SWEET SYNDROME

Sweet syndrome is a rare inflammatory skin condition characterized by the sudden onset of fever and a painful rash that can appear on the arms, legs, trunk, face, or neck. It is also referred to as acute febrile neutrophilic dermatosis.

Experiencing a fever for several days followed by the sudden onset of a rapidly spreading, painful rash can be concerning and may indicate various health conditions, including one less commonly known: Sweet syndrome.

Sweet syndrome is a rare skin condition that typically resolves on its own without treatment. However, it can be triggered by underlying health issues such as infections or, in some cases, cancer. It’s important to note that Sweet syndrome is not contagious, meaning it cannot be transmitted from person to person.

Prevalence of Sweet Syndrome

Acute febrile neutrophilic dermatosis is considered a rare medical condition, with only several hundred cases reported to date.

Causes Sweet Syndrome

Acute febrile neutrophilic dermatosis can occur spontaneously without an identifiable cause in most cases. However, in some instances, it may be associated with various underlying conditions or triggers, including:

Blood Cancer: Sweet syndrome may manifest in individuals with blood cancers such as leukemia or lymphoma.
Bowel Diseases: Conditions like ulcerative colitis or Crohn’s disease can be linked to the development of acute febrile neutrophilic dermatosis.
Respiratory Infections: Infections affecting the chest, such as strep throat, have been associated with acute febrile neutrophilic dermatosis.
Cancer: Certain types of cancers, including colon or breast cancer, may trigger acute febrile neutrophilic dermatosis.
Skin Injury: acute febrile neutrophilic dermatosis. may develop at the site of skin injuries, such as needle pricks or insect bites.
Pregnancy: Sweet syndrome can occur during pregnancy, although it is relatively rare.
Rheumatoid Arthritis: Individuals with rheumatoid arthritis may be at risk of developing acute febrile neutrophilic dermatosis.
Drug Reactions: Sweet syndrome can be triggered by certain medications. Common drugs like nonsteroidal anti-inflammatory drugs (e.g., Advil, Motrin), and antibiotics like Bactrim may induce acute febrile neutrophilic dermatosis. However, the most common culprit is granulocyte-colony stimulating factor, a medication used to boost immune function in some cancer patients.

Symptoms of Sweet Syndrome

The primary indicator of acute febrile neutrophilic dermatosis is the sudden appearance of a rash, often occurring a few days to weeks following a fever.

Rash Characteristics: The rash typically presents as small red or purple bumps or lumps, initially appearing on the arms, legs, face, or neck, although they can emerge in other areas as well. These lesions tend to proliferate rapidly, eventually merging to form larger patches on the skin.
Rash Presentation: The rash can be accompanied by pain, and individuals may develop blisters or pimple-like bumps, which have the potential to rupture and become infected.

Other Associated Symptoms:

In addition to the rash and fever, individuals with acute febrile neutrophilic dermatosis may experience a range of additional symptoms, including:

Flu-like Symptoms: Feeling unwell, akin to having the flu, characterized by generalized malaise and fatigue.
Headaches: Individuals may experience headaches of varying intensity.
Joint Pain: Joint pain or discomfort may be present, contributing to overall discomfort.
Mouth Sores: Some individuals may develop sores or lesions within the mouth.
Pink Eye: Conjunctivitis, or pink eye, may occur concurrently with the rash and other symptoms.

Risk Factors

Sweet syndrome can affect individuals of any age or gender, but it is most commonly observed in women aged between 30 and 50 years.

Diagnosis

Diagnosing acute febrile neutrophilic dermatosis typically involves a combination of clinical evaluation and diagnostic tests to confirm the condition and rule out other possible causes. Diagnostic procedures may include:

Visual Examination: Your doctor may be able to identify Sweet syndrome based on the characteristic appearance of the rash. However, additional tests are usually conducted to confirm the diagnosis and determine the underlying cause.
Skin Biopsy: A small sample of the rash is collected and examined under a microscope to look for specific features consistent with Sweet syndrome.
Blood Tests: Blood samples may be analyzed to assess levels of white blood cells, particularly neutrophils, and to check for any abnormalities indicative of underlying blood disorders or systemic inflammation.
Imaging Tests: In cases where an underlying condition is suspected, your doctor may recommend imaging studies such as X-rays or computed tomography (CT) scans. These imaging tests provide detailed images of internal organs and structures to help identify any potential contributing factors or complications associated with acute febrile neutrophilic dermatosis.

Treatment for Sweet Syndrome

Sweet syndrome may resolve spontaneously without treatment if it is not associated with an underlying health condition, although this process can take several weeks or even months.

Corticosteroids: Corticosteroid pills are commonly prescribed to alleviate symptoms such as redness, itching, swelling, and allergic reactions associated with Sweet syndrome. Additionally, steroid creams or gels may be recommended, particularly for smaller lesions, to provide relief from pain and inflammation. In cases of severe pain or swelling, your doctor may administer steroid injections directly into the affected areas.
Addressing Underlying Conditions: If Sweet syndrome is secondary to an underlying health problem such as cancer or a drug reaction, treating the underlying cause is essential for resolving the skin manifestations. Discontinuing the medication responsible for triggering acute febrile neutrophilic dermatosis often leads to the resolution of the rash.
Follow-Up and Monitoring: After treatment, most cases of acute febrile neutrophilic dermatosis heal without leaving scars, unless there are open sores present. However, skin discoloration may persist for several months following resolution of the rash. It’s important to note that acute febrile neutrophilic dermatosis may recur, especially if cancer is the underlying cause. Therefore, regular follow-up appointments with your doctor are necessary for monitoring and early detection of any recurrence or associated health issues.

Post-Treatment Expectations:

Following treatment, most individuals experience resolution of the rash and skin lesions associated with Sweet syndrome without scarring. However, skin discoloration may persist for several months after symptom disappearance.

Complications of Sweet Syndrome

In some instances, Sweet syndrome may lead to complications involving other organ systems in the body, including the nervous and gastrointestinal systems. Eye problems are among the most frequent complications associated with acute febrile neutrophilic dermatosis. These may encompass:

Eye inflammation
Increased eye pressure (glaucoma)
Corneal ulceration is characterized by the development of sores on the cornea.

Prevention

As the exact cause of Sweet syndrome remains unknown, preventive measures are not currently available.

The prognosis for Sweet Syndrome

For the majority of individuals, symptoms of Sweet syndrome either resolve spontaneously or with appropriate medical intervention. The duration of symptoms can vary, lasting for weeks or months. Prolonged symptom duration may occur if Sweet syndrome is secondary to an underlying medical condition or medication.

Living With Sweet Syndrome

Contact your doctor if you experience symptoms of acute febrile neutrophilic dermatosis or if your symptoms worsen. Seeking prompt medical attention can facilitate timely diagnosis and appropriate management of the condition.

Sources

Clevland Clinic: “Sweet’s Syndrome.”
Multiple Myeloma Research Foundation: “What are colony-stimulating factors?”
British Association of Dermatologists: “Sweet’s Syndrome (Acute Febrile Neutrophilic Dermatosis).”
WebMD: “Sweet’s Syndrome.”
Mayo Clinic: “Sweet’s Syndrome.”
National Organization for Rare Disorders: “Sweet Syndrome.”
National Institutes of Health; Genetic and Rare Diseases Information Center: “Acute Febrile Neutrophilic Dermatosis.”