ReviewSweet Syndrome: A Review and UpdateSíndrome de Sweet: Revisión y puesta al día
Introduction
Since it was first described by Dr. Robert Douglas Sweet, originally known as Gomm-Button disease (in reference to the first two patients), Sweet's syndrome, also referred to as febrile neutrophilic dermatosis, has been reported in hundreds of patients worldwide.1 Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that include Sweet's syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis,2 the former being the most represented and the focus of this review.
The purpose of this article is to make a review of Sweet syndrome, its clinical manifestations, definition, pathogenesis, diagnosis and management of this entity. It is essential for dermatologists to know the different aspects of Sweet's syndrome as well as its proper diagnosis, prevention and treatment.
Section snippets
Definition
Clinically, Sweet's syndrome presents in patients, all of which show characteristic neutrophilic infiltrate in the upper dermis.3
Sweet's syndrome can present as one of three clinical types: classical (or idiopathic) Sweet's syndrome, malignancy-associated Sweet's syndrome, or drug-induced Sweet's syndrome. Specific diagnostic criteria were proposed by Su and Liu4 and subsequently revised and modified by von den Driesch.5 Laboratory abnormalities may be found and are included in the diagnostic
Etiology
Sweet's syndrome is an inflammatory skin disorder characterized by the extensive infiltration of neutrophils into the epidermis and dermis. For a dermatologist, understanding the pathophysiology of Sweet's syndrome is crucial for treatment.
The underlining biological pathways responsible for this cutaneous neutrophilic dermatosis have remained elusive. However, the association of this disease with infection, autoimmune diseases, neoplasms and drugs suggests an unusual hypersensitivity that may
Clinical manifestations
Classical Sweet's syndrome has a worldwide distribution, usually presenting in middle age women with a 4:1 female to male ratio, no racial disparity, and recurrence in one-third of patients.3 It presents as an acute febrile neutrophilic dermatosis characterized by a constellation of clinical symptoms, physical features, and pathological findings that include fever, neutrophilia, asymmetrically distributed painful tender erythematous skin lesions, consisting of papules, nodules and plaques,
Histopathology
Histopathological analysis that characterizes a dense and diffuse dermal neutrophilic infiltrate is important for the diagnosis of the disease because the differential diagnosis of Sweet's syndrome is extensive.
Histopathological diagnostic criteria include the presence of diffuse neutrophilic infiltrate in the dermis, edema, and fragmentation of the nuclei of neutrophils (Fig. 2). The predominant cells that comprise the infiltrate in the dermis of cutaneous Sweet's syndrome lesions are mature
Management
The management of patients with a Sweet's syndrome can be performed in 3 steps: assessment, workup, and treatment. Assessment includes the identification of the type of cutaneous lesion, the existence of possible extracutaneous sites, and the search for associated disease. In every case, it is important to rule out the presence of an infection and to have a precise drug-therapy history. Also, the potential presence of hematological diseases must be systematically investigated. Furthermore, a
Protection of human and animal subjects
The authors declare that no experiments were performed on humans or animals for this investigation.
Confidentiality of data
The authors declare that no patient data appears in this article.
Right to privacy and informed consent
The authors declare that no patient data appears in this article.
Conflicts of interest
The authors declare that they have no conflicts of interest.
References (100)
Sweet's syndrome (acute febrile neutrophilic dermatosis)
J Am Acad Dermatol
(1994)- et al.
Sweet's syndrome: is the pathogenesis mediated by helper T cell type 1 cytokines?
J Am Acad Dermatol
(1998) Sweet's syndrome: a revisit for hematologists and oncologists
Crit Rev Oncol/Hematol
(2013)- et al.
Analysis of HLA antigens in Caucasian patients with acute febrile neutrophilic dermatosis (Sweet's syndrome)
J Am Acad Dermatol
(1997) - et al.
Alteration in the gene encoding protein tyrosine phosphatase nonreceptor type 6 (PTPN6/SHP1) may contribute to neutrophilic dermatoses
Am J Pathol
(2011) - et al.
Sweet's syndrome with bullous lesions
Acta Dermo-Sifiliogr
(2005) - et al.
Sweet syndrome in a pregnant woman
Acta Dermo-Sifiliogr
(2010) - et al.
The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients
J Am Acad Dermatol
(2006) - et al.
Sweet's syndrome and cancer
Clin Dermatol
(1993) - et al.
Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome
J Am Acad Dermatol
(1996)