Sweet syndrome


Sweet syndrome

Description, Causes and Risk Factors:

Alternative Name: Acute febrile neutrophilic dermatosis.

Abbreviation: SS.

Sweet's syndrome is a relatively rare and worldwide disease with no racial predilection. Although it may be seen at any age, but usually occurs in women at 4th or 5th decades of life. The disease usually starts with high grade and persistent fever accompanied by severe ill condition. Later, skin lesions in the form of nodules or erythematous plaques (irregular and very sensitive), will appear on the forearm and neck. The lesions progress either into a wound or are filled with vesicles and pustules after 2-4 weeks. Sometimes wound heals centrally without leaving scar, but may progress to the surrounding area.

The exact cause of Sweet's syndrome is not known. In some people, it is triggered by an infection, illness or certain medications. Sweet's syndrome can also occur with some types of cancer.

Types:

    Idiopathic (classical): In most cases, the cause of Sweet's syndrome is not 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 is also been associated with pregnancy and inflammatory bowel disease.

  • Malignancy-associated: In about 20 percent of cases, Sweet's syndrome is associated with malignancy (cancer), 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 is 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:

    Being a woman.

  • Being between 30 and 50 years of age.

  • Having other health problems.

  • Being pregnant.

  • A previous history of the condition

The duration of disease varies from one to eight months. The best medication is systemic corticosteroids which treat the symptoms promptly. The fever and the general illness improve after a few days while the lesions disappear within one or two weeks. Potassium iodine, clofazimine, colchicine, indomethacin, and retinoids have also proved to be useful.

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.

Other signs and symptoms of Sweet's syndrome may include:

    Moderate-to-high fever preceding the skin lesions.

  • Conjunctivitis or sore eyes

  • Tiredness.

  • Aching joints.

  • Headache.

  • Mouth ulcers.

Clinical presentation:

    Fever (50%).

  • Myalgia/Arthralgia/Arthritis (62%).

  • Eye involvement (38%).

  • Oral aphthous ulcers (13%).

Diagnosis:

Differential diagnoses may include mainly erythemamultiforme, erythema elevatum diutinum, erythemanodosum, and pyoderma gangrenosum.

Your dermatologist can usually diagnose Sweet's syndrome simply by looking at the lesions. But you are likely to have certain tests to rule out conditions that may include:

    Blood tests: A small sample of your blood may be sent to a laboratory where it is 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 are not likely to need stitches, and the incision should heal without scarring.

Lab signs:

    Moderate leukocytosis.

  • Elevated ESR (erythrocyte sedimentation rate).

  • Slightly elevated ALP (alkaline phosphatase).

  • ANCA (antineutrophil cytoplasmic antibodies) positive in some cases.

Treatment:

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.

Systemic corticosteroids are generally very effective in treating Sweet's syndrome. You typically take these oral antiinflammatory 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 antiinflammatory properties. Follow your doctor's instructions exactly when taking these medications and be sure your doctor knows about any other medications you are taking, to avoid harmful drug interactions.

Risk and benefits must be discussed with the doctor.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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