-
Actas Dermo-sifiliograficas Jun 2016Sweet syndrome is the most representative entity of febrile neutrophilic dermatoses. It typically presents in patients with pirexya, neutrophilia, painful tender... (Review)
Review
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.
Topics: Algorithms; Humans; Sweet Syndrome
PubMed: 26826881
DOI: 10.1016/j.ad.2015.12.001 -
American Journal of Clinical Dermatology May 2022Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous... (Review)
Review
Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous papules/plaques/pustules/nodules commonly appearing on the upper limbs, trunk, and head and neck; histologically, SS is characterized by dense neutrophilic infiltrate in the dermis. SS is accompanied by fever; an elevation of inflammatory markers (e.g., erythrocyte sedimentation rate, C reactive protein) in serum may also be observed. Although most cases of SS are idiopathic, SS also occurs in the setting of malignancy or following administration of an associated drug. SS has also been reported in association with pregnancy and a burgeoning list of infectious (most commonly upper respiratory tract infections) and inflammatory diseases; likewise, the litany of possible iatrogenic triggers has also grown. Over the past several years, a wider spectrum of SS presentation has been realized, with several reports highlighting novel clinical and histological variants. Corticosteroids continue to be efficacious first-line therapy for the majority of patients with SS, although novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory SS. New mechanisms of SS induction have also been recognized, although the precise etiology of SS still remains elusive. Here, we catalogue the various clinical and histological presentations of SS, summarize recently reported disease associations and iatrogenic triggers, and review treatment options. We also attempt to frame the findings of this review in the context of established and emerging paradigms of SS pathogenesis.
Topics: Adrenal Cortex Hormones; Humans; Iatrogenic Disease; Neck; Skin; Sweet Syndrome
PubMed: 35157247
DOI: 10.1007/s40257-022-00673-4 -
Dermatology (Basel, Switzerland) 2023Sweet syndrome (SS), the prototypical neutrophilic dermatosis, is characterized by abrupt onset of tender plaques and nodules, classically accompanied by fever and... (Review)
Review
Sweet syndrome (SS), the prototypical neutrophilic dermatosis, is characterized by abrupt onset of tender plaques and nodules, classically accompanied by fever and leukocytosis. While management mainly relies on systemic corticosteroids, inadequate response can be seen in some patients that necessitates exploring other treatment options. Early diagnosis of malignancy-associated SS (MA-SS) along with detection of concomitant malignancy is crucial for improving patients' outcomes. Data regarding various clinical manifestations, extracutaneous associations, treatment, and outcomes are poorly characterized in the literature. We aimed to review all published case reports and case series to portray clinical features of SS including extracutaneous manifestations. We also describe reported treatment options and their outcomes to draw attention toward unmet therapeutic needs in the management of SS. In addition, for clinical and practical purposes, we attempted to delineate the distinction between MA-SS and nonmalignant subtypes of SS.
Topics: Humans; Sweet Syndrome; Leukocytosis; Fever; Adrenal Cortex Hormones; Neoplasms
PubMed: 37019090
DOI: 10.1159/000530519 -
Dermatologic Clinics Apr 2022Pediatric Sweet syndrome (SS) is thought to be a hypersensitivity reaction to an underlying inflammatory or infectious state and typically is diagnosed using criteria... (Review)
Review
Pediatric Sweet syndrome (SS) is thought to be a hypersensitivity reaction to an underlying inflammatory or infectious state and typically is diagnosed using criteria created for adult patients. Although more studies are needed to understand the etiology and natural course of pediatric SS, guidelines for work-up and treatment have been suggested. Herein, the available literature is reviewed and guidelines summarized for the clinical evaluation and management of pediatric SS.
Topics: Adult; Child; Humans; Hypersensitivity; Sweet Syndrome
PubMed: 35366971
DOI: 10.1016/j.det.2021.12.005 -
Orphanet Journal of Rare Diseases Jul 2007Sweet's syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic... (Review)
Review
Sweet's syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Several hundreds cases of Sweet's syndrome have been published. Sweet's syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. Classical Sweet's syndrome (CSS) usually presents in women between the age of 30 to 50 years, it is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. Approximately one-third of patients with CSS experience recurrence of the dermatosis. The malignancy-associated Sweet's syndrome (MASS) can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet's syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered; MASS is most commonly related to acute myelogenous leukemia. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's cancer. Hence, MASS can be the cutaneous harbinger of either an undiagnosed visceral malignancy in a previously cancer-free individual or an unsuspected cancer recurrence in an oncology patient. Drug-induced Sweet's syndrome (DISS) most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, however, other medications may also be associated with DISS. The pathogenesis of Sweet's syndrome may be multifactorial and still remains to be definitively established. Clinical and laboratory evidence suggests that cytokines have an etiologic role. Systemic corticosteroids are the therapeutic gold standard for Sweet's syndrome. After initiation of treatment with systemic corticosteroids, there is a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions. Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions. Other first-line oral systemic agents are potassium iodide and colchicine. Second-line oral systemic agents include indomethacin, clofazimine, cyclosporine, and dapsone. The symptoms and lesions of Sweet's syndrome may resolved spontaneously, without any therapeutic intervention; however, recurrence may follow either spontaneous remission or therapy-induced clinical resolution.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Sweet Syndrome
PubMed: 17655751
DOI: 10.1186/1750-1172-2-34 -
Dermatologic Therapy Jan 2021Tyrosine kinase inhibitors are a class of targeted anticancer drugs that inhibit cancer cell proliferation by inactivating proteins involved in signal transduction... (Review)
Review
Tyrosine kinase inhibitors are a class of targeted anticancer drugs that inhibit cancer cell proliferation by inactivating proteins involved in signal transduction cascades. Various cutaneous adverse events have been observed after tyrosine kinase inhibitor administration, including Sweet syndrome. We queried the PubMed database to identify 14 cases of Sweet syndrome thought to be secondary to tyrosine kinase inhibitors. Tyrosine kinase inhibitor-induced Sweet syndrome had a median of 2 months latency following drug administration. All cases but one had morphologic features classic for Sweet syndrome (erythematous and tender papules, plaques, or nodules). All cases also had classic histopathologic findings (dermal neutrophilic infiltrate without vasculitis or necrosis). Using diagnostic criteria for drug-induced Sweet syndrome and the Naranjo Drug Reaction Probability Scale for a drug-induced cutaneous eruption, we found that six cases favored a drug-induced etiology over malignancy, two cases favored a malignancy-associated Sweet syndrome, and the remaining eight met drug-induced Sweet syndrome criteria but had low Naranjo scores. Nine cases resulted in medication discontinuation, while five cases continued anticancer therapy and were treated only with corticosteroids with quick resolution of skin lesions. Dermatologists should be aware of this adverse cutaneous reaction to tyrosine kinase inhibitors and should treat on a case-by-case basis, though limited evidence in this review suggests that oncologic therapy may safely be continued with prompt corticosteroid treatment.
Topics: Drug Eruptions; Humans; Neoplasms; Protein Kinase Inhibitors; Skin; Sweet Syndrome
PubMed: 33112465
DOI: 10.1111/dth.14461 -
Ugeskrift For Laeger May 2018Sweet syndrome (SS) exists as classical, malignancy-associated, drug-induced and as the new variants: giant cellulitis-like SS and neutrophilic dermatosis of the hands.... (Review)
Review
Sweet syndrome (SS) exists as classical, malignancy-associated, drug-induced and as the new variants: giant cellulitis-like SS and neutrophilic dermatosis of the hands. SS exhibits different morphologies. Skin manifestations are usually accompanied by fever and neutrocytosis. All variants respond to systemic corticosteroids, but SS can recur. SS may be the first sign of malignancy or recurrence of previous cancer. It is important to be aware of the disease, which may mimic other reactive and febrile diseases, to enable patients to obtain the correct diagnostic set-up and treatment.
Topics: Diagnosis, Differential; Humans; Sweet Syndrome
PubMed: 29761780
DOI: No ID Found -
Dermatologic Clinics Oct 2008Neutrophilic panniculitis encompasses a heterogeneous group of diseases histopathologically characterized by an inflammatory infiltrate in the subcutaneous fat mainly... (Review)
Review
Neutrophilic panniculitis encompasses a heterogeneous group of diseases histopathologically characterized by an inflammatory infiltrate in the subcutaneous fat mainly composed of mature neutrophils. This group of panniculitides includes alpha(1)-antitrypsin deficiency, infectious panniculitis, factitious panniculitis, subcutaneous Sweet syndrome, neutrophilic/pustular panniculitis associated with rheumatoid arthritis, erythema nodosum-like lesions of Behçet disease, bowel bypass panniculitis, and iatrogenic panniculitis. This article reviews subcutaneous Sweet syndrome, which is a rare idiopathic panniculitis characterized by a dense neutrophilic infiltrate in the subcutis and is often related to hematologic malignancies. The relationship of subcutaneous Sweet syndrome and erythema nodosum is discussed as well as the differential diagnosis with other neutrophilic panniculitis.
Topics: Biopsy; Diagnosis, Differential; Glucocorticoids; Humans; Subcutaneous Tissue; Sweet Syndrome
PubMed: 18793988
DOI: 10.1016/j.det.2008.06.003 -
The New England Journal of Medicine Apr 2020
Topics: Abdominal Abscess; Humans; Male; Middle Aged; Neck; Sweet Syndrome
PubMed: 32294348
DOI: 10.1056/NEJMicm1911025 -
Journal Der Deutschen Dermatologischen... Nov 2017The diagnosis of Sweet's syndrome (SS) is based on a set of criteria that requires the presence of two major and at least two minor criteria. In some cases, however, the... (Review)
Review
The diagnosis of Sweet's syndrome (SS) is based on a set of criteria that requires the presence of two major and at least two minor criteria. In some cases, however, the diagnosis is not as straightforward due to the absence of certain criteria. The objective of the present study was to review the clinical, histopathological, and laboratory features of the current diagnostic criteria for SS, and to evaluate their validity in the cases reported in the literature as well as in 40 patients treated at our institution. Our comprehensive review of the current criteria for SS reveals that the two major criteria have been consistently present in all cases - including ours - since the first description of SS in 1964. With regard to the minor criteria, on the other hand, there has been marked variability between different studies, and many cases failed to fulfill the requirement of showing two minor criteria. In order to simplify the diagnosis, avoid misdiagnosis, and allow for prompt treatment, we propose two sets of revised diagnostic criteria for SS. The first set comprises constant clinical and histopathological features that must be present and are by themselves sufficient for the diagnosis of SS to be established. The second set includes variable features whose absence does not warrant ruling out SS.
Topics: Adrenal Cortex Hormones; Adult; Aged; Biopsy; Diagnosis, Differential; Female; Humans; Inflammation Mediators; Male; Middle Aged; Neutrophils; Skin; Sweet Syndrome; Treatment Outcome
PubMed: 28981179
DOI: 10.1111/ddg.13350