Syndrome. The Journal of the American Osteopathic Association, May; 116:330 (online).

Kim, J., Seo, J. and Oh, S. (2015) Unusual presentation of histiocytoid Sweet’s syndrome in a pediatric patient. International Journal of Dermatology, Sept 4 (PubMed).

Majeed, H., Kalaawi, M., Mohanty, D., Teebi, A., Tunjekar, M., al-Gharbawy, F.,  Majeed, S. and al-Gazzar, A. (1989) Congenital dyserythropoietic anemia and chronic recurrent multifocal osteomyelitis in three related childrenand the association with Sweet syndrome in two siblings. The Journal of Pediatrics, Nov;115(5 Pt 1):730-4 (online).

Pace, S., Bingham, J. and Royer, M. (2015) Histopathologic features in a case of hyperimmunoglobulinemia D syndrome. Indian Dermatology Online Journal, Dec;6 (Suppl 1):S33-6.

Parsapour, K.,  Reep, M., Gohar, K., Shah, V., Church, A. and Shwayder, T. (2003) Familial Sweet’s syndrome in 2 brothers, both seen in the first 2 weeks of life. Journal of the American Academy of Dermatology; 49:132-138 (PubMed).

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 (online). Available as free PDF.

Quinn, N., MacMahon, J., Irvine, A. and Lowry, C. (2015) Sweet syndrome revealing systemic lupus erythematosus. Irish Medical Journal, Feb;108(2):59-60 (PubMed).

Santos, T., Sales, B., Sigres, M., Rosman, F. and Cerqueira, A. (2015) Sweet Syndrome in Childhood. Anais Brasileiros de Dermatologia, Aug;90(4):567-9 (online).

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).

Shinozuka, J., Tomiyama, H., Tanaka, S., Tahara, J., Awaguni, H., Makino, S., Maruyama, R. and Imashuku, S. (2015) Neonatal Sweet’s Syndrome Associated with Rectovestibular Fistula with Normal Anus. Pediatric Reports, Jun 24;7(2):5858 (online).

Torrelo, A. (2017) CANDLE Syndrome As a Paradigm of Proteasome-Related Autoinflammation. Frontiers in Immunology, Aug 9 (PMC).

Wang, T., Liu, H., Wang, L., Guo, Z. and  Li, L. (2014) An Unusual Case of Sweet Syndrome in a Child: Overlapping Presentation With Erythema Elevatum Diutinum. The American Journal of Dermatopathology, Feb (PubMed).

© 2012-2017 Sweet’s Syndrome UK

Sweet’s syndrome associated with antiphospholipid antibody syndrome

Sweet’s syndrome (SS) can develop secondary to autoimmune conditions. This is a rare case of SS developing in association with antiphospholipid antibody syndrome (APS) (Alves et al, 2017).

Case-study.

A 55-year-old man developed skin lesions, fever, joint pain in hands, and the inflammatory eye condition, episcleritis. All of these symptoms were consistent with SS.

Blood tests revealed increased levels of inflammation in the body, and a biopsy of a lesion showed SS. The patient also had a stroke, cavernous sinus thrombosis (blood clot in the hollow spaces located under the brain, and behind each eye socket), and was positive for antiphospholipid antibodies. He was then given a diagnosis of SS-associated APS. Unfortunately, the patient did not respond well to steroids, the most common treatment for SS. The medications methotrexate and hydroxychloroquine were started but stopped, due to bone marrow suppression. Treatment with the biologic, infliximab, led to significant improvement.

Are there any other cases of SS developing in association with APS?

Yes. An earlier case of SS-associated APS was reported in a man with multiple pulmonary emboli (blockages in the artery that carries blood from the heart to the lungs) (Cohen, 2007). He was given the steroid, prednisone, which initially worked well, but experienced SS flare-ups when the dosage was reduced. In 2008, Sweet-like lesions were reported in a 37-year-old man with insufficient blood flow to the brain and APS (Tomb et al, 2008).

What is APS?

APS, or Hughes syndrome, is an autoimmune condition where the immune system produces abnormal antibodies called antiphospholipid antibodies (NHS Choices, 2015). These antibodies then target proteins attached to fat molecules (phospholipids), making the blood more likely to clot.

What are the symptoms of APS?

Symptoms of APS include high blood pressure; deep vein thrombosis; stroke; heart attack; pulmonary embolism. The syndrome can also cause other symptoms, which are sometimes mistaken for the symptoms of multiple sclerosis – balance and mobility problems; vision problems; speech and memory problems; tingling or pins and needles; fatigue; repeat headaches or migraines.

Further information.

What causes Sweet’s syndrome? Includes information on SS triggers.

References.

Alves, L., Castro, F., Sousa, T., Alverenga, C., Abreau, I., Padova, P., Costa, G. and Souza, E. (2017) Sweet’s syndrome associated with antiphospholipid antibody syndrome – case report. Brazilian Journal of Rheumatology, 57(suppl 1): 371 (Science Direct). Article in Portuguese. Use translate.

Cohen, P. (2007) Clinical description: Figure 4, in Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases; 2: 34 (PMC).

NHS Choices (2015) Antiphospholipid syndrome (APS) (online). Last reviewed on 9/11/15. Accessed on 3/09/17.

Tomb, R., Maalouf, E. and Attoui, S. (2008) [Cutaneous nodular lesions and antiphospholipid syndrome]. Annales de dermatologie et de vénéréologie, Jun-Jul;135(6-7):484-7 (EM|Consulte). Full-text in French.

© 2012-2017 Sweet’s Syndrome UK

Fournier’s Gangrene: A Rare Complication of Sweet’s Syndrome

This is the first reported case of Fournier’s gangrene (FG) as a rare complication of Sweet’s syndrome (SS) (Choi et al, 2017).

Case-study.

A 31-year-old woman was admitted to hospital with a 7 day history of itchy, red skin lesions of various sizes all over her body. She also had a fever, and blood tests showed a raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), indicating inflammation in the body. A procalcitonin test was given to determine whether or not she had developed sepsis – a rare, life-threatening complication of infection, that can occur when chemicals that the immune system releases into the bloodstream to fight infection end up causing inflammation throughout the entire body. In this case, the procalcitonin test result was normal. A skin biopsy of a lesion showed white blood cells called neutrophils in the tissues and damage to the vessels caused by neutrophils releasing noxious substances. The former was indicative of SS. The patient was treated with high dose steroids for 5 days, which she responded well to.

Three days after being discharged, the patient returned to hospital, having developed a painful 2 cm-sized lesion and swelling on her buttock. The drainage from this area had the odour of rotten fish and looked like greenish pus. An X-ray of the area did not show anything unusual, but computed tomography (CT) scan showed that gas had entered the gluteus maximus muscle layer. This is because infection can result in gas in the soft tissues. Bacterial culture showed the presence of several bacteria: Streptococcus anginosus, Pseudomonas, and Clostridium. The patient was eventually diagnosed with FG, and treated with wound debridement (surgical removal of dead tissue) and antibiotics. Two months later, the buttock lesion was completely healed.

What is Fournier’s gangrene?

FG is a rapidly progressive necrotizing fasciitis (NF) that affects the perineal, genital and perianal regions. NF is a rare and serious bacterial infection that affects the superficial fascia – a layer of connective tissue that lies beneath the skin and between the muscles and organs in the body. FG usually develops secondary to other perirectal or periurethral infections, and the main form of treatment is wound debridement and broad-spectrum intravenous antibiotics (Bracho-Riquelme, 2017; Choi et al, 2017).

Some additional & key points.

  • FG most commonly affects those with a weakened immune system (Choi et al, 2017). This includes those with a weakened immune system as a result of taking medications that suppress the immune system (immunosuppressants), e.g. the steroid, prednisone.
  • FG can be confused with the conditions erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, or cellulitis.
  • CT scan is an important diagnostic tool in FG, and can detect the presence of soft tissue air.
  • Early aggressive wound debridement and antibiotics are vital in the treatment of FG.
  • Doctors should consider the possibility of FG when a SS patient who takes immunosuppressants, develops painful lesions on the perianal and perineal area (Ibid).
  • FG or NF should not be confused with the rare SS variant, necrotizing Sweet’s syndrome (NSS). This variant mimics NF, and the main form of treatment is high-dose steroids. Antibiotics do not work in the treatment of NSS, and wound debridement must be avoided as it can lead to the development of new lesions.

References.

Bracho-Riquelme, R. (2017) Fournier Gangrene. NORD: National Organization for Rare Disorders (online). Rodolfo L. Bracho-Riquelme, MD, is a general and colorectal surgeon at Hospital General de Durango, Mexico. Accessed 6/06/17.

Choi, H., Kim, Y., Na, C. and Shin, B. (2017) Fournier’s Gangrene: A Rare Complication of Sweet’s Syndrome. Annals of Dermatology, Jun;29(3):387-389 (PubMed).

© 2012-2017 Sweet’s Syndrome UK

Celebrate Sweet’s Syndrome UK Day on June 2nd!

June 2nd 2017 is Sweet’s Syndrome UK Day & the 5th anniversary of Sweet’s Syndrome UK.

What can you do to help spread awareness?

  1. Like our Facebook page, and share one of our posts.
  2. Join our HealthUnlocked forum. This is a free online community that’s available in English, Spanish, and Portuguese.
  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. Share 5 key facts or 10 myths about Sweet’s syndrome.
  8. 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.
  9. Make a donation to Skin Care Cymru. This a Welsh charity that gives a voice to those with skin conditions in Wales.

BEE sweet and buzz for Sweet’s – help us spread the word!

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Yes, this is my own work!

Image: ‘I’m a resurcher’, Contrived Platitudes.

There has been ongoing confusion over whether or not the information on the Sweet’s Syndrome UK blog is my own work. The answer is ‘Yes, this is my own work!’ 😲

Most of the Sweet’s syndrome information I provide has not simply been copied and pasted, reblogged or reshared, as certain individuals have insinuated or claimed. Occasionally, I do share information from other blogs or websites, but I make it clear when the work is not my own.

If you would like to access free full-text medical case-studies and articles about Sweet’s syndrome, most of which have been referenced in my blog posts, click here.

Thank you,

Michelle Holder. 💛 💛

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Can vaccination trigger Sweet’s syndrome?

Updated 4/09/17.

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 very 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 11 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.
  • Hepatitis B (Enokawa et al, 2017). One case in a 69-year-old man with the autoimmune condition, systemic lupus erythematosus. Symptoms of Sweet’s syndrome started to develop 48 hours after vaccination, and there were no lesions at the vaccination site.
  • 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 flu) vaccination.
  • Smallpox (Carpentier et al, 2002: 82; Cruz-Velasquez et al, 2016). Two cases reported in 1975, occurring 3 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.

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 may be doing so for one of the following reasons: they have no real understanding of vaccination or Sweet’s syndrome; they are trying to scare you; they are trying to promote their own agenda, e.g. anti-vax or financial; they are trying to sell you something, e.g. ‘detox’ products that will supposedly cleanse your body of vaccine ‘toxins’, and thereby, cure your Sweet’s syndrome.

Why do vaccinations trigger Sweet’s syndrome?

Vaccination can trigger Sweet’s syndrome because of hypersensitivity reaction.

What is hypersensitivity reaction in Sweet’s syndrome?

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 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).

Antigens are 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. The presence of antigens associated with certain health conditions, medications and vaccinations can potentially trigger Sweet’s syndrome by stimulating the innate immune system to produce chemical messengers called cytokines, which eventually leads to the activation of white blood cells called neutrophils (Gosheger et al, 2002: 70). The neutrophils migrate to skin tissues and sometimes other tissues, causing skin lesions or other 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 very rare, but if it does happen then symptoms usually develop within hours or days, 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, most commonly, 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.
  • Immunodeficiency.
  • 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 (online). Article in Spanish, use translate.

Enokawa, M., Giovanella, L., Zardo, B., Cunha, J., Rachid Filho, A., Zeni, L., Bisognin, M., Rosseto, C. and Guimaraes, A. (2017) Sweet’s Syndrome Discharged (Caused) by Hepatitis B Vaccine. Brazilian Journal of Rheumatology, 57(suppl 1):S197 (Science Direct). Article in Portuguese, 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.