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Sweet syndrome
By Mayo Clinic staffMayo Clinic Health Manager
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Definition
Sweet syndrome — also known as acute febrile neutrophilic dermatosis — is a skin condition marked by fever and painful skin lesions that appear mainly on your face, neck, back and arms. Although middle-aged women are most likely to develop Sweet syndrome, the condition can also affect men, older adults and even infants.
The exact cause of Sweet syndrome often isn't known. In some people, it's triggered by an infection, illness or certain medications. Sweet syndrome can also occur with some types of cancer and other serious health problems.
Most often, Sweet syndrome isn't serious and will clear on its own in a few months. Healing is much more rapid with treatment.
Symptoms
The most obvious signs of Sweet syndrome are distinctive skin lesions that usually develop according to this pattern:
- A series of small red bumps appear suddenly on your back, neck, arms and face, often after a fever or upper respiratory infection.
- The bumps grow quickly in size, spreading into clusters called plaques that may be a centimeter in diameter or larger.
- The eruptions are tender or painful and may develop blisters, pustules or even ulcers.
- Lesions may persist for weeks to months and then disappear on their own, without medication. With medical treatment, you're likely to be free of skin lesions in just a few days.
Other signs and symptoms of Sweet syndrome may include:
- Moderate to high fever
- Pink eye (conjunctivitis) or sore eyes
- Tiredness
- Aching joints and headache
- Mouth ulcers
When to seek medical advice
Sweet syndrome is rare. When it occurs, it often develops after an upper respiratory tract infection. If you develop a red rash that quickly grows in size soon after a bout with strep throat or another upper respiratory infection, see your doctor for appropriate treatment.
Although the rash may eventually disappear without treatment, the right medication can make the rash go away in just a few days. Sweet syndrome may also be a reaction to a more serious condition, such as an infection or leukemia.
Causes
In many cases, the cause of Sweet syndrome is impossible to determine (idiopathic). But sometimes, it can be a sign of an immune system response to one of the following:
- An upper respiratory tract infection, such as a chest infection or strep throat
- Blood disorders, especially acute myelogenous leukemia, a cancer of the blood and bone marrow
- Inflammatory bowel disease, such as ulcerative colitis or Crohn's disease
- Bowel or breast cancer
- Pregnancy
- Rheumatoid arthritis
- An injury at the site where the rash appears, such as an insect bite or needle prick
- Certain medications, including nonsteroidal anti-inflammatory drugs (NSAIDs)
Risk factors
Sweet syndrome is uncommon, but certain factors increase your risk, including:
- Your sex. Women are far more likely to have Sweet syndrome than men are.
- Age. Though older adults and even infants can develop Sweet syndrome, the condition mainly affects women between the ages of 30 and 50.
- Other health problems. Sweet syndrome often follows an upper respiratory infection, and many people report having flu-like symptoms before the rash appears. Sweet syndrome can also be associated with a number of other illnesses, including inflammatory bowel disease, certain systemic infections and cancer.
- Pregnancy. Some pregnant women develop Sweet syndrome during their first or second trimester. In these cases, the condition usually clears without treatment.
- Medical history. Sweet syndrome tends to recur. About one-third of people who have had Sweet syndrome once get it again.
Complications
Although it can be extremely uncomfortable, Sweet syndrome often isn't serious. But in some cases, it can be a warning sign of another health problem. Sweet syndrome sometimes develops very early in the course of cancer, for instance. It may also occur in conjunction with:
- Inflammatory bowel disease
- Infections of the upper respiratory tract, including strep throat, pneumonia and tonsillitis
- Urinary tract infections
- Infections of the liver and gastrointestinal tract
- Certain systemic infections
Preparing for your appointment
Your family doctor or general practitioner is likely to refer you to a dermatologist for diagnosis and treatment of Sweet syndrome. If tests reveal an underlying condition, you'll also be referred to the appropriate specialist, such as a gastroenterologist or oncologist.
Because appointments can be brief and there's often a lot of ground to cover, it can help to be well prepared. Here are some tips to help you get ready for your appointment and what to expect from your doctor.
What you can do
- Write down all your signs and symptoms — even those that seem unrelated to your rash. Sweet syndrome can be a sign of several illnesses, so it's important that your doctor know all of your symptoms. Include key personal information, such as major stresses or recent life changes.
- Bring a list of all medications, including vitamins, herbs and over-the-counter drugs that you're taking. Even better, bring the original bottles and a written list of the dosages and directions.
- If possible, bring along a family member or friend. It can be difficult to absorb all the information provided to you during an appointment. The person who accompanies you may remember something that you forgot or missed.
- Write down questions that you want to ask your doctor. Don't be afraid to ask questions or to speak up when you don't understand something your doctor says. Start with the problems that concern you most. If you run out of time, ask to speak with a nurse or physician's assistant or leave a message for your doctor.
If you have symptoms of Sweet syndrome, questions you may want to ask include:
- What might be causing my rash?
- What tests do I need to confirm the diagnosis?
- Is this condition temporary or chronic?
- What is the best course of action?
- What are the alternatives to the primary treatment approach that you're suggesting?
- I don't like the idea of taking steroids. Are there other medications you can prescribe?
- Is there a generic alternative to the medicine you're prescribing me?
- What if I just wait to see if my signs go away on their own?
- Do you have any literature on this condition? Is there a Web site you can recommend where I can learn more?
What to expect from your doctor
Your doctor is likely to ask you a number of questions, such as:
- When did your symptoms first start?
- Did they come on suddenly or gradually?
- What did the rash look like when it first started?
- Is the rash painful?
- What, if anything, makes it better?
- What, if anything, makes it worse?
- Were you sick before the rash started?
- Do you have other symptoms that started about the same time?
- What medications do you take?
Tests and diagnosis
Your dermatologist can usually diagnose Sweet syndrome simply by looking at the lesions. But you're likely to have certain tests to rule out conditions that have similar symptoms and to search for the underlying cause. These tests include:
Blood tests. A small sample of your blood may be sent to a laboratory where it's checked for an unusually large number of white blood cells and certain blood disorders, both of which are common in Sweet syndrome.
For the test, a small amount of blood is removed from a vein in your arm. Risks of the procedure are almost nonexistent, and you can return to your ordinary activities right away.
- Tissue sample. Your doctor may remove a small piece of affected tissue (biopsy) for examination under a microscope. The tissue is analyzed to determine whether it has the characteristic abnormalities of Sweet syndrome. The area where the sample is taken is numbed, and a small piece of skin is removed with an instrument that looks like a small cookie cutter — a procedure called a punch biopsy. You're not likely to need stitches, and the incision should heal without scarring.
Treatments and drugs
Left untreated, Sweet syndrome not associated with a more serious condition may disappear on its own within one to three months. Medications can improve skin lesions and associated symptoms in just two or three days, with the worst of the lesions disappearing within one to four weeks.
With or without treatment, the lesions rarely leave a mark or scar when they eventually disappear. Your doctor may advise continuing treatment because recurrence of the condition is common.
Medications
Doctors usually prescribe systemic corticosteroids (prednisone or prednisolone) to treat Sweet syndrome. These oral anti-inflammatory medications reduce redness, itching, swelling and allergic reactions. But long-term use of corticosteroids can cause serious side effects, including an increased risk of hard-to-treat infections, osteoporosis, eye problems and diabetes. Topical corticosteroids may be used to provide immediate relief of swelling.
Lifestyle and home remedies
If you have Sweet syndrome, it's important to treat your skin gently. These steps can help:
- Avoid injury to your skin. Wear protective clothing if you think you might injure or damage your skin.
- Apply sunscreen with a sun protection factor (SPF) of 15 or greater before you head outdoors.
Prevention
There is no known way to prevent Sweet syndrome. The most difficult part of dealing the condition is that it commonly comes back. Be certain to follow your doctor's recommendations for preventing recurrences.
