Sweet's syndrome

Also known as acute febrile neutrophilic dermatosis, or Gomm-Button disease.

Sweet's Syndrome which happened in 1 on 9 million is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis.

The syndrome was first described in 1964 by Dr Robert Douglas Sweet. It was also known as Gomm-Button disease in honour of the first two patients Dr Sweet diagnosed with the condition.

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.

Signs and symptoms of Sweet's syndrome may include:

·                                 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.

·                                 Moderate to high fever preceding the skin lesions

·                                 Pink eye (conjunctivitis) or sore eyes

·                                 Tiredness

·                                 Aching joints and headache

·                                 Mouth ulcers.

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:

1-     Classical Sweet's syndrome (CSS) usually presents in women between the age of 30 to 50 years, it is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. Approximately one-third of patients with CSS experience recurrence of the dermatosis.

2-      The malignancy-associated Sweet's syndrome (MASS) can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet's syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered; MASS is most commonly related to acute myelogenous leukemia. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's cancer. Hence, MASS can be the cutaneous harbinger of either an undiagnosed visceral malignancy in a previously cancer-free individual or an unsuspected cancer recurrence in an oncology patient.

3-      Drug-induced Sweet's syndrome (DISS) most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, Other medications associated with DISS  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 factots:

-          Being a woman

-          Age between 30 – 50, 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 URTI, 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

-          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.

The pathogenesis of Sweet's syndrome may be multifactorial and still remains to be definitively established. Clinical and laboratory evidence suggests that cytokines have an etiologic role. Systemic corticosteroids are the therapeutic gold standard for Sweet's syndrome. After initiation of treatment with systemic corticosteroids, there is a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions. Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions. Other first-line oral systemic agents are potassium iodide and colchicine. Second-line oral systemic agents include indomethacin, clofazimine, cyclosporine, and dapsone. The symptoms and lesions of Sweet's syndrome may resolved spontaneously, without any therapeutic intervention; however, recurrence may follow either spontaneous remission or therapy-induced clinical resolution

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

Did you ever had sweet's syndrome before ?? please leave comment to let other patients know that they are not alone.

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