Summary about Disease
Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare inflammatory condition characterized by the sudden appearance of painful, red or purplish bumps or plaques on the skin, often accompanied by fever, and an elevated white blood cell count (neutrophilia). It can occur in association with various underlying conditions, including infections, cancers, and inflammatory diseases, or can be drug-induced or idiopathic (cause unknown).
Symptoms
Skin lesions: Painful, tender, red or purplish papules (small bumps) and plaques (larger raised areas) that often appear suddenly and can enlarge and merge together. They are typically found on the upper body, arms, legs, and face, but can appear anywhere.
Fever: Elevated body temperature, often above 100.4°F (38°C).
Neutrophilia: An abnormally high number of neutrophils (a type of white blood cell) in the blood.
Other possible symptoms: Fatigue, malaise (a general feeling of discomfort or illness), joint pain (arthralgia), muscle pain (myalgia), headache, eye inflammation (conjunctivitis, episcleritis, uveitis), and, less commonly, involvement of internal organs.
Causes
The exact cause of Sweet syndrome is often unknown (idiopathic). However, it can be associated with:
Infections: Upper respiratory infections, strep throat, Yersinia.
Cancer: Especially hematologic malignancies like leukemia and lymphoma.
Inflammatory diseases: Inflammatory bowel disease (Crohn's disease, ulcerative colitis), rheumatoid arthritis, systemic lupus erythematosus.
Drug-induced: Certain medications, including granulocyte colony-stimulating factor (G-CSF), antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), and some cancer drugs.
Pregnancy: Can occur during or after pregnancy.
Medicine Used
Corticosteroids: Oral or topical corticosteroids (like prednisone) are the most common treatment. Oral corticosteroids are generally used for more severe or widespread cases.
Topical corticosteroids: May be sufficient for milder, localized cases.
Other medications: For patients who cannot tolerate or do not respond to corticosteroids, other options include:
Dapsone
Colchicine
Potassium iodide
Cyclosporine
Indomethacin
TNF inhibitors (etanercept, infliximab) if associated with inflammatory conditions.
Address Underlying Cause: Treat any underlying infection, cancer, or inflammatory condition. Discontinue any medication suspected of causing the syndrome.
Is Communicable
No, Sweet syndrome is not contagious. It is not caused by an infectious agent and cannot be spread from person to person.
Precautions
Consult a doctor: If you develop symptoms of Sweet syndrome, see a doctor promptly for diagnosis and treatment.
Avoid potential triggers: If your Sweet syndrome is drug-induced, avoid the causative medication.
Manage underlying conditions: If Sweet syndrome is associated with an underlying condition (infection, cancer, inflammatory disease), manage that condition appropriately.
Protect skin: Keep affected skin clean and moisturized. Avoid harsh soaps and chemicals that can irritate the skin. Protect the skin from sun exposure.
How long does an outbreak last?
Without treatment, Sweet syndrome outbreaks can last for weeks to months. With corticosteroid treatment, symptoms usually improve rapidly within days to weeks. Relapses are common, occurring in up to one-third of patients.
How is it diagnosed?
Diagnosis of Sweet syndrome typically involves:
Clinical examination: Evaluation of the characteristic skin lesions and other symptoms.
Skin biopsy: A small sample of affected skin is removed and examined under a microscope to look for specific inflammatory changes, including a dense infiltrate of neutrophils.
Blood tests: To assess white blood cell count (neutrophilia) and to rule out other conditions.
Evaluation for underlying conditions: Depending on the clinical picture, further investigations may be needed to look for associated infections, cancers, or inflammatory diseases.
Timeline of Symptoms
Sudden onset: Skin lesions typically appear suddenly, often over a period of hours to days.
Rapid progression: Lesions can enlarge and merge quickly.
Fever: Fever may be present at the onset of the lesions or develop shortly thereafter.
Resolution with treatment: With appropriate treatment (usually corticosteroids), symptoms typically resolve within days to weeks.
Relapses: Recurrence of symptoms can occur in some patients, even after successful treatment.
Important Considerations
Underlying cause: It is crucial to identify and treat any underlying cause of Sweet syndrome, as this may influence the treatment approach and prognosis.
Drug-induced Sweet syndrome: Always consider the possibility of drug-induced Sweet syndrome, especially if the patient has recently started a new medication.
Cancer-associated Sweet syndrome: Be aware of the association between Sweet syndrome and cancer, particularly hematologic malignancies.
Relapses: Be aware that relapses are possible even after successful treatment and monitor patients accordingly.
Differential diagnosis: Sweet syndrome can mimic other skin conditions, so it is important to consider the differential diagnosis and obtain a skin biopsy for confirmation.
Systemic involvement: Though primarily a skin condition, Sweet syndrome can sometimes affect internal organs. Be aware of the possibility of systemic involvement and monitor patients accordingly.