Sweet syndrome or neutrophilic dermatosis is a rare inflammatory disorder where the patient develops acute onset of lesions (sores or blisters) on the skin (neutrophilic lesions) associated with fever, joint pains, and headache. Sweet syndrome is idiopathic but could be caused by medications, cancer, certain infections, or illness. While it may resolve on its own without any intervention, medication can speed up the process.
Diagnosis of Sweet syndrome is difficult because the symptoms presented are commonly seen in other disorders. The diagnosis is done based on medical history and blood work.
There is no definitive cure for Sweet syndrome. Symptomatic treatment with corticosteroids and other medications are done but the symptoms may recur. However, a patient treated with granulocyte and monocyte adsorption apheresis (GMA) therapy one time a week for three consecutive weeks responded well, and skin lesions were resolved within the first week of the treatment. Blood work showed normal levels of neutrophil, leukocyte, and amyloid count and C-reactive protein levels in the serum within the second week. These are the parameters used to analyze the inflammatory processes in the body.
What are the symptoms of Sweet syndrome?
Sweet syndrome is mostly observed in women aged between 20 and 40 years old. However, it can affect males, women, and people of all ages, and it can occur in any ethnic population. This disorder is uncommon in children.
The symptoms of Sweet syndrome include:
- Skin rash appears a few days to weeks after the fever
- The rash is characterized by small red painful bumps that usually appear on the face, arms, legs, and back but may also be seen on other parts of the body.
- These bumps eventually grow into large blisters and form into patches of about two and a half inches on the skin.
- The skin rash may be painful, and the blisters or bumps formed may break open and get infected.
The patient is commonly presented to a doctor with skin rash characterized as painful blisters.
The other symptoms may include:
What are the risk factors of Sweet syndrome?
Sweet syndrome is usually idiopathic, and the exact cause may not be known. However, sometimes, it may be associated with other infections or illnesses in the body.
- Sweet syndrome has been linked to cancer, such as leukemia (most often), breast and colon cancer.
- Other myeloproliferative disorders include:
- Sweet syndrome can occur after an upper respiratory infection, and many patients complain of flu-like symptoms before the skin rash appears.
- Sweet syndrome is also seen after infections, such as:
- Sweet syndrome is linked to several inflammatory and autoimmune diseases, such as:
Drug-induced Sweet syndrome is a well-known condition. Medications that have been linked to its causation include:
- Granulocyte colony-stimulating factor: This is a cytokine that is most commonly associated with Sweet syndrome
- Anticancer drugs: Bortezomib, imatinib mesylate, ipilimumab, lenalidomide, topotecan and vemurafenib
- Antibiotics: Minocycline, nitrofurantoin, trimethoprim-sulfamethoxazole, norfloxacin, and ofloxacin
- Antihypertensives: Hydralazine and furosemide
- Nonsteroidal anti-inflammatory drugs: Diclofenac and celecoxib
- Immunosuppressives: Azathioprine
- Antiepileptics: Carbamazepine and diazepam
- Antipsychotics: Clozapine
- Antithyroid: Propylthiouracil
Sweet syndrome occurs after exposure as well as reexposure to the causative substance in these situations and resolves with or without corticosteroids following drug withdrawal.
- The development of Sweet syndrome is seen in two percent of pregnant women, and symptoms may resolve spontaneously after delivery without any severe complications to the mother or infant.
How is Sweet syndrome diagnosed?
The diagnosis of Sweet syndrome is not straightforward because its symptoms are similar to other diseases. The diagnosis is made by analyzing the medical history and conducting the following tests.
- Skin biopsy: A small sample of the skin is taken from the site of the rash and is examined under the microscope to identify Sweet syndrome.
- Blood workup: Blood samples are collected to determine the complete blood count to check for cytopenias, such as increased neutrophils, leukocytes, and other blood cells.
Other tests, such as computed tomography scans, biopsies, and pregnancy confirmation tests, may be done to confirm diseases that may cause Sweet syndrome.
What are the treatment options for Sweet syndrome?
Sweet syndrome sometimes resolves spontaneously without a need for medical intervention. If the symptoms are associated with other medical conditions, treating the cause relieves the symptoms. Drug-induced Sweet syndrome resolves with the withdrawal of the drug. However, severe symptoms, such as painful and infected blisters and high fever, are treated with corticosteroids and antibiotics.
Intralesional corticosteroid injections and topical corticosteroids can be used, particularly in localized Sweet syndrome. Several steroid-sparing medicines, including potassium iodide, colchicine, dapsone, isotretinoin, methotrexate, doxycycline, indomethacin, chlorambucil, and cyclosporine, have allegedly demonstrated effectiveness in cases of illness recurrence following corticosteroid tapering.
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Heath MS, Ortega-Loayza AG. Insights Into the Pathogenesis of Sweet's Syndrome. Front Immunol. 2019 Mar 12;10:414. https://www.frontiersin.org/articles/10.3389/fimmu.2019.00414/full
Vashisht P, Bansal P, Goyal A, et al. Sweet Syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021. https://www.ncbi.nlm.nih.gov/books/NBK431050/
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