From the Guidelines
Systemic corticosteroids are the most effective treatment for neutrophilic dermatosis, particularly Sweet's syndrome, which is a type of acute neutrophilic dermatosis. Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by the accumulation of neutrophils in the skin without infection 1. The condition is often associated with underlying diseases such as inflammatory bowel disease (IBD) 1.
Key Characteristics
- Tender, red inflammatory nodules or papules, usually affecting the upper limbs, face or neck
- Strong predilection for women and patients with colonic involvement and other extraintestinal manifestations
- The rash is mostly associated with active disease
Treatment Options
- Systemic corticosteroids have been reported to be effective in treating Sweet's syndrome 1
- Alternative options may include dapsone, colchicine, or potassium iodide for steroid-resistant cases
- Immunosuppressants such as cyclosporine or TNF-alpha inhibitors like infliximab may be used for severe or refractory cases
Monitoring and Management
- Patients should be monitored for underlying conditions as neutrophilic dermatoses often occur secondary to malignancies, inflammatory bowel disease, or rheumatologic disorders
- The pathophysiology involves dysregulation of neutrophil chemotaxis and activation, with overproduction of pro-inflammatory cytokines like IL-1, IL-8, and TNF-alpha, explaining why anti-inflammatory and immunomodulatory medications are effective treatment strategies 1.
From the Research
Definition and Characteristics of Neutrophilic Dermatosis
- Neutrophilic dermatosis, also known as Sweet's syndrome or acute febrile neutrophilic dermatosis, is characterized by fever, elevated neutrophil count, and painful erythematous skin lesions with an infiltrate of mature neutrophils in the upper dermis 2, 3, 4.
- The syndrome can present with extra-cutaneous manifestations and can be classified into classical, malignancy-associated, and drug-induced variants 2, 3.
Clinical Settings and Associations
- Classical Sweet's syndrome usually presents in women between 30 to 50 years old, 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 established cancer or previously undiscovered hematologic dyscrasia or solid tumor 3.
- Drug-induced Sweet's syndrome is most commonly associated with granulocyte-colony stimulating factor, but other medications may also be implicated 3.
Treatment Options
- Systemic corticosteroids are considered the gold standard for treating Sweet's syndrome, with prompt response and dramatic improvement of symptoms and skin lesions 2, 3, 5, 6.
- Alternative first-line therapies include potassium iodide and colchicine, while second-line treatments include indomethacin, clofazimine, cyclosporine, and dapsone 2, 3, 5.
- Topical high-potency corticosteroids or intralesional corticosteroids may be effective for localized lesions, and other treatment modalities such as hyperbaric oxygen and plasmapheresis may be used in certain cases 5, 6.
Pathogenesis and Associated Diseases
- The pathogenesis of Sweet's syndrome is thought to be multifactorial and may involve cytokines, with clinical and laboratory evidence suggesting an etiologic role 3.
- Sweet's syndrome can be associated with various systemic diseases, including inflammatory bowel disease, pregnancy, and hematologic malignancies 2, 3, 4.