From the Guidelines
Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) is diagnosed based on its characteristic tender, red inflammatory nodules or papules, and managed with systemic corticosteroids.
Diagnosis
- The condition is characterized by tender, red inflammatory nodules or papules, usually affecting the upper limbs, face or neck 1.
- It can be distinguished from other acute neutrophilic dermatoses, such as pyoderma gangrenosum, by its appearance, distribution, and histological features.
Management
- Systemic corticosteroids have been reported to be effective in managing Sweet Syndrome 1.
- The rash is mostly associated with active disease, particularly in patients with colonic involvement and other extraintestinal manifestations, suggesting that treatment of the underlying condition may also be necessary.
- There is a strong predilection for women, and patients with a history of extraintestinal manifestations, which may inform the diagnosis and management of Sweet Syndrome 1.
From the Research
Diagnosis of Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)
- The diagnosis of Sweet Syndrome is based on a combination of clinical symptoms, physical features, and pathologic findings, including fever, neutrophilia, tender erythematous skin lesions, and a diffuse infiltrate consisting predominantly of mature neutrophils in the upper dermis 2, 3.
- The syndrome can present in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced 3.
- Classical Sweet's syndrome 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 3.
- Malignancy-associated Sweet's syndrome 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 3, 4.
Management of Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)
- Systemic corticosteroids are the therapeutic gold standard for Sweet's syndrome, with a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions 2, 3, 5.
- Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions 3.
- Other first-line oral systemic agents include potassium iodide and colchicine, while second-line oral systemic agents include indomethacin, clofazimine, cyclosporine, and dapsone 3.
- Novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory Sweet's syndrome 5.
- Early diagnosis of malignancy-associated Sweet's syndrome along with detection of concomitant malignancy is crucial for improving patients' outcomes 4.