and histologically. Whittle et al. leukocytosis with polymorphonuclear predominance. dense dermal infiltration with mature neutrophils  Later. were the first to use the term “Sweet’s syndrome” as titles of their articles . neck and extremities. INTRODUCTION  Sweet’s syndrome (SS) is an acute febrile neutrophilic dermatosis first described by Robert Douglas Sweet in 1964  four cardinal manifestations of the disease: fever. and Crow et al. painful elevated plaques on the face.

in particular. EPIDEMIOLOGY  Universal distribution  Women are more commonly affected and. seem to be more affected by the idiopathic or drug-induced forms  The onset of the disease can occur at any age. but the peak incidence is usually from the fourties to the seventies  Among the earliest cases are children 10 to 15 days old .

diazepam. diclofenac. CLASSIFICATION  classic or idiopathic  malignancy-associated . trimethoprim-sulfamethoxazole. .In 21% of the patients with SS there is an association with malignant diseases. among others. dipyrone. quinolones. radiocontrast agents. trans retinoic acid. usually a hematologic disorder (AML)  drug-induced such as granulocyte colony stimulating factor (G-CSF). carbamazepine. minocycline. oral contraceptives.

there may be central clearing. The skin typically presents multiple erythematous or violaceous plaques that are well demarcated and often painful  With progression of the lesion. malaise and headache. CLINICAL MANIFESTATION  Sweet’s syndrome is acute and accompanied by fever. giving the lesion an annular pattern similar to erythema multiforme .

neck. the syndrome may be preceded by an infection (of the upper respiratory tract. back and upper extremities  Often. CLINICAL MANIFESTATION  The areas primarily affected are face. which is more common. or gastrointestinal tract) or even be associated with inflammatory bowel disease or pregnancy . chest.



HISTOLOGY OVERVIEW  the disease is characterized by the presence of a dense dermal predominantly neutrophilic inflammatory infiltrate associated with subepidermal edema of variable intensity and nuclear dust .


 The disease is defined by the presence of two major criteria and at least two minor criteria . Diagnostic criteria  Diagnostic criteria for SS were proposed by Su and Liu in 1986 and modified by von den Driesch in 1994.

MAJOR CRITERIA  Abrupt onset of nodules or painful erythematous plaques associated with histopathologic evidence of a dense neutrophilic infiltrate in the dermis without leukocytoclastic vasculitis .

positive C-reactive protein. leukocyte count above 8.000. neutrophil blood count over 70% and excellent response to treatment with corticosteroids or potassium iodide . presence of the following laboratory findings (3 of 4): ESR above 20 mm. MINOR CRITERIA  Fever above 38°C  Infection of the respiratory tract or gastrointestinal tract  Inflammatory disease  Neoplasia or pregnancy.

5 to 1.5 mg / kg / day.treatment of the underlying disease and discontinuation of the drug . tapering in 2 to 4 weeks  Other options considered as first line theraphy by some authors are potassium iodide and colchicine  Localized lesions can be managed with intralesional or high potency topical corticoids  In cases associated with malignancy or drug-induced SS. THERAPY  Systemic corticosteroid therapy is considered the gold standard treatment for SS: prednisone or prednisolone at an initial dose of 0.

is a factor of poor prognosis . according to some authors. PROGNOSIS  SS may precede or occur concurrently with the underlying disease and.

MATERIALS AND METHODS  We conducted a retrospective epidemiological study by examining the medical records of 23 patients who met the diagnostic criteria for the disease from March 1995 to July 2009.  We collected clinical and epidemiological data on the patients. such as lesion location. such as leukocyte count and ESR . conditions associated with SS and some laboratory data. presence of cutaneous and extracutaneous manifestations.

conjunctivitis and arthritis. RESULTS  The age of the patients in the study ranged from 2 to 75 years. followed by arthralgia and myalgia.  The triggering factors most commonly identified were infections of the respiratory tract.  Associated neoplasia occurred in 30% of the patients. There were more females.  The lesions mostly affected the trunk and upper limbs. especially hematologic neoplasia. .  Fever was the most common systemic manifestation.

it is necessary to know how to perform the diagnosis. carry out a full investigation as well as do the patient's follow up .  Given the high prevalence of malignant diseases in patients with Sweet's syndrome. CONCLUSION  The clinical and epidemiological data found in our study are mostly similar to those found in the literature.