Sweet's Syndrome

Sweet's syndrome — also known as acute febrile neutrophilic dermatosis — is a skin condition marked by fever and painful skin lesions that appear mainly on your arms, face and neck.
The exact cause of Sweet's syndrome isn't always known. In some people, it's triggered by an infection, illness or certain medications. Sweet's syndrome can also occur with some types of cancer.

Most often, Sweet's syndrome will clear on its own in a few months or once the underlying cause is resolved or eliminated. Healing is much more rapid with treatment.

Symptoms

The most obvious signs of Sweet's syndrome are distinctive skin lesions that usually develop according to this pattern: A series of small red bumps appear suddenly on your arms, neck, face or back, often after a fever or upper respiratory infection. The bumps grow quickly in size, spreading into clusters called plaques that may be up to an inch or so in diameter. 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's syndrome may include:
- Moderate to high fever preceding the skin lesions
- Pink eye (conjunctivitis) or sore eyes
- Tiredness
- Aching joints and headache
- Mouth ulcers

When to see a doctorSweet's 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's syndrome may also be a reaction to a more serious condition, such as leukemia, or to certain medications.

Causes
Although Sweet's syndrome may be associated with infections, the condition itself isn't infectious. Sweet's syndrome is typically divided into three categories, depending on its associations:
Idiopathic (classical). In most cases, the cause of Sweet's syndrome isn't known (idiopathic). Idiopathic Sweet's syndrome predominantly affects women between the ages of 30 and 50, and is often preceded by an upper respiratory or gastrointestinal infection. It's also been associated with pregnancy and inflammatory bowel disease.

Malignancy-associated. In about 20 percent of cases, Sweet's syndrome is associated with cancer (malignancy), most often acute leukemia. A few cases may be associated with a solid tumor, such as breast or colon cancer. Sweet's syndrome can occur as an early sign of a cancer, after diagnosis or as a sign of a recurrence. Fever is often present but skin lesions typically aren't preceded by an upper respiratory infection, as is the case with idiopathic Sweet's syndrome. Malignancy-associated Sweet's syndrome appears to affect men and women equally but among older adults, it's more likely to occur in women.

Drug-induced. Although uncommon, Sweet's syndrome may occur as a reaction to a medication, most commonly to granulocyte colony-stimulating factor, a hormone preparation designed to increase your white blood cell count. Other medications associated with Sweet's syndrome include certain antibiotics, oral contraceptives, diuretics and anti-epileptic drugs, among others. Once the offending drug is discontinued, Sweet's syndrome usually goes away.

Risk factors
Sweet's syndrome is uncommon, but certain factors increase your risk, including:
Being a woman. Women are far more likely to have idiopathic Sweet's syndrome than men are.
Being between 30 and 50 years of age. Though older adults and even infants can develop Sweet's syndrome, the condition mainly affects women between the ages of 30 and 50.
Having other health problems. Sweet's syndrome often follows an upper respiratory infection, and many people report having flu-like symptoms before the rash appears. Sweet's syndrome can also be associated with a number of other illnesses, including inflammatory bowel disease, certain systemic infections and cancer.

Being pregnant. Some pregnant women develop Sweet's syndrome during their first or second trimester. In these cases, the condition usually clears without treatment.
A previous history of the condition. Sweet's syndrome tends to recur. About one-third of people who have had Sweet's syndrome once get it again.

Complications
There is a risk of the skin lesions becoming infected. Follow your doctor's recommendations for caring for the affected skin. The most challenging aspect of Sweet's syndrome may be in dealing with recurrences, which occur in about a third of cases. Signs and symptoms may reappear, especially if treatment is tapered off too quickly. Be certain to follow your treatment plan exactly as your doctor recommends.

Preparing for your appointment:
Your family doctor or general practitioner is likely to refer you to a dermatologist for diagnosis and treatment of Sweet's 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's 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. Make a list of all medications, including vitamins, herbs and over-the-counter drugs, that you're taking. Even better, take the original bottles and a written list of the dosages and directions. If possible, take 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's 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 and symptoms go away on their own?
What to expect from your doctorYour doctor is likely to ask you a number of questions, such as:
When did your symptoms start?
Did they come on suddenly or gradually?
What did the rash look like when it first appeared?
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's 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.
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's 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's 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. This is true even for malignancy-associated Sweet's syndrome, although treatment or remission of the associated cancer will help, too.

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 Systemic corticosteroids (prednisone or prednisolone) are generally very effective in treating Sweet's syndrome. You typically take these oral anti-inflammatory medications for about four to six weeks. Topical corticosteroids may be used to provide immediate relief of swelling.

Other first line medications your doctor may use include potassium iodide therapy, which you take as an oral tablet or as drops, and colchicine, which has anti-inflammatory properties. Follow your doctor's instructions exactly when taking these medications and be sure your doctor knows about any other medications you're taking, to avoid harmful drug interactions.
Lifestyle and home remedies

If you have Sweet's syndrome, it's important to treat your skin gently. These steps can help reduce additional injury to the skin:
Avoid injury to your skin. Wear protective clothing if you think you might injure or damage your skin.

Apply sunscreen. Use sunscreen with a sun protection factor (SPF) of 15 or greater before you head outdoors.

References
Farhi D, et al. The neutrophilic dermatoses. Dermatology Nursing. 2008;20:274.
Moschella SL. Neutrophilic dermatoses. http://www.uptodate.com/home/index.html/. Accessed April 5, 2010.
Sweet's syndrome: A dermatologic condition associated with fever and frequently confused with an infectious process. In: Mandell GL, et al. Mandell, Douglas, and Bennet's Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa.: Churchill Livingstone Elsevier; 2010. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-0-443-06839-3..00052-7--s0060&uniq=193269557&isbn=978-0-443-06839-3&sid=978680989#lpState=open&lpTab=contentsTab&content=4-u1.0-B978-0-443-06839-3..00052-7--s0060%3Bfrom%3Dtoc%3Btype%3DbookPage%3Bisbn%3D978-0-443-06839-3. Accessed April 5, 2010.
Cohen PR. Sweet's syndrome — A comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases. 2007;2:34.
Franks AG Jr. Skin manifestations of internal disease. Medical Clinics of North America. 2009;93:1265.
Colchicine: Drugdex Evaluations. Micromedex Healthcare Series. http://www.micromedex.com. Accessed April 5, 2010.
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June 24, 2010
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Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect that causes cyanosis or blueness. The basic problem is that the blood flow coming back from the lungs (which is full of Oxygen) through pulmonary veins is diverted to the right atrium so that there is complete mixing of blood (red and Blue) within the heart. These abnormalities are divided into two major groups - obstructed and unobstructed. Unobstructed is the most common and this frequently involves the common pulmonary vein being connected by an abnormal vertical vein to the superior vena cava and emptying into the right atrium. There can also be a direct connection of the common pulmonary vein to the right atrium. Either way this causes complete mixing of the blood within the right atrium. These babies are usually a little blue at birth, but not necessarily very sick. Many times they will go home from the nursery and be found to be breathing fast by the pediatrician. This is because the mixing of blood causes the heart to work harder than normal. This is usually relatively easy to fix. It does involve open-heart surgery. Once fixed, the heart is for the most part normal.

Obstructive veins are another matter. These babies are usually very ill at birth. In this situation the blood flow leaving the lungs through the pulmonary veins is not only going to the wrong place but is at least partially blocked, this makes it harder for blood to enter the lungs. Most of the time these abnormal connections occur in the liver. They can occur in the upper part of the chest as well. When the veins are obstructed It also makes the pressure in the right ventricle higher than normal. When blood cannot easily enter the lungs the babies are very blue because not enough blood goes to the lungs and there is a lot of mixing of blood (blue blood with red blood) at the level of the PDA and Foramen Ovale (opening in wall between the upper chambers of the heart). Because not enough oxygen is being circulated in the body the baby becomes acidotic and the muscle tissue and kidneys, etc. do not work well. When the oxygen level is low, the arteries in the lungs tend to tighten up, making it harder for blood to be pumped through the lungs and thus the situation even worse. When the lungs are full of blood they become stiff, making it harder to breathe. Very frequently in this condition the actual veins draining back from the lungs are smaller than normal. This is because they never developed normally in the first place. This condition is lethal unless it can be fixed quickly after diagnosis.

Most of the time we are able to diagnose this condition by echocardiogram. One can see the abnormal flow patterns of blood coming from the lungs into the SVC or we can see abnormal patterns in the liver, which is where most of the obstructed veins return. We also look to see if we can see the normal pulmonary veins returning to the left atrium. We can measure oxygen levels in the right atrium with an umbilical catheter and this will be abnormally high.

TAPVR can closely mimic a condition called persistent fetal circulation. In this case the pulmonary veins are normal but very little blood flow is going to the lungs. This can sometimes occur with stressed or infected babies and can be very difficult to treat. The pressures in the lungs can be very high and little blood flow goes through the lungs. As with TAPVR this is a lot of mixing of blood within the heart and the oxygen level can be quite low. This condition can be treated with a ventilator, pulmonary vasodilators, nitric oxide and sometimes ECMO. It can be very difficult to distinguish these two conditions as they have many similar clinical features.

TAPVR is not something that can be treated medically. The longer the delay in surgery, the worse the outcome. This condition is frequently associated with other rare conditions such as dextrocardia, and ambiguous situs. In this situation a baby's internal organs can have either two right sides or two left sides.

The outcome for unobstructive TAPVR is usually quite good. The outcome of obstructive TAPVR can vary and ultimately depends on the overall size of the pulmonary veins. Many attempts have been made to enlarge these surgically and with balloons and stents but the results are seldom satisfactory.

If you have any questions, please ask one of the doctors.

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