Sweet's syndrome was originally described in 1964 by Dr Robert Douglas Sweet as an '
acute febrile neutrophilic dermatosis'. The syndrome is characterized by
pyrexia, elevated neutrophil count, painful red papules, nodules, plaques (which may be recurrent) and an infiltrate consisting predominantly of mature neutrophils that are diffusely distributed in the upper dermis. In addition to skin and mucosal lesions,
Sweet's syndrome can also present with extra-cutaneous manifestations.
Sweet's syndrome can be classified based upon the clinical setting in which it occurs: classical or idiopathic
Sweet's syndrome,
malignancy-associated
Sweet's syndrome and
drug-induced
Sweet's syndrome. Systemic
corticosteroids have been considered the 'gold standard' for the treatment of patients with
Sweet's syndrome; in addition, treatment with topical and/or intralesional
corticosteroids may be effective as either monotherapy or adjuvant
therapy. However, spontaneous resolution of the symptoms and lesions has occurred in several patients with
Sweet's syndrome for whom disease-specific therapeutic intervention was not initiated and in some of the patients with
drug-induced
Sweet's syndrome after withdrawal of the
dermatosis-causing medication. Oral
therapy with either
potassium iodide or
colchicine typically results in rapid resolution of
Sweet's syndrome symptoms and lesions; therefore, in patients with
Sweet's syndrome who have a potential systemic
infection or in whom
corticosteroids are contraindicated, it is reasonable to initiate treatment with these agents as a first-line
therapy.
Indomethacin,
clofazimine,
dapsone, and
cyclosporine have also been effective therapeutic agents for managing
Sweet's syndrome. However,
indomethacin and
clofazimine appear less effective than
corticosteroids,
potassium iodide, and
colchicine. Appropriate initial and follow-up laboratory monitoring is necessary when treating with either
dapsone or
cyclosporine because of the potential for severe adverse
drug-associated effects. Systemic antibacterials with activity against Staphylococcus aureus frequently result in partial improvement of
Sweet's syndrome lesions when they are impetiginized or secondarily infected. In some patients with
dermatosis-associated
bacterial infections, organism-sensitive specific systemic antibacterials have been helpful in the management of their
Sweet's syndrome. Although patients with
hematologic malignancy-associated
Sweet's syndrome often receive cytotoxic
chemotherapy agents and antimetabolic drugs for the treatment of their underlying disorder, these agents are seldom used solely for the management of the symptoms and lesions of
Sweet's syndrome. The treatment of patients with
Sweet's syndrome with either
etretinate or
interferon-alpha have been reported as single case reports; both patients had improvement of not only their
Sweet's syndrome lesions, but also their associated hematologic disorder.