From the Guidelines
Sweet syndrome should be suspected in patients presenting with tender, red inflammatory nodules or papules, usually affecting the upper limbs, face, or neck, particularly in those with a history of inflammatory bowel disease or other extraintestinal manifestations. The condition is characterized by its distinctive appearance, distribution, and histological features, which distinguish it from other acute neutrophilic dermatoses such as pyoderma gangrenosum 1. Key features of Sweet syndrome include:
- Tender, erythematous plaques or nodules that appear suddenly
- Predilection for women and patients with colonic involvement and other extraintestinal manifestations
- Association with active disease, particularly in patients with inflammatory bowel disease
- Effective treatment with systemic corticosteroids, as reported in the literature 1. When evaluating a patient with suspected Sweet syndrome, it is essential to consider the patient's medical history, including any underlying conditions that may be associated with the condition, such as malignancies, inflammatory bowel disease, or recent infections. Laboratory findings, including leukocytosis with neutrophilia and elevated inflammatory markers, can also support the diagnosis. A skin biopsy showing dense neutrophilic infiltrates in the dermis without vasculitis can help confirm the diagnosis.
From the Research
Presentation of Sweet Syndrome
Sweet syndrome presents with a constellation of clinical symptoms, physical features, and pathologic findings, including:
- Fever
- Neutrophilia
- Tender erythematous skin lesions (papules, nodules, and plaques)
- A diffuse infiltrate consisting predominantly of mature neutrophils in the upper dermis 2, 3, 4, 5, 6
Clinical Settings
Sweet syndrome can occur in three clinical settings:
- Classical (or idiopathic) Sweet's syndrome, which usually presents in women between the age of 30 to 50 years, often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy 2, 5
- Malignancy-associated Sweet's syndrome, which 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 2, 5
- Drug-induced Sweet's syndrome, which most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, but other medications may also be associated with it 2, 3
When to Suspect Sweet Syndrome
Sweet syndrome should be suspected in patients who present with:
- Fever and neutrophilia
- Tender erythematous skin lesions
- A history of upper respiratory tract infection, inflammatory bowel disease, or pregnancy
- A history of malignancy or exposure to certain medications 2, 3, 4, 5, 6
- Extra-cutaneous manifestations, such as eye, joint, or gastrointestinal involvement 3, 5
Key Features
Key features of Sweet syndrome include: