Sweet syndrome

Sweet syndrome
Synonyms	Acute febrile neutrophilic dermatosis
Pronounce	N/A
Specialty	N/A
Symptoms	Fever, painful skin lesions
Complications
Onset
Duration
Types	N/A
Causes	Idiopathic, associated with infections, malignancies, or medications
Risks
Diagnosis	Clinical, skin biopsy
Differential diagnosis
Prevention	N/A
Treatment	Corticosteroids, potassium iodide
Medication
Prognosis
Frequency	Rare
Deaths	N/A

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare skin condition characterized by the sudden onset of fever, elevated white blood cell count, and tender, erythematous skin lesions. It was first described by Dr. Robert Douglas Sweet in 1964.

Etiology

Sweet syndrome can be classified into three main categories based on its etiology:

• Idiopathic Sweet syndrome: The cause is unknown and it occurs spontaneously without any identifiable trigger.
• Malignancy-associated Sweet syndrome: This form is associated with underlying hematologic malignancies such as acute myeloid leukemia or myelodysplastic syndromes.
• Drug-induced Sweet syndrome: Certain medications, such as granulocyte colony-stimulating factor, can trigger the condition.

Pathophysiology

The exact pathophysiological mechanism of Sweet syndrome is not fully understood. It is believed to involve an abnormal immune response, leading to the infiltration of neutrophils in the dermis. This results in the characteristic skin lesions and systemic symptoms.

Clinical Features

Sweet syndrome is characterized by the following clinical features:

• Fever: Patients often present with a high fever.
• Skin Lesions: The hallmark of Sweet syndrome is the presence of tender, erythematous papules, plaques, or nodules. These lesions are typically found on the face, neck, and upper extremities.
• Mucosal Involvement: In some cases, oral or ocular mucosa may be involved.

Diagnosis

The diagnosis of Sweet syndrome is primarily clinical, supported by histopathological findings from a skin biopsy. The biopsy typically shows a dense infiltrate of neutrophils in the upper dermis without evidence of vasculitis.

Differential Diagnosis

The differential diagnosis for Sweet syndrome includes:

• Pyoderma gangrenosum
• Erythema multiforme
• Vasculitis
• Infectious diseases such as cellulitis

Treatment

The mainstay of treatment for Sweet syndrome is systemic corticosteroids, which usually lead to rapid improvement of symptoms. Other treatment options include:

• Potassium iodide: An alternative treatment for patients who cannot tolerate corticosteroids.
• Colchicine: May be used in some cases.
• Dapsone: Another alternative treatment.

Prognosis

The prognosis for Sweet syndrome is generally good, especially when associated with drug reactions or idiopathic cases. However, the prognosis may vary in malignancy-associated cases, depending on the underlying cancer.

See Also

• Neutrophilic dermatoses
• Dermatology

External Links

• [Link to a dermatology resource]

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