From the Guidelines
Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare skin condition that should be treated with systemic corticosteroids as the primary treatment option. The condition is characterized by tender, red inflammatory nodules or papules, usually affecting the upper limbs, face or neck, and has been recognized as an extraintestinal manifestation of inflammatory bowel disease (IBD) 1.
The recommended treatment regimen is:
- Prednisone 0.5-1 mg/kg/day orally for 4-6 weeks
- Gradually taper the dose over several weeks to prevent recurrence Additional treatments may include:
- Topical corticosteroids for localized lesions
- Colchicine 0.5 mg twice daily for mild cases or as a steroid-sparing agent
- Dapsone 100-200 mg daily for refractory cases Patients should also address any underlying conditions that may trigger Sweet syndrome, such as infections, malignancies, or inflammatory diseases 1.
Systemic corticosteroids have been reported to be effective in treating Sweet syndrome, as they suppress the inflammatory response and neutrophil activity, which are key factors in the development of Sweet syndrome lesions 1. The gradual tapering of corticosteroids helps prevent rebound flares. Alternative treatments like colchicine and dapsone work by inhibiting neutrophil function through different mechanisms. It's essential to monitor for potential side effects of corticosteroids and adjust the treatment plan as needed, with regular follow-up with a healthcare provider to assess treatment response and manage any complications 1.
From the Research
Definition and Characteristics of Sweet's Syndrome
- Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful red papules, nodules, plaques, and an infiltrate consisting predominantly of mature neutrophils in the upper dermis 2, 3, 4, 5, 6.
- The syndrome can present with extra-cutaneous manifestations and can be classified into classical or idiopathic Sweet's syndrome, malignancy-associated Sweet's syndrome, and drug-induced Sweet's syndrome 2, 4.
Treatment Options for Sweet's Syndrome
- Systemic corticosteroids are considered the 'gold standard' for the treatment of patients with Sweet's syndrome, with topical and/or intralesional corticosteroids also being effective as either monotherapy or adjuvant therapy 2, 4, 5.
- Other treatment options include oral therapy with potassium iodide or colchicine, which can result in rapid resolution of symptoms and lesions 2, 3, 4.
- Indomethacin, clofazimine, dapsone, and cyclosporine have also been effective therapeutic agents for managing Sweet's syndrome, although indomethacin and clofazimine appear less effective than corticosteroids, potassium iodide, and colchicine 2.
- Spontaneous resolution of symptoms and lesions can occur in some patients, and recurrence may follow either spontaneous remission or therapy-induced clinical resolution 2, 4, 5.
Clinical Settings and Associated Disorders
- Sweet's syndrome can occur in various clinical settings, including classical or idiopathic, malignancy-associated, and drug-induced 2, 4.
- The syndrome can be associated with various disorders, such as inflammatory bowel disease, pregnancy, and malignancies, and regular monitoring is necessary to eliminate these conditions and initiate early specific treatment 4, 5.
- Histopathological variants of Sweet's syndrome, including subcutaneous, eosinophilic, histiocytoid, and lymphocytic types, have been described in the literature 5.