Review
Sweet Syndrome: A Review and UpdateSíndrome de Sweet: Revisión y puesta al día

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Abstract

Sweet syndrome is the most representative entity of febrile neutrophilic dermatoses. It typically presents in patients with pirexya, neutrophilia, painful tender erytomatous papules, nodules and plaques often distributed asymmetrically. Frequent sites include the face, neck and upper extremities. Affected sites show a characteristical neutrophilic infiltrate in the upper dermis. Its etiology remains elucidated, but it seems that can be mediated by a hypersensitivity reaction in which cytokines, followed by infiltration of neutrophils, may be involved. Systemic corticosteroids are the first-line of treatment in most cases. We present a concise review of the pathogenesis, classification, diagnosis and treatment update of this entity.

Resumen

El síndrome de Sweet es la entidad más representativa de las dermatosis neutrofílicas. Por lo general se presenta en pacientes con fiebre, neutrofilia, pápulas erytomatosas dolorosas, nódulos y placas. Los sitios frecuentemente afectados incluyen la cara, cuello y extremidades superiores los cuales característicamente presentan un infiltrado neutrofílico en la dermis superior. Su etiología no esta bien establecida, pero parece que puede estar mediada por una reacción de hipersensibilidad de las citocinas, seguido por un infiltrado de neutrófilos. Los corticosteroides sistémicos son la primera línea de tratamiento en la mayoría de los casos. Se presenta una revisión actual de la patogénesis, clasificación, diagnóstico y tratamiento de esta entidad.

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.

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      These nodules rarely ulcerate and, in these cases, they resemble the clinical appearance of pyoderma gangrenosum lesions. Their predominant distribution is on the face, trunk, and proximal extremities, but can occur in areas of trauma, characterizing a pathergy reaction.21 It is more frequent in women between 30 and 60 years of age.

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