Sweet’s syndrome: a neutrophilic dermatosis classically associated with acute onset and fever
Section snippets
History
Dr. Robert Douglas Sweet encountered eight women with “a distinctive and fairly severe illness” during the 15-year period from 1949 to 1964. He authored a paper entitled “An Acute Febrile Neutrophilic Dermatosis” in order to “draw a composite picture of the condition and behaviour of these patients and to describe briefly their variations.” The paper was published in the August–September 1964 issue of the British Journal of Dermatology.1 Dr. Sweet recommended that the name for this condition
Epidemiology
Sweet’s syndrome has a worldwide distribution and no racial predilection.5, 6, 7, 8, 9, 10, 19, 20 Several of the cases of Sweet’s syndrome are associated with cancer,3, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32 suggesting a classification that distinguishes between the classical and the malignancy-associated form of the disease. Recently, several reports have appeared of patients having a drug-induced variant of the dermatosis.4 Classical Sweet’s syndrome predominantly affects women
Associated diseases
In Sweet’s syndrome patients, several conditions have been observed3, 4, 5, 6, 7, 8, 10, 18, 19, 40, 42, 53, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 83, 84, 85, 86, 87, 88, 52, 89, 90, 91, 92, 93, 94, 95, 96, 97: see Table 4 Table 5, and Table 6. Although some of these conditions may have an etiologic role in the pathogenesis of Sweet’s syndrome, others are more likely to represent a coincidental occurrence of the condition in a
Symptoms
The systemic symptoms that characteristically accompany the skin eruption are fever and leukocytosis, and patients with Sweet’s syndrome often appear dramatically ill. The fever may precede the skin disease by several days to weeks; it may also be present throughout the entire disease course. In addition to fever, other symptoms that might occur include (to varying degrees) arthralgia, general malaise, headache, and myalgia. Not all cases of Sweet’s syndrome, however, express the entire
Pathology
The most prominent histopathologic features of Sweet’s syndrome are (1) a dense infiltrate predominantly composed of mature neutrophils located primarily in the superficial dermis and (2) edema of the dermal papillae and papillary dermis Figure 2, Figure 3, Figure 4. The inflammatory infiltrate is characteristically diffuse; however, it may also be perivascular. Occasional eosinophils and lymphocytes may be present in the infiltrate. Fragmented neutrophil nuclei (leukocytoclasia or
Laboratory findings
The most consistent laboratory findings in Sweet’s syndrome are (1) an elevated erythrocyte sedimentation rate and (2) peripheral leukocytosis with neutrophilia (Table 1); however, leukocytosis is not always present in patients with biopsy-confirmed Sweet’s syndrome.13 Anemia, a normal or low neutrophil count, and/or an abnormal platelet count may be observed in patients with malignancy-associated Sweet’s syndrome.3 The presence of neutrophils in the cerebrospinal fluid has been described in
Diagnosis and differential diagnosis
Table 7 summarizes the diagnostic criteria for classical Sweet’s syndrome (which in this list of diagnostic criteria includes not only the patients with idiopathic Sweet’s syndrome but also patients with malignancy-associated Sweet’s syndrome) and for drug-induced Sweet’s syndrome.4, 8, 155 Conditions that might clinically mimic Sweet’s syndrome are listed in Table 8 (Fig 5). The3, 8, 13, 22, 55, 156, 157, 158 histologic differential diagnosis of Sweet’s syndrome includes conditions
Treatment
The fever, leukocytosis, and symptoms of Sweet’s syndrome suggest a septic process; however, the dermatosis usually persists following treatment with only antibiotics. Because the skin lesions have often become secondarily impetiginized, systemic therapy directed toward Staphyloccocus aureus may result in slight improvement.
There are isolated reports of Sweet’s syndrome that have resolved after treatment with antibiotics. One team166 described a patient with Crohn’s disease whose lesions
Pathogenesis
The specific etiology of Sweet’s syndrome is not known. Although there is no evidence that a bacterial infection has a causative role in this dermatosis, the accompanying fever and peripheral leukocytosis suggest a septic process. Many patients with classical Sweet’s syndrome have an upper respiratory tract infection, tonsillitis, or influenza-like illness that precedes their skin lesions by 1 to 3 weeks. In addition, some of the patients with Sweet’s syndrome have a culture-positive,
Conclusions
Sweet’s syndrome, originally described as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings that typically include (1) fever, (2) neutrophilia, (3) erythematous tender plaques and/or nodules that may be recurrent, and (4) a diffuse dermal infiltrate composed primarily of mature neutrophils. Extracutaneous manifestations of Sweet’s syndrome can involve the following systems: cardiovascular, CNS, gastrointestinal, hepatic, musculoskeletal, and
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