Current Opinion in Hematology:
MYELOID BIOLOGY: Edited by David C. Dale
Acute febrile neutrophilic dermatosis (Sweet's syndrome)
Anzalone, Charles L.a; Cohen, Philip R.b,c,d
bDepartment of Dermatology, University of Texas Medical School at Houston
cUniversity of Houston Health Center
dDepartment of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
Correspondence to Philip R. Cohen, MD, 10991 Twinleaf Court, San Diego, CA 92131, USA. E-mail: email@example.com
Purpose of review: To highlight the recent observations regarding not only research but also the clinical features and management of Sweet's syndrome.
Recent findings: Some of the new insights concerning Sweet's syndrome include: (1) bortezomib-induced Sweet's syndrome (some of which are the histiocytoid variant), (2) a rare extracutaneous manifestation of Sweet's syndrome with cardiovascular involvement including coronary artery occlusion, and (3) the possibility that photosensitivity may have a role in the pathogenesis of Sweet's syndrome.
Summary: Animal models of Sweet's syndrome and new associated medication have been observed. The definitive mechanism of pathogenesis still remains to be elucidated. Recent observations in paediatric patients suggest evaluation of dermatosis-related cardiac involvement in patients with post-Sweet's syndrome cutis laxa. Treatment advances include antitumour necrosis factor- alpha drugs; however, these medications have also been associated with inducing Sweet's syndrome. Nearly 50 years after the initial description of an acute febrile neutrophilic dermatosis by Dr Robert Douglas Sweet, the dermatosis remains a fascinating condition with regard to laboratory investigation, clinical manifestations and treatment.
Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by pyrexia, mature neutrophils in the blood and the upper dermis, painful red skin lesions and response to corticosteroid treatment. The features of this dermatosis are reviewed in several current studies [1,2▪,3,4▪,5–8,9▪,10–12,13▪▪,14–30,31▪,32,33▪▪,34–48,49▪▪,50–58,59▪▪,60–62,63▪,64–87,88▪▪,89,90▪,91,92]. We briefly describe the salient aspects of Sweet's syndrome and summarize the more recent advances regarding ‘what's new’ in this condition.
Acute febrile neutrophilic dermatosis was originally described by Dr Robert Douglas Sweet in 1964. Originally known as Gomm–Button disease (in reference to the first two patients), the condition eventually acquired the established eponym ‘Sweet's syndrome’ .
The geographic distribution of Sweet's syndrome is worldwide with no racial predilection. The condition presents in three clinical settings: classic (or idiopathic) Sweet's syndrome, malignancy-associated Sweet's syndrome and drug-induced Sweet's syndrome; diagnostic criteria have been established for the former and the latter [2▪].
CAUSE AND PATHOGENESIS
The pathogenesis of Sweet's syndrome remains to be elucidated. The process may be multifactorial and many causes have been proposed. Sweet's syndrome may result from a hypersensitivity reaction to a bacterial, tumour or viral antigen. The accompanying fever and peripheral leukocytosis suggests a septic process. Circulating autoantibodies, cytokines, dermal dendrocytes, immune complexes, human leukocyte antigen serotypes and leukotactic mechanisms have all been postulated to have an etiologic role in the development of Sweet's syndrome symptoms [3,4▪].
WHAT IS NEW?
Animal models of Sweet's syndrome have been described: an alteration in the gene encoding protein tyrosine phosphatase nonreceptor type 6 – originally found in a mouse with clinical and histopathological characteristics resembling Sweet's syndrome – appears to be involved in the pathogenesis of certain subsets of neutrophilic dermatoses, including Sweet's syndrome . A sterile neutrophilic dermatosis of the skin – similar to Sweet's syndrome – has been described in a female Dachschund; the condition responded rapidly to corticosteroid therapy . Lesions resembling Sweet's syndrome were also reported in a female standard poodle; subsequent examination also revealed extracutaneous involvement of the oesophagus, heart, lungs and tarsus (joint space and synovium) .
The immunoreactivity of several cytokines (interleukin-8, interleukin-17 and tumour necrosis factor-alpha), inflammatory cell markers (CD3, CD163 and myeloperoxidase), metalloproteinases (metalloproteinase-2 and metalloproteinase-9) and vascular endothelial growth factor has displayed significantly higher values in the lesional skin of patients with Sweet's syndrome than in the lesional skin of non-Sweet's syndrome individuals or patients with other neutrophilic dermatoses . The coexistence of Sweet's syndrome, Hashimoto thyroiditis and psoriasis in a 48-year-old white woman suggests a possible CD4+ T-cell dysfunction .
Sweet's syndrome has been reported in a 5-week-old Japanese girl with non-B54 types of human leukocyte antigen [9▪]. She is one of the youngest patients reported with Sweet's syndrome; this observation suggests the importance of genetic background in association with the dermatosis [9▪].
It has been postulated that photosensitivity may play a role in the pathogenesis of Sweet's syndrome, although the pathomechanism is unknown. One theory suggests an isomorphic Koebner reaction; another proposed mechanism associates ultraviolet-B radiation with neutrophil activation and epidermal production of tumour necrosis factor-alpha and interleukin-8 . Indeed, experimental induction of Sweet's syndrome has been elicited by phototesting . Also, photoactivate drug-associated Sweet's syndrome has been observed in a woman who was receiving trimethoprim-sulfamethoxazole .
Pertinent clinical findings of Sweet's syndrome include characteristic cutaneous lesions with accompanying patient history symptoms, as well as other related findings (extracutaneous manifestation and associated conditions).
Sweet's syndrome patients may appear dramatically ill. Fever is the most frequent symptom; the skin eruptions are also usually accompanied by leukocytosis. Arthralgia, general malaise, headache and myalgia are other symptoms associated with Sweet's syndrome .
What is new?
Sweet's syndrome and its systemic manifestations closely mimic familial Mediterranean fever. The clinical similarities suggest the possibility of either a common underlying mechanism or that both diseases represent a continuum of a reactive neutrophilic condition .
Sweet's syndrome skin lesions are typically tender, red to violaceous papules or nodules; they may be pseudovesicular in appearance. The lesions are often distributed asymmetrically; frequent sites include the face, neck and upper extremities (Figs 1–3) [13▪▪].
What is new?
A new variant of neutrophilic dermatosis, described as an ‘acute necrotizing Sweet's syndrome’, is characterized by the rapid onset of edematous, erythematous, warm cutaneous lesions with deep tissue neutrophilic infiltration and soft tissue necrosis in the absence of an infectious cause .
‘Neutrophilic dermatosis of the dorsal hands’ or ‘pustular vasculitis of the dorsal hands’ refers to Sweet's syndrome lesions located predominantly on the dorsal hands [15–17]. Neutrophilic dermatosis of the hands has been reported following influenza vaccination . Several other patients have also developed Sweet's syndrome (not limited to the hands) after vaccination, including Bacillus Calmette–Guérin vaccine, influenza vaccine, pneumovax vaccine and small pox vaccine .
Sweet's syndrome lesions have also been located on the area of postmastectomy lymphedema in breast cancer patients .
Wolff's isotopic response is a dermatological eponym used to describe the occurrence of a new skin disorder at the site of another unrelated and already healed skin disease. Several manifestions of Sweet's syndrome have presented in this manner: a 59-year-old woman with Sweet's syndrome triggered by scalding , a 56-year-old woman with localized Sweet's syndrome in an irradiated field  and a bullous variant of Sweet's syndrome on the side of the check and neck where a herpes zoster infection previously occurred in a 75-year-old man . A possible relationship between Sweet's syndrome and a henna tattoo has also been reported .
Related physical findings
Common related physical findings include extracutaneous manifestations, associated conditions, associated neutrophilic dermatoses and concurrent leukaemia cutis.
Sites of extracutaneous manifestations of Sweet's syndrome include bones, central nervous system, ears, eyes, kidneys, intestines, liver, heart, lung, mouth, muscles and spleen. Ocular involvement may present in the classic form of Sweet's syndrome. In patients with haematologic disorders, oral mucosal ulcers occur more frequently than in patients with classic Sweet's syndrome [13▪▪,25].
What is new?
Neuro-Sweet's disease is a rare occurrence that can affect regions of the central nervous system, causing a wide variety of neurological symptoms [26–28]. Neuro-Sweet's disease presenting with self-remitting and reversible parkinsonism has been described in a 72-year-old man . Optic nerve involvement of Sweet's syndrome with panuveitis was reported in a 49-year-old man . A 47-year-old Thai woman with underlying Sweet's syndrome demonstrated bilateral endogenous endophthalmitis with chorioretinitis arising from nontuberculous mycobacterial infection .
A 76-year-old man experienced Sweet's syndrome associated pulmonary involvement and systemic inflammatory response syndrome [31▪]. Infection-related systemic inflammatory response syndrome is referred to as sepsis; however, the patient's persistently negative cultures suggested that his systemic inflammatory response syndrome was caused by Sweet's syndrome [31▪].
Sweet's syndrome with panniculitis has been reported in a 45-year-old white woman ; in addition, Rochael et al.[33▪▪] reported findings of panniculitis in 80% of patients in which the hypodermis was affected. MRI findings of dermatosis-associated neutrophilic fasciitis and musculoskeletal involvement were reported in a 62-year-old man with Sweet's syndrome . Sweet's syndrome with spleen and lymph node involvement preceded by parvovirus B19 infection was described in a 45-year-old white woman . A rapid, profound loss of hearing was reported in a 65-year-old woman with a 10-year history of Sweet syndrome; 7 years prior, she had been diagnosed with bilateral, progressive sensorineural hearing loss after a severe exacerbation of Sweet's syndrome . A rare extracutaneous manifestation of Sweet's syndrome with cardiovascular involvement including coronary artery occlusion in a 43-year-old man was recently reported .
Cancers (Figs 1–3), such as haematologic malignancies (most commonly acute myelogenous leukaemia) and solid tumours (most commonly carcinomas of the genitourinary organs, breast, and gastrointestinal tract), infections of the upper respiratory tract (streptococcosis) and the gastrointestinal tract (salmonellosis and yersiniosis), inflammatory bowel disease (Crohn's disease and ulcerative colitis), medications (most commonly granulocyte colony-stimulating factor), pregnancy and vaccinations (BCG and influenza) are conditions that are ‘probably’ related to Sweet's syndrome related. Possibly associated conditions include Behcet's disease, erythema nodosum, relapsing polychondritis, rheumatoid arthritis, sarcoidosis and thyroid disease (Grave's disease and Hashimoto's thyroiditis). Sweet's syndrome has been described in patients with several other conditions such as aortitis (Takayasu's arteritis), aplastic anaemia, bronchiolitis obliterans and organizing pneumonia, chronic granulomatous disease, cutis laxa (acquired, Marshall syndrome), hepatitis C infection, HIV and Sjogren's syndrome. However, the validity of their association in Sweet's syndrome patients remains to be established [2▪,4▪].
What is new?
Recently, the following conditions have been postulated to be associated with Sweet's syndrome: coeliac disease , cervical cancer and tuberculosis , Chlamydophila pneumonia infection leading to tracheo-bronchial involvement , chronic recurrent multifocal osteomyelitis , Fanconi anaemia , kidney transplantation , leptospirosis , microscopic polyangitis , pemphigus vulgaris , sporotrichosis  and systemic lupus erythematosus . Further research is required to demonstrate the legitimacy of each condition's association with Sweet's syndrome.
Sweet's syndrome associated cardiovascular involvement in children has been reported several times in the literature [49▪▪]. Recently, Sweet's syndrome with postinflammatory elastolysis and takayasu arteritis was reported in a 22-month-old white boy [49▪▪]. Three patients, ages 17, 16 and 7 months, developed cardiovascular involvement due to elastolysis of the heart and aorta, which resulted in death in two patients .
Human leukocyte antigen analysis serves as an important differentiator between Sweet's syndrome and Behcet's disease; in Japan, the B54 marker is more frequently associated with Sweet's syndrome, whereas the B51 marker is significantly higher in Behcet's disease .
Associated neutrophilic dermatoses
An inflammatory infiltrate of mature polymorphonuclear leukocytes is the characteristic feature of neutrophilic dermatoses involving the skin and mucosa. Erythema elevatum diutinum, neutrophilic eccrine hidradenitis, pyoderma gangrenosum, subcorneal pustular dermatosis and vasculitis are neutrophilic dermatoses that have been associated – either concurrently or sequentially – with Sweet's syndrome [1,25].
Concurrent leukaemia cutis
In patients with haematologic disorders, Sweet's syndrome may present in three settings: as a paraneoplastic syndrome (underlying an unsuspected malignancy or recurrence of a previously-treated malignancy), as a drug-induced dermatosis or as a skin lesion of the dermatosis, which concurrently demonstrates leukaemia cutis [4▪,25].
Several laboratory findings are required to confirm a diagnosis of Sweet's syndrome.
Sweet's syndrome is characterized by a diffuse infiltrate of mature neutrophils located in the papillary and upper reticular dermis; oedema may also be present. Although Sweet's syndrome is not considered to be a primary leukocytoclastic vasculitis, ‘secondary’ leukocytoclastic vasculitis – in which vasculitis-like changes are observed as an epiphenomenon ‘secondary’ to extensive presence of vascular neutrophils – may be observed in the skin lesions of some patients with Sweet's syndrome. Alteration of the epidermis or neutrophilic infiltration of the adipose tissue can also be observed [33▪▪,52].
What is new?
The presence of eosinophils in Sweet's syndrome may range from rare to abundant and may be unrelated to the use of medication [33▪▪]. A dense neutrophil infiltrate is a characteristic feature of Sweet's syndrome; however, histopathologic discretion must be taken, as this presenting feature may be mimicked by arthropod bites .
’Histiocytoid’ Sweet's syndrome refers to an infiltrate of immature myeloid cells with a histiocytoid appearance, which may be misinterpreted as histiocytes. A case of histiocytoid Sweet's syndrome was reported with neutropenia; similarly, this rare histological variant was also reported in a patient with underlying Hodgkin's lymphoma [54,55]. Histiocytoid Sweet's syndrome has also been reported subcutaneously; a 71-year-old man with myelodysplastic syndrome and refractory anaemia presented with a monocytic-histiocytic appearing infiltrate . Drug-induced histiocytoid Sweet's syndrome in patients receiving bortezomib has also been repeatedly observed . Histiocytoid Sweet's syndrome may pathologically resemble myeloid leukaemia cutis; hence a myeloperoxidase stain can be utilized to differentiate between the microscopically similar appearing conditions .
Other laboratory tests
Peripheral leukocytosis with neutrophilia and an elevated erythrocyte sedimentation rate are the most consistent laboratory abnormalities of Sweet's syndrome. However, these findings may not always be present, such as in oncology patients with anaemia, neutropaenia and/or abnormal platelet counts. Therefore, a complete blood count with leukocyte differential and platelet count, evaluation of acute phase reactants, serum chemistries and a urinalysis should be performed [2▪].
Further testing, less commonly implemented, may be used to confirm extracutaneous manifestations and malignancy in patients with Sweet's syndrome.
Evaluation for extracutaneous manifestations
Evaluation of extracutaneous manifestations of Sweet's syndrome may require other laboratory tests. Central nervous system involvement in patients with Sweet's syndrome may show cerebrospinal fluid abnormalities or changes in the structure or function on brain studies. Kidney and liver involvement in patients with Sweet's syndrome may require urinalysis and hepatic serum enzyme evaluation. Pulmonary involvement in patients with Sweet's syndrome may demonstrate corticosteroid-responsive culture negative infiltrates and pleural effusion on chest roentgrams [4▪,13▪▪].
Newly diagnosed Sweet's syndrome in patients without a prior cancer may be the initial manifestation of a previously undiagnosed malignancy. Age-appropriate workup, as specified by the American Cancer Society, should be considered for the detection of cancer in asymptomatic persons. As the diagnosis of Sweet's syndrome has preceded the associated haematologic malignancy by as long as 11 years, it may be reasonable to repeat the complete blood count with leukocyte differential and platelet count every 6–12 months [4▪,13▪▪].
Clinical and histologic features are considered in generating a differential diagnosis in patients with possible Sweet's syndrome.
Clinical differential diagnosis
Sweet's syndrome skin and mucosal lesions may resemble that of other conditions (Table 1). Cutaneous conditions and systemic diseases, as well as infectious and inflammatory disorders, neoplastic conditions, reactive erythemas and vasculitis should be considered in the clinical differential diagnosis .
What is new?
In addition to familial Mediterranean fever, azathioprine hypersensitivity reaction [59▪▪], chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome  and gemcitabine-associated Sweet syndrome-like eruptions  are recently reported conditions to be considered in the differential diagnosis of Sweet's syndrome.
Histologic differential diagnosis
Conditions characterized by neutrophilic dermatosis or neutrophilic panniculitis should be considered in the histologic differential diagnosis of Sweet's syndrome (Table 1). Lesional tissue culture for bacteria, fungi and mycobacteria should be performed to rule out infection, as pathologic changes associated with Sweet's syndrome are similar to abscess or cellulitis [33▪▪,52]. Sweet's syndrome-like reaction due to arthropod bites may also be considered in the histopathologic differential diagnosis .
Sweet's syndrome skin lesions may become secondarily infected. Reappearance of the dermatosis may signify a recurrence of cancer in malignancy-associated Sweet's syndrome. Sweet's syndrome-associated systemic manifestions – such as inflammatory bowel disease, sarcoidosis and thyroid diseases – may require disease-specific treatment [4▪].
Prognosis and clinical course
In classic Sweet's syndrome, the lesions and symptoms may eventually resolve without therapeutic intervention; however, the lesions can persist for weeks to months. Successful management of the underlying cancer results in subsequent improvement of Sweet's syndrome; likewise, discontinuation of the associated medication in drug-induced Sweet syndrome will result in a spontaneous resolution of the condition. The duration of Sweet's syndrome is variable; indeed, Sweet's syndrome recurrence may occur not only in cancer patients – prompting evaluation for the return of their malignancy – but also in individuals with either idiopathic or disease-associated Sweet's syndrome [2▪,4▪].
Systemic corticosteroids – usually administered orally – are the treatment of choice for Sweet's syndrome. Topical or intralesional corticosteroids have proven to efficaciously treat localized Sweet's syndrome lesions. Intravenous methylprednisone delivered in daily pulses may be used to treat patients with refractory disease [62,63▪].
Colchicine and potassium iodide are also considered to be first-line systemic treatments for Sweet's syndrome. Clofazimine, cyclosporine, dapsone and indomethacin are second-line therapies for Sweet's syndrome. Isolated reports and small studies have also described other effective drugs to treat Sweet's syndrome: chlorambucil, cyclophosphamide, danazol, etretinate, hepatitis therapy, immunoglobulin, interferon-α and tumour necrosis factor antagonists (adalimumab, etanercept, infliximab and thalidomide) [62,63▪].
What is new?
Several new treatment options have been proposed as a therapeutic option for Sweet's syndrome. Intravenous immunoglogulin in conjunction with standard treatment may provide a possible therapeutic role in the treatment of Sweet's syndrome . Anakinra, an anti-interleukin-1 receptor antagonist, has shown promise in the treatment of refractory Sweet's syndrome .
Drugs implicated in development of Sweet's syndrome continue to be observed (Table 2). More recently, the following medications have been associated with the dermatosis: aceclofenac , antiretroviral therapy (efavirenz, lamivudine, lopinavir, ritonavir and stavudine) [33▪▪,67], azacitidine [68,69,70], azathioprine [59▪▪,71,72], benzylthiouracil , bortezomib [57,74–76], carbamazepine , chloroquine , ciprofloxacin , clindamycin , cobalt chrome alloy , cyclooxygenase-2 inhibitor , hydralazine [77,83], interleukin-2 therapy , mitoxantrone , oral contraceptives [33▪▪] orphenadrine citrate [33▪▪] and propylthiouracil . The first report of trimethoprim-sulfamthoxazole induced Sweet's syndrome in a child was described by Khaled et al.. Interestingly, some of the drugs used for the treatment of Sweet's syndrome have also been observed, though rarely, to elicit the condition. These drugs include the tumour necrosis factor inhibitors adalimumab [88▪▪,89], etanercept , infliximab [88▪▪,89] and lenalidomide [91,92].
Sweet's syndrome, initially described by Dr Robert Douglas Sweet nearly 50 years ago as an ‘acute febrile neutrophilic dermatosis’, remains a fascinating dermatological condition with regard to clinical manifestations, laboratory investigation and treatment.
There is no one to acknowledge. There are neither colleagues nor funding bodies nor grants nor sponsorship to acknowledge.
Conflicts of interest
There are no conflicts of interest with regard to this article.
REFERENCES AND RECOMMENDED READING
Papers of particular interest, published within the annual period of review, have been highlighted as:
▪ of special interest
▪▪ of outstanding interest
Additional references related to this topic can also be found in the Current World Literature section in this issue (pp. 68–70).
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Journal of InfectionThe diagnostic yield of skin biopsy in patients with leukemia and suspected infectionJournal of Infection
acute; dermatosis; febrile; neutrophilic; Sweet's syndrome
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