SWEET'S SYNDROME

Acute febrile neutropbilic dermatosis was first establisbed as an uncommon but distinct clinical entity wben Robert Douglas Sweet described eigbt cases be bad accumulated over a 15-year period in tbe British Journal of Dermatology in 1962.' Currently, tbe eponym Sweet's syndrome (ss) is preferred to "acute febrile neutropbilic dermatosis," because we now recognize that fever and neutropbilia are variable features of tbe disease, extracutaneous manifestations are common, and cbronic recurrent forms exist.^~'' Sweet's syndrome is currently classified witb tbe neutropbilic dermatoses,•'•" a clinically diverse group of disorders tbat sbare many bistopatbologic and possibly patbogenic similarities, as well as sbowing response to similar tberapies. Tbe definition of tbe disease bas remained relatively constant since Sweet's original description of a distinct disease witb four cardinal features: (1) fever, (2) peripberal leukocytosis, (3) tender red plaques on tbe limbs, face, and neck, and (4) a dense dermal infiltrate of mature neutropbils.'-^'^ Absence of infection and a rapid response to glucocorticoid tberapy are important aspects suggestive of SS. Tbe syndrome bas been described in association witb a large number of malignancies, autoimmune diseases, and antecedent infections, most commonly upper respiratory infections.'"^'''"''-' Various drugs and vaccinations bave occasionally been implicated in inducing Sweet's syndrome.''•'"' Pbotoinduction bas been described.^' Tbe question of wbetber it represents a reactive pbenomenon or a specific entity is still unanswered, bowever cbaracteristic clinical and bistopatbologic features are present to allow for a definite diagnosis. Sui and Lui' bave proposed tbe diagnostic criteria outlined in Table 1 and feel a definitive diagnosis of ss can be made by fulfilling botb major criteria and at least two of tbe minor criteria.