What are the symptoms of Sweet’s syndrome?

Updated 21/05/2017.

What are the symptoms of Sweet’s syndrome?

The most common symptoms.

The main symptom of Sweet’s syndrome is skin lesions (Sweet’s 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. Slightly less likely to occur in patients who develop Sweet’s syndrome secondary to cancer and blood disorders.
  • Fatigue: a medical term that includes a number of different symptoms. It is not 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 are 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; poor concentration or memory (‘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 (myalgia).
  • Eye problems, e.g. conjunctivitis, episcleritis, or iridocyclitis.
  • Occasionally mouth ulcers (aphthous-like ulcers), but this is a symptom more commonly associated with the similar condition 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:

Neurological.

  • Neuro-Sweet’s disease. This is a rare neurological variant of Sweet’s syndrome and can cause a number of different symptoms, e.g. headaches, meningitis, encephalitis, psychiatric symptoms, and other.
  • Guillain-Barre syndrome (Cohen, 2007).
  • Idiopathic hypertrophic cranial pachymeningitis.
  • Bilateral sensorineural hearing loss (Cala et al, 2015). Read case-study in layman’s terms here.
  • Polyneuropathy (Cala er al, 2015; Cohen, 2007). Symptoms may include numbness or pins and needles in extremities such as hands and feet. Also, in the Cala et al case-study, the polyneuropathy occurred alongside bilateral sensorineural hearing loss. See above.

Ears, eyes, mouth & throat.

Ears.

  • Bilateral sensorineural hearing loss. 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.

Eyes.

  • More serious or sight-threatening eye problems, including peripheral ulcerative keratitis and retinal vasculitis. Click on the ‘eye’ link for further information and references.

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).
  • 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).

Thyroid.

  • On very rare occasions, autoimmune thyroiditis could possibly be a symptom of Sweet’s syndrome, but there is insufficient evidence to prove a definite connection between the two conditions (Ratten et al, 2016). If a patient does have autoimmune thyroiditis that is caused by Sweet’s syndrome, then both conditions should start to settle with steroid treatment.

Lungs & heart.

Lungs (very rare).

  • Cough (Li et al, 2015; Yang et al, 2015).
  • Shortness of breath or difficulty breathing (Li et al, 2015; Sesti et al, 2017; Yang et al, 2015).
  • Pulmonary rales. This is a crackling sound when breathing (Yang et al, 2015).
  • Low levels of oxygen in the blood (Yang et al, 2015).
  • Pleural effusion. This is a collection of fluid next to the lungs (Cohen, 2007).
  • Acute respiratory distress 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.

Please note that lung problems directly caused by Sweet’s syndrome are often misdiagnosed as bacterial pneumonia (Yang et al, 2015). This can lead to a patient being prescribed and unsuccessfully treated with antibiotics instead of steroid medication, the main form of treatment for Sweet’s syndrome.

Heart.

  • 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 infiltration by neutrophils.
  • 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.

Liver & kidneys.

Liver.

  • Abnormal liver function tests (Cohen, 2007).
  • Enlarged liver (hepatomegaly) (Cohen, 2007; Liaw, 2017).
  • Hepatic portal triad with neutrophilic inflammation (Cohen, 2007).

Kidneys.

  • 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.

Spleen.

  • 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. Also, an asceptic abscess in the spleen of a child (Johnson and Sadik, 2016). Drained fluid from the abscess showed neutrophilic infiltration.

Lymph nodes.

  • 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.

Bone.

  • 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).

Soft & connective tissue.

  • Inflammation of the muscles (myositis) (Cohen, 2007).
  • Neutrophilic myositis (muscle biopsy showing dense neutrophilic infiltrate).
  • Inflammation of the fascia (fasciitis). The fascia is the connective tissue surrounding muscles, blood vessels and nerves.
  • Inflammation of a tendon (tendinitis).
  • Inflammation of the fluid-filled sheath that surrounds a tendon (tenosynovitis) (Ibid).

Gastrointestinal.

  • 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).

Gastrointestinal symptoms directly caused by Sweet’s syndrome are rare. More commonly, gastrointestinal symptoms in Sweet’s syndrome patients are caused by infection or inflammatory bowel disease (IBD), e.g. Crohn’s disease or ulcerative colitis. As both infection and IBD are known causes for Sweet’s syndrome, they can then potentially trigger the Sweet’s syndrome.

Genital.

  • Genital ulceration (Hisanaga et al, 1999; Jo et al, 2015).

Other.

  • Systemic inflammatory response syndrome (in a patient with malignancy-associated Sweet’s syndrome) (Shugarman et al, 2011).

If you experience some of the symptoms listed above, they may or may not be associated with Sweet’s syndrome. Please seek advice from your doctor.

References.

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).

Berg, I., Nenninger, T., Dimaio, D., Bierman, P. and Sandkovsky, U. (2013) Rash and ear swelling in a patient with febrile neutropenia. International Journal of Infectious Diseases, May;17(5):e360-1 (online).

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).

Cala, C. , Kole, L. and Sami, N. (2015) Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet’s Syndrome. Case Reports in Otolaryngology, Dec 6th (Hindawi).

Cohen, P. (2007) Extracutaneous Manifestations: Table 4. In Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis, Orphanet Journal of Rare Diseases (online).

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).

Hisanaga, K., Hosokawa, M., Sato, N., Mochizuki, H., Itoyama, Y. and Iwasaki, Y. (1999) “Neuro-Sweet Disease”: Benign Recurrent Encephalitis With Neutrophilic Dermatosis. Archives of Neurology;56(8):1010-1013 (online).

Jo, M., Lim, Y., Shin, H., Choe, J., Seul, J. and Jang T. (2012) A Case Report of Sweet’s Syndrome with Parotitis. Archives of Plastic Surgery, Jan;39(1):59-62 (online).

Jo, T., Horio, K. and Migita, K. (2015) Sweet‘s syndrome in patients with MDS and MEFV mutations. The New England Journal of Medicine;372(7):686-8 (online).

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

Kaballo, M. (2016) Sweet’s Syndrome and the Kidney. Renal Fellow Network, Apr 18 (online).

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).

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).

Liaw, T. (2017) A case of Sweet syndrome successfully treated by anti-inflammatory property of doxycycline. Journal of the Formosan Medical Association, Jan 17 (online).

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 (online).

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).

Saliba, W., Goldstein, L., Habib, G. and Elias, M. (2004) Sweet’s syndrome affecting the external auditory canal and tympanic membrane. The Journal of Laryngology and Otology, Jan;118(1):48-9 (PubMed).

Sesti, A., Rappersberger, K. and Posch, C. (2017) [Bullous Sweet’s syndrome with pulmonary involvement]. Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete, Mar 16 (PubMed). Full article in German.

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). 

Sweet’s Syndrome UK (2015a) Can Sweet’s syndrome affect the eyes? (online).

Sweet’s Syndrome UK (2015b) Neuro-Sweet’s disease: a neurological variant (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).

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).

Further information.

Ngan, V. (2002) Behcet Disease. DermNet NZ (online). Accessed 6/02/2017.

Sweet’s syndrome in children.

What is Sweet’s syndrome?

© 2012-2017 Sweet’s Syndrome UK

8 thoughts on “What are the symptoms of Sweet’s syndrome?

    • This the first post I have seen that addresses the ears. I was glad to see that, I had to have a graft to my ear drum. The Ear, Nose, and Throat doctor would not confirm that the ear drum was damaged by Sweet’s (which he had never heard of before). However based on the pictures taken, I believe Sweet’s was the cause. The ear drum membrane looked alot like the pictures on another website of a Sweet’s issue on the eye.

      Like

      • Hi Ron, I’m glad that you’ve found the information useful. Unfortunately, SS symptoms other than skin lesions are often not recognised by doctors due to a lack of understanding. I hope that everything’s okay now, and that your SS has settled down.

        Like

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