Sweet’s Syndrome in Children

The information on this blog is currently being updated.

Updated 14/01/18.

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; muscle pain; joint pain or joint pain and swelling (arthritis); headaches or migraine; sore eyes or other eye problems; mouth ulcers.

Can Sweet’s syndrome affect children?

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

By Jan 2018, at least 85 cases of SS in children had been documented in medical literature.

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

Cases reported before 2013.

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.
  • Immunodeficiency secondary to HIV (human immunodeficiency virus) infection.
  • 3 children had probable CANDLE syndrome (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) – a rare genetic autoinflammatory condition (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; Gray et al, 2012; Majeed et al, 1989).

Cases reported 2013-2018.

Between 2013 and Jan 2018, at least 17 cases of SS were reported in children and teenagers (includes Sweet’s-like conditions).

  • 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.
  • A Sweet’s-like syndrome in a 5-week-old girl with HIDS (Pace et al, 2015).
  • 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).
  • A 5-year-old child with erythema elevatum diutinum (Wang et al, 2014).
  • A case of histiocytoid SS secondary to underlying disease (Kim et al, 2015).
  • A 14-year-old boy with Crohn’s disease (Fernandez-Torres et al, 2014). This is the first reported case of subcutaneous histiocytoid SS in a paediatric patient.
  • The first case of histiocytoid SS in a 10-year-old boy where there was no underlying systemic disease (Yeom et al, 2017).
  • A 1-year-old boy with a 15 day history of fever, and a sore throat (Sharma et al, 2015).
  • A girl aged 1 year and 11 months who had a urine infection (Santos et al, 2015).
  • A 1.5 month girl with a perineal infection associated with rectovestibular fistula (Shinozuka et al, 2015). In this case, the SS was initially mistaken for chickenpox.
  • The first case of SS in a child developing secondary to Mycoplasma infection (Hsieh et al, 2017).
  • A 6-year-old child with SS that caused an aseptic splenic abscess (Johnson and Sadik, 2016). The child also demonstrated pathergy and an ulcerated skin lesion developed at the site of a splenic drainage tube after it had been removed.
  • A 2-month-old girl demonstrating neurological problems – impaired awareness and eye fixation – which were possibly symptoms of SS (Aoki et al, 2016).
  • A 15-year-old girl with acute myeloid leukaemia who had initially been misdiagnosed with severe cellulitis (Chen et al, 2016).
  • 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.
  • A child with systemic lupus erythematosus (Quinn et al, 2015).
  • A 5-month-old boy who experienced multiple flares when his steroids were tapered (Tangtatco et al, 2018).

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 cancer. When cancer does occur it tends to be a type of blood cancer, and in children over the age of 3 years (Sharma et al, 2015).
  • Unlike most autoinflammatory conditions, SS is not a genetic condition. It can be a symptom of other autoinflammatory conditions which are genetic, but this is very rare (see Majeed, CANDLE and HIDS).
  • SS is not hereditary, and is very rare in families. In 2003, two brothers were reported to have developed SS 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 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 (see listed cases).

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

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 which 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).
  • Potassium iodide in SS resistant to treatment (Tangtatco et al, 2018).

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 12/01/18.

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

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 12/01/18.

Tellez, J. (2015) He Has What? In SAID Support Blog. Autoflammatory Alliance; Jan 8th (online). Accessed 12/01/18.


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).

Tangtatco, J., Ho, N., Drucker, A. and Forse, C. (2018) Potassium iodide in refractory, recurrent pediatric Sweet syndrome: Guidance in dosing and monitoring. Pediatric Dermatology, Jan 4 (PubMed).

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).

Yeom, S., Ko, H., Moon, J.,  Kang, M., Byun, J., Choi, G. and Shin, J. (2017) Histiocytoid Sweet Syndrome in a Child without Underlying Systemic Disease. Annals of Dermatology, Oct;29(5):626-629 (PMC online).

2012-2018 Sweet’s Syndrome UK

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Sterile peritonitis as a rare symptom of Sweet’s syndrome

Links checked 15/01/18.

This is the first reported case of sterile peritonitis, a type of peritonitis not caused by infection, as a symptom of Sweet’s syndrome (SS) (Rajjoub et al, 2017). Peritonitis is inflammation of the peritoneum, the thin layer of tissue that lines the inside of the abdomen.

Case-study.

A 37-year-old female patient who had previously been diagnosed with SS in 1998 had been having repeat episodes of peritonitis. On admission to hospital, she had a 48-hour history of right sided abdominal pain and feeling sick, high temperature, fast pulse rate, low blood pressure, and a raised white blood cell count, including neutrophil count. There was no vomiting, problems with bowel movements, urinary symptoms, or vaginal discharge, and the patient reported that she had never been sexually active. One month prior to admission, her SS had also flared up, which was treated with steroid medication.

Click here and here to learn more about the role of neutrophils in SS.

It was in 2000 that the patient had first started experiencing abdominal pain, alongside left groin pain and a high temperature. She was treated with antibiotics, and then had a laparoscopy, otherwise known as abdominal ‘keyhole’ surgery. This is a minimally invasive surgical procedure that allows a surgeon to access the inside of the abdomen, while avoiding large incisions. The laparoscopy showed a large amount of pus in the pelvis and abdomen associated with pelvic adhesions. These are fibrous bands between tissues and organs that occur as a result of inflammation or injury. Due to the complexity of the situation, the laparoscopy was changed to ‘open’ or traditional surgery so that the patient’s abdomen could be more thoroughly examined. Her appendix, although it appeared to be normal, was removed. A sample of the pus was sent for analysis, but showed no bacterial growth, i.e. no signs of infection. It was thought that the absence of infection was due to the patient being given antibiotics before surgery.

On a later admission, the patient had another laparoscopy for ongoing lower abdominal pain. Once again, there was a large amount of pus in the pelvis and abdomen, and a subphrenic abscess. This is a collection of pus between the diaphragm, liver, and spleen. There were also extensive adhesions, and as a result of this finding, the patient was started on antibiotics and initially diagnosed with pelvic inflammatory disease (PID) of unknown cause. PID is an infection of the female upper genital tract, including the womb, fallopian tubes and ovaries, and mostly affects sexually active women aged 15 to 24.

As the patient had reported never being sexually active and having a SS flare-up before admission, it was decided that the PID diagnosis was incorrect, and her peritonitis and pus in the pelvis and abdomen were probably SS-associated. Rajjoub et al have hypothesized that this is because neutrophils have the potential to infiltrate the peritoneal cavity causing a widespread reaction and development of pus and adhesions. It was also hypothesized that steroids may have initially worsened the existing problem, but not caused it, by increasing the movement of white blood cells into the peritoneum.

The patient was eventually discharged from hospital with a pelvic drain in place to drain away any further fluid or pus, and antibiotics to prevent infection.

Further information.

NHS Choices (2015) Pelvic Inflammatory Disease (online). Reviewed 3/09/15. Accessed 15/01/18.

NHS Choices (2015) Peritonitis (online). Reviewed 18/02/15. Accessed 15/01/18.

References.

Rajjoub, Y., Saffaf, N. and Goodman, A. (2017) A rare case report describing the relation between sweet syndrome and spontaneous recurrent peritonitis. International Journal of Surgery Case Reports, Jul 21;39:93-97 (PMC).

2012-2018 Sweet’s Syndrome UK

Sweet’s syndrome associated with antiphospholipid antibody syndrome

Links checked 16/01/18.

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 16/01/18.

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.

2012-2018 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

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. 💛

2012-2018 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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