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).
- Chronic recurrent multifocal osteomyelitis (CRMO).
- Acute myeloid leukaemia.
- Acute lymphoblastic leukaemia.
- Juvenile chronic myelomonocytic (myeloid or 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’s 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.
- In 25% of cases, SS in children is associated with cancer (Britton and Stratman, 2013). 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 hasn’t been proven.
- In children, SS rarely affects areas other than the skin, e.g. internal joints and organs (Gray et al, 2012).
- In 20% of cases, children with SS demonstrate pathergy (Britton and Stratman, 2013).
- 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, particularly in early infancy.
How is Sweet’s syndrome diagnosed? Find out here.
In children, blood tests show a raised white cell count (95%), anaemia (95%), raised neutrophil count (45%), raised platelet count (50%), and elevated erythrocyte sedimentation rate (95%) (Britton and Stratman, 2013).
2. Additional diagnostic recommendations, particularly in early infancy.
Additional diagnostic recommendations include:
- Further haematological investigation to rule out underlying illness, including cancer (Gray et al, 2012). If the full blood count or FBC (CBC in some countries) with differential shows abnormalities, then peripheral smear blood test should be evaluated (Britton and Stratman, 2013). If the peripheral smear shows abnormalities, then a bone marrow biopsy is needed.
- A broad immunodeficiency screen, including neutrophil function and antibody testing (Gray et al, 2012). Britton and Stratman note that SS ‘precedes all diagnoses of immunodeficiency’ (2013).
- An extensive viral screen which could possibly include HIV testing if there’s multisystem involvement (Gray et al, 2012).
- X-ray or MRI if bone or joint pain are present (Britton and Stratman, 2013). Arthritis and CRMO occur in 20% of childhood cases.
- ECG and echocardiogram are recommended if there’s cardiac involvement (Britton and Stratman, 2013). Cardiovascular involvement increases mortality to 40% .
- Screening perineal region. As SS has been triggered by perineal infection associated with rectovestibular fistula, it’s been recommended that the perineal region should be screened for changes following SS diagnosis in infants (Shinozuka et al, 2015).
Other underlying conditions to be considered include:
- Neonatal lupus erythematosus. On average, neonatal lupus erythematosus appears at 6 weeks of age, but shouldn’t be discounted outside of the immediate neonatal period (Gray et al, 2012).
- CANDLE syndrome, particularly if there’s multisystem involvement, and violaceous ring-shaped lesions can be present in both SS and CANDLE (Gray et al, 2012; Torrelo, 2017).
- Also, see ‘A list of documented 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’s 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.
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.
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).
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).
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).
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).
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).
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.
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).
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).
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