Currently being updated 9/12/18.
What are the symptoms of Sweet’s syndrome?
The most common symptoms.
The main symptom of Sweet’s syndrome is skin lesions that often appear as a sore or painful red or purple rash. In patients who have developed Sweet’s syndrome secondary to cancer, the lesions are more likely to appear in pustular (containing pus) or bullous form (bluish or grey abscess-like lumps and blisters that can sometimes ulcerate) (Bhat et al, 2015: 527). On rare occasions, there are no skin lesions (Sweet’s Syndrome UK, 2015a & b). Patients commonly develop other symptoms too. These symptoms include:
- Unexplained fever: normally 38 degrees centigrade or above.
- Fatigue: a medical term that includes a number of different symptoms. It isn’t the same as ordinary tiredness, and occurs as a result of the inflammation in the body that Sweet’s syndrome causes. Therefore, the things that normally help when you’re tired, e.g. getting some sleep, eating more healthily, or getting some fresh air, may help a little bit, but will not resolve the fatigue completely. Symptoms of fatigue can include tiredness or exhaustion, the kind that you can never seem to ‘shake-off’ and can be debilitating; ‘fogginess’, mild dizziness, poor concentration or memory, otherwise known as ‘brain-fog’; flu-like symptoms; limb heaviness which can sometimes make every movement seem like a real effort.
- Headache, and less commonly, migraine.
- Joint pain (arthralgia) or joint pain and swelling (arthritis).
- Muscle aches and pain.
- Eye problems, e.g. conjunctivitis, episcleritis, or iridocyclitis.
- Mouth ulcers (aphthous-like ulcers), but this is a symptom more commonly associated with a similar condition called ‘Behcet’s syndrome’.
Less common and rare symptoms.
Most people will only develop some of the symptoms listed above, but Sweet’s syndrome can potentially affect any organ of the body. When this does happen, the most commonly affected organs are the bones, eyes, central nervous system, kidneys, intestine, liver and heart (Li et al, 2015: 339).
A list of less common and rare symptoms of Sweet’s syndrome include:
- Neuro-Sweet’s disease. This is a rare neurological variant of Sweet’s syndrome and can cause a number of different symptoms, e.g. meningitis and encephalitis.
- Idiopathic hypertrophic cranial pachymeningitis (Cohen, 2007).
- Guillain-Barre syndrome (Cohen, 2007).
- Bilateral sensorineural hearing loss and polyneuropathy (Cala et al, 2015; Cohen, 2007). Symptoms of the latter may include numbness or pins and needles in extremities such as hands and feet. Read more here.
Ears, eyes, mouth & throat.
- Bilateral sensorineural hearing loss. Symptoms can include dizziness and tinnitus. See ‘Neurological’.
- Redness, tenderness and swelling in the external auditory canal (Saliba et al, 2004).
- Lesions in the external auditory canal (nodules, pustules and plaques).
- Lesions affecting the tympanic membrane, otherwise known as the eardrum (nodules, pustules and plaques) (Ibid).
- Redness, tenderness and swelling of the external ear alongside lesions (papules on outer superior pinna) (Berg et al, 2013).
- Ear pain, or referred ear pain (Contrucci and Martin, 2015). ‘Referred pain’ means that a problem exists somewhere else in the body other than where you feel the pain.
- More serious or sight-threatening eye problems, including peripheral ulcerative keratitis and retinal vasculitis. Click on the ‘Eye problems’ link at the top for further information.
Mouth & throat.
- Cracks or fissures on the corners of the mouth (Contrucci and Martin, 2015).
- Lesions on the inside of the lips (haemorrhagic bullae and vesicles, and necrotic nodules) (Cohen, 2007).
- Lesions on the gums (haemorrhagic bullae and vesicles).
- Necrotizing ulcerative periodontitis.
- Enlargement of the gums (gingival hyperplasia) (Ibid).
- Lesions on the tongue (aphthous-like ulcers, ulcers, and macerated papules) (Cohen, 2007; Kasirye et al, 2011: 135) .
- Tongue pain, and swollen or enlarged tongue in association with lesions (Cohen, 2007; Contrucci and Martin, 2015; Kasirye et al, 2011: 135). A swollen tongue can also be a sign of severe allergic reaction. If you develop a swollen tongue, please seek medical attention immediately!
- Lesions on the roof of the mouth (macerated papules, individual and grouped pustules, ulcers, and bullae) (Cohen, 2007; Contrucci and Martin, 2015).
- Lesions affecting the pharynx (individual and grouped pustules) (Cohen, 2007).
- Lesions on the inside of the cheeks (aphthous-like ulcers, and ulcers) (Cohen, 2007).
- Inflammation of the saliva glands in the cheeks (parotitis) and associated cheek swelling (Jo et al, 2012).
- Throat pain, painful swallowing, and hoarseness (Contrucci and Martin, 2015).
- On very rare occasions, autoimmune thyroiditis could possibly be a symptom of Sweet’s syndrome, but there’s insufficient evidence to prove a definite connection between the two conditions (Ratten et al, 2016). If a patient does have autoimmune thyroiditis that’s caused by Sweet’s syndrome, then both conditions should start to settle with steroid treatment.
Lungs & heart.
Lungs (9/12/18) – symptoms can range from mild to severe. Sweet’s syndrome with lung involvement is commonly misdiagnosed as bacterial pneumonia (Yang et al, 2015). Skin lesions frequently appear before lung symptoms, but not always (Vettakkara et al, 2018; Yang et al, 2015).
- Cough, shortness of breath, low levels of oxygen in blood, pulmonary rales – crackling sound when breathing (Li et al, 2015; Yang et al, 2015).
- Pleural effusion – a collection of fluid next to the lungs (Cohen, 2007). In 2018, systemic inflammatory response syndrome (SIRS) and pleural effusion were reported in a 35-year-old male patient with Sweet’s syndrome (Vettakkara et al, 2018). Chest X-ray showed bilateral pleural effusion, and aspiration (sample of fluid from around lungs) showed lots of white blood cells called neutrophils, but no signs of infection.
- Acute respiratory distress syndrome and SIRS in malignancy-associated Sweet’s syndrome (Shugarman et al, 2011).
- Blocked upper airway and respiratory failure (Cohen, 2007; Yang et al, 2015).
Information for doctors.
Radiological changes & histopathology.
Radiological changes of pulmonary Sweet’s syndrome include the presence of nodular, reticular or patchy infiltration, with or without effusion (Li et al, 2015: 342). Histopathologically, pulmonary Sweet’s syndrome is characterized by interstitial inflammation, oedema and mild fibrosis, in which a large number of neutrophils and occasional lymphocytes, macrophages and eosinophils infiltrate the alveoli.
- Chest pain (Li et al, 2015: 340, 342).
- Aortitis (segmental) (Cohen, 2007; Li et al, 2015: 342).
- Aortic stenosis (neutrophilic and segmental).
- Enlargement of the heart (cardiomegaly).
- Myocardial neutrophilic infiltration.
- Coronary artery occlusion (Ibid).
- Blood clot in the heart (ball thrombus in the left ventricle) (Tatebe et al, 2003).
- Vascular dilatation (aorta, bracheocephalic trunk and coronary arteries) (Cohen, 2007).
- Heart failure (Cohen, 2007).
Liver & kidneys.
- Abnormal liver function tests (Cohen, 2007).
- Enlarged liver (hepatomegaly) (Cohen, 2007; Liaw, 2017).
- Hepatic portal triad with neutrophilic inflammation (Cohen, 2007).
- Protein in urine (most common finding) (Cohen, 2007; Kaballo, 2016).
- Blood in urine (Cohen, 2007; Kaballo, 2016; Macarini et al, 2016).
- Membranoproliferative glomerulonephritis, also known as mesangiocapillary glomerulonephritis (Cohen, 2007; Kaballo, 2016).
Spleen & lymph nodes.
- Enlarged spleen (splenomegaly) (Cohen, 2007; Liaw, 2017; Macarini et al, 2016).
- Mutiple lesions in the spleen of a women who had been previously infected with parvovirus B19 (Fortna et al, 2010). Radiographic evidence and splenectomy showed diffuse neutrophil-predominant infiltrate with formation of numerous abscesses. Other cases: aseptic abscess in the spleen of a child, the drained fluid from the abscess showing neutrophilic infiltration (Johnson and Sadik, 2016). Aseptic splenic abscesses in an 84-year-old man with multiple myeloma who had been taking trimethoprim/sulfamethoxazole for a urinary tract infection (Sutton et al, 2018).
- Widespread lymphadenopathy developing alongside lesions in the spleen (Fortna et al, 2010). Lymphadenopathy can cause swollen or enlarged lymph nodes or glands. A paratracheal lymph node biopsy showed complete effacement of nodal architecture by a mixed inflammatory and fibrotic process including neutrophils, with features reminiscent of cat-scratch disease.
Bone, soft & connective tissue.
- Inflammation of bone (focal aseptic osteitis) (Cohen, 2007).
- Inflammation and overgrowth of the lining of a joint (pigmented villonodular synovitis).
- Chronic recurrent multifocal osteomyelitis (CRMO) (Ibid).
Sweet’s syndrome affecting the joints can be mistaken for joint infection or periprosthetic joint infection (Lee et al, 2017).
Soft & connective tissue.
- Myositis (a rare condition that causes inflammation of the muscles) and neutrophilic myositis (Cohen, 2007). In regards to the latter, muscle biopsy shows a dense neutrophilic infiltrate.
- Inflammation of the fascia (fasciitis). The fascia is the connective tissue surrounding muscles, blood vessels and nerves.
- Inflammation of a tendon (tendonitis/tendinitis).
- Inflammation of the fluid-filled sheath that surrounds a tendon (tenosynovitis) (Ibid).
- Diarrhoea (Ainechi and Carlson, 2016).
- Inflammation of the intestine (extensive and diffuse neutrophilic inflammation) (Cohen, 2007).
- Sweet’s syndrome affecting the end of the small intestine (neutrophilic ileal infiltrate).
- Pancolitis. This is a severe form of ulcerative colitis (Ibid).
Most of the time, Sweet’s syndrome doesn’t directly cause GI symptoms. More commonly, GI symptoms in Sweet’s syndrome are caused by an underlying condition such as infection or inflammatory bowel disease (IBD), e.g. Crohn’s disease or ulcerative colitis. Both infection and IBD are known triggers for Sweet’s syndrome.
- Genital ulceration (Hisanaga et al, 1999; Jo et al, 2015).
- Sterile peritonitis – symptoms include abdominal pain, left groin pain, large amount of pus in the pelvis and abdomen, and pelvic adhesions (Rajjoub et al, 2017). Can be misdiagnosed as pelvic inflammatory disease.
- Systemic inflammatory response syndrome (SIRS) (Shugarman et al, 2011; Vettakkara et al, 2018). SIRS can rapidly progress to multiorgan dysfunction, and systemic steroids should be started as soon as possible.
If you experience any of the symptoms listed above, they may or may not be associated with Sweet’s syndrome. Please seek advice from your doctor.
Ngan, V. (2002) Behcet Disease. DermNet NZ (online). Accessed 6/04/2018.
Ainechi, S. and Carlson, J. (2016) Neutrophilic Dermatosis Limited to Lipo-Lymphedematous Skin in a Morbidly Obese Woman on Dasatinib Therapy. The American Journal of Dermatopathology, Feb;38(2):e22-6 (PubMed).
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Contrucci, R. and Martin, D. (2015) Sweet syndrome: A case report and review of the literature. ENT Journal, July;94(7):282-284 (online). Sign-up to the ENT Journal for free to access the full article.
Fortna, R., Toporcer, M., Elder, D. and Junkins-Hopkins, J. (2010) A case of sweet syndrome with spleen and lymph node involvement preceded by parvovirus B19 infection, and a review of the literature on extracutaneous sweet syndrome. The American Journal of Dermatopathology, Aug;32(6):621-7 (PubMed).
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Li, B., Ma, Z., Xu, X., Yin, J., Wang, X., Ren, J., Wang, S., Yang, J., Ma, T., Zhang, Q., Yu, J. and Yan, B. (2015) Multi-organ involvement of Sweet’s syndrome: a case report and literature review. Internal Medicine (Tokyo, Japan);54(3):339-43 (PDF).
Marcarini, R., Araujo, R., Nóbrega, M., Medeiros, K., Gripp, A. and Maceira, J. (2016) Histiocytoid Sweet’s syndrome presenting with annular erythematous plaques. Anais Brasileiros de Dermatologia, Sep-Oct;91(5 suppl 1):154-156 (PMC).
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).
Rattan, R., Sharma, A., Shanker, V., Tegta, G. and Verma, G. (2016) Transient autoimmune thyroiditis associated with Sweet’s syndrome. Indian Journal of Dermatology, Venereology and Leprology, Jan-Feb;82(1):112 (online).
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Shugarman, I., Schmit, J., Sbicca, J. and Wirk, B. (2011) Easily Missed Extracutaneous Manifestation of Malignancy-Associated Sweet’s Syndrome: Systemic Inflammatory Response Syndrome. American Society of Clinical Oncology, Aug 20; 29(24) (online).
Tatebe, S., Iwaki, H., Kuraoka, S. and Iijima, S. (2003) A ball thrombus in the left ventricle in association with Sweet syndrome. The Journal of Thoracic and Cardiovascular Surgery, Jan;125(1):209-11 (PubMed).
Taylor, G., Borgnakke, W., Anderson, P. and Shannon, C. (2009) Oral and General Health – Exploring the Connection. Oral Health of Non-Head & Neck Cancer Patients: Professional Version. Delta Dental Plans Association & University of Michigan, Research Review Dec 2010:1-38 (PDF).
Vettakkara, K.M., Banerjee, S., Mittal, A., Goel, P., Kumar, P., Baitha, U., Jorwal, P., Soneja,M. and Biswas, A. (2018) Not so sweet; severe Sweet’s syndrome presenting as SIRS and pleural effusion. Journal of Family Medicine and Primary Care, Nov;7:1584-7 (online).
Yang, M., Zhang, J., He, Z., Deng, J., Bai, J., Zhong, X. and Liu, G. (2015) [Lung involvement of 3 cases of idiopathic sweet syndrome and literature review]. Chinese Journal of Tuberculosis and Respiratory Diseases, Aug;38(8):589-94 (PubMed).
2012-present, Sweet’s Syndrome UK