Sweet’s Syndrome in Children

Updated 18/05/17.

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 (not autoimmune) 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 (a 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, and sometimes mouth ulcers. On rare occasions, the lesions can develop in internal joints and organs causing more serious symptoms. Very rarely, SS may be life-threatening. The cause of SS is poorly understood, but it is associated with infections, autoimmune conditions, inflammatory bowel disease, cancers and blood disorders (malignancy-associated or paraneoplastic), medications (drug-induced), pregnancy, skin damage, overexposure to sunlight or ultraviolet (UV) light, and vaccination. In regards to the latter, SS caused 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 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.

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 June 2016, at least 81 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 was in a 6-week-old girl was documented in literature (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.

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.
  • 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.


Further information.

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). This is a medical case-study and not patient information.

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. This is medical not patient information.

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).

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 (online).

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).

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). 

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).

If your doctor is not familiar with Sweet’s syndrome, the Gray et al medical article provides an excellent overview of Sweet’s syndrome in children.

© 2012-2017 Sweet’s Syndrome UK

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2 thoughts on “Sweet’s Syndrome in Children

  1. Tousseau, J. (2014) How Inflammation Affects Mood and Behavior in Systemic Autoinflammatory Diseases. SAID Support, Feb (online).

    ‘Many questions have been asked about behavior issues, and if there is a connection with autoinflammatory syndromes, especially with younger kids who can get moody and even aggressive in a flare and have a hard time explaining their emotions. There is research on how inflammation causes “sickness behavior syndrome.” While the research is not specific to autoinflammatory illnesses, the inflammatory cytokines studied are the same ones that are involved in the various autoinflammatory diseases…

    With autoinflammatory conditions in particular, the sickness behavior can seem to make a person, especially young kids, appear moody and unpredictable. This especially true in the conditions that have distinct periods of illness, or flares, and then periods of health where the person has few if any symptoms of their disease. ‘

    Read more at: http://saidsupport.org/how-inflammation-affects-behavior-in-systemic-autoinflammatory-diseases/

    Like

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