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2.1
Sweet Syndrome
Acute Febrile Neutrophilic Dermatosis

Images

WIKIDATA, CC BY 2.0
WIKIDATA, CC BY 2.0
WIKIDATA, CC BY 2.0

Presentation

A temporal relationship between drug intake, clinical presentation/recurrence, and temporally related resolution of lesions after drug withdrawal is described. [cancertm.com]

Eosinophils were present in 41.1% of the biopsies. In 47.9% of the cases, the infiltration extended as far as the deep dermal layer and 63% presented exocytosis. [scielo.br]

[…] atypical pyoderma gangrenosum when that condition presents on the hands. [thedoctorsdoctor.com]

We present a patient with a bullous presentation of Sweet syndrome who developed an aseptic splenic abscess. [ncbi.nlm.nih.gov]

Entire Body System

  • Fever

    Sweet's syndrome (Acute febrile neutrophilic dermatosis) is a rare skin disorder characrerized by fever, neutrophilic leucocytosis, and erythematous skin nodules. Wolff K, Goldsmith LA, Katz SI, et al: Sweet's Syndrome. [medical-dictionary.thefreedictionary.com]

    Major criteria Abrupt onset of tender or painful red or purplish plaques or nodules Biopsy shows inflammation that is composed mainly of neutrophils without vasculitis Minor criteria Preceding fever or infection Accompanying fever, painful joints, conjunctivitis [dermnetnz.org]

    Larger series of patients showed that fever and neutrophilia are not consistently present. [en.wikipedia.org]

  • Anemia

    Tolerance of anemia has to be evaluated preoperatively and should require transfusion. [accessanesthesiology.mhmedical.com]

    CONCLUSION: When caring for patients with Sweet syndrome, dermatologists should be aware of the potential association of leukopenia, anemia, thrombocytopenia, absence of arthralgia, and histiocytoid or subcutaneous histopathology with malignancy. [ncbi.nlm.nih.gov]

    Premalignant conditions that have been reported in association with pediatric Sweet syndrome include myelodysplastic syndrome, aplastic anemia, and Fanconi anemia. [cancertherapyadvisor.com]

  • Malaise

    A 49-year-old man developed an acute exacerbation of bilateral panuveitis and optic disc edema together with symptoms of a papular rash on his forearms, fevers, malaise, and arthalgias. [ncbi.nlm.nih.gov]

    Eleven days after the second treatment with infliximab, the patient presented with myalgias, subfebrile temperatures, and general malaise. [ard.bmj.com]

    Systemic symptoms include fever, malaise, and joint pains. There is an association with myeloid dysplastic syndromes. Biopsy shows lobular infiltration of neutrophils in the subcutaneous fat without vasculitis. [dermnetnz.org]

    She had malaise and reported that she felt hot but had no known fever. No laboratory tests were ordered. [mdedge.com]

  • Fatigue

    Systemic side effects of PCV13 include chills, fever, headache, vomiting, fatigue, arthralgia, myalgias, decreased appetite, and diarrhea, whereas its cutaneous adverse reactions are local injection site reactions, Sweet's syndrome, and deep morphea. [medical-dictionary.thefreedictionary.com]

    Anyone can get myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). Experts aren’t exactly sure what causes it, but they believe certain people might be at greater risk. [webmd.com]

    A 50-year-old male, with no previous medical illnesses, presented with fatigue, weight loss and arthralgia for several weeks. Clinical examination was unremarkable. [renalfellow.blogspot.com]

    Case report The patient, a 68-year-old man, first showed symptoms in April 2000 with minimum effort dyspnea, accompanied by constitutional symptoms and generalized myalgias with weakness and muscular fatigue. [elsevier.es]

  • High Fever

    […] definition Acute disease characterized by dark-red and slowly growing painful papules and plaques particularly on the face and the extremities accompanied by high fever and neutrophilic leukocytosis in the blood. [dermis.net]

    Sweet syndrome (acute febrile neutrophilic dermatosis) is characterized by a dramatic onset of high fever, neutrophilia and typical skin lesions. About 20 % of patients have an associated malignancy, most commonly hematologic diseases. [ncbi.nlm.nih.gov]

    Signs and Symptoms High fever (>100.4 F) Tender or painful nodules, papules and plaques on the head, neck, legs, and arms. [medrx-education.com]

Jaw & Teeth

  • Aphthous Stomatitis

    Finally, our patients did not have a family history or past evidence for aphthous stomatitis, arthritis, myalgias, serositis, or abdominal symptoms suggestive of a congenital autoinflammatory syndrome. [jamanetwork.com]

    Important diseases for the differential diagnosis are: 107 EAR (recurrent aphthous stomatitis) and aphthae, cyclic neutropenia, viral infections, herpes simplex infection, cytomegalovirus infection, ulcers caused by other sexually transmitted diseases [scielo.br]

Musculoskeletal

  • Arthritis

    Infliximab and methotrexate in the treatment of rheumatoid arthritis. Anti-Tumor Necrosis Factor Trial in Rheumatoid Arthritis with Concomitant Therapy Study. N Engl J Med 2000 ; 343 : 1594 –602. Kraetsch HG, Antoni C, Kalden JR, Manger B. [ard.bmj.com]

    […] accompanied by pustules; it is more common in females; in males, it is associated with cancer including lymphoma, leukaemia, solid tumours and myeloproliferative disorders; it is also associated with ulcerative colitis, monoclonal gammopathies, rheumatoid arthritis [medical-dictionary.thefreedictionary.com]

    Arthritis tends to parallel skin lesions and usually affects the hands, wrists, ankles, or knees. [rheumaknowledgy.com]

    At the time of this presentation, her maintenance medications for rheumatoid arthritis included sulfasalazine and azathioprine; this regimen had been stable for 3 years. [nejm.org]

    Semin Arthritis Rheum 1988 ; 17 : 143 –55. 6 Nolla JM, Juanola X, Valverde J, Roig‐Escofet D, Pagerols X, Servitge O. Arthritis in acute febrile neutrophilic dermatosis (Sweet's syndrome). [rheumatology.oxfordjournals.org]

  • Arthralgia

    Systemic features include fever (>38°C in >80% of patients), malaise, conjunctivitis, episcleritis, iridocyclitis, oral ulcers, proteinuria, arthralgias, myalgias, and arthritis. [rheumaknowledgy.com]

    Systemic side effects of PCV13 include chills, fever, headache, vomiting, fatigue, arthralgia, myalgias, decreased appetite, and diarrhea, whereas its cutaneous adverse reactions are local injection site reactions, Sweet's syndrome, and deep morphea. [medical-dictionary.thefreedictionary.com]

    It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. [ncbi.nlm.nih.gov]

Skin

  • Papule

    An uncommon self-limited (5–12 weeks) condition characterised by single or multiple erythematous papules and plaques ± accompanied by pustules; it is more common in females; in males, it is associated with cancer including lymphoma, leukaemia, solid tumours [medical-dictionary.thefreedictionary.com]

    […] definition Acute disease characterized by dark-red and slowly growing painful papules and plaques particularly on the face and the extremities accompanied by high fever and neutrophilic leukocytosis in the blood. [dermis.net]

    Cutaneous — The cutaneous lesions of Sweet syndrome typically present as tender, edematous, and inflamed papules, plaques, and nodules ( picture 1A-B ). [mulicia.pixnet.net]

    Four months later he presented with fever, constitutional symptoms and skin rash in the form of reddish papules & nodules involving the trunk, neck & face. Lesions got progressively worse and coalesced to form plaques. [renalfellow.blogspot.com]

    Figure 2 Back showing erythematous macules, papules, and some nodules below the neck. A 38-year-old woman developed malaise, fever, chills, bilateral conjunctivitis, arthralgia, and a dry cough. [jaci-inpractice.org]

  • Erythema

    […] pustules; it is more common in females; in males, it is associated with cancer including lymphoma, leukaemia, solid tumours and myeloproliferative disorders; it is also associated with ulcerative colitis, monoclonal gammopathies, rheumatoid arthritis, erythema [medical-dictionary.thefreedictionary.com]

    Desquamation of skin following febrile illness Desquamation secondary to acute systemic illness Drug-induced desquamation of skin Epidermal collarette Erythema elevatum diutinum Erythema fugax Erythema marginatum in acute rheumatic fever Erythema scarlatiniforme [icd9data.com]

    Usual clinical differential diagnosis includes erythema multiforme, drug eruption, and erythema nodosum. Histiocytoid Sweet syndrome is considered an uncommon histopathological variant of the disease. [ncbi.nlm.nih.gov]

    Differential Diagnosis Leukocytoclastic vasculitis Erythema nodusum Drug reaction Erythema multiforme Managements Drug of choice: Systemic glucocorticoids Second-line agents are dapsone, colchicine, or potassium iodide Xtra High Yield Point On the boards [medrx-education.com]

  • Blister

    The histopathological findings of the left arm biopsy showed a subepidermal blister with dense infiltrating neutrophils within the blister that were superficial to the middle layer of the dermis. [ncbi.nlm.nih.gov]

    Small papules (bumps) or vesicles (blisters) Larger thickened or swollen plaques (flat patches) or nodules (lumps) Pseudovesicular appearance (almost blistered) Annular (ring-shaped) lesions Erosions and ulcers resembling atypical pyoderma gangrenosum [dermnetnz.org]

    (C) Blisters show a favourable development towards the healing after to begin Infliximab 5mg/kg. Figure 1. [academic.oup.com]

    A: Blisters and crusted ulcers In the tho racolumbar region. B: Blisters and crusted ulcers in the left arm. Figure 2 Clinical Image. Blisters in right upper extremity. [scielo.conicyt.cl]

  • Skin Rash

    Case Presentations Case 1 A man, aged 48 years, with a history of myelodysplastic syndrome, post-decitabine chemotherapy was admitted with a fever, skin rash, and pancytopenia. [ncbi.nlm.nih.gov]

    Discussion Sweet's syndrome has been described for the 1 st time by Robert Sweet as a case of skin rash, neutrophilic infiltration and fever. [jahjournal.org]

    Four months later he presented with fever, constitutional symptoms and skin rash in the form of reddish papules & nodules involving the trunk, neck & face. Lesions got progressively worse and coalesced to form plaques. [renalfellow.blogspot.com]

  • Skin Plaque

    Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet syndrome. The confused mentality, fever, and erythematous skin plaques resolved after the administration of systemic corticosteroids. [ekjm.org]

    The characteristic dermatologic features of this condition include painful erythematous skin plaques, fever, leukocytosis, and dermal infiltration of neutrophils at skin biopsy. [pubs.rsna.org]

    Sweet's syndrome (acute febrile neutrophilic dermatosis) is an infrequent skin disease characterized by sudden onset of fever, leucocytosis and erythematous plaques or nodules infiltrated by neutrophils. [spandidos-publications.com]

Face, Head & Neck

  • Malar Rash

    rash ANA, anti-Sm/RNP, anti-dsDNA Neutrophilic dermatitis Prednisone Responsive to treatment Gollol-Raju et al., 2009 [4] Female 23 NA Malar rash (lupus nephritis previously) ANA Acute inflammatory cells with overlying keratin debris, abundant neutrophilic [hindawi.com]

Neurologic

  • Headache

    Systemic side effects of PCV13 include chills, fever, headache, vomiting, fatigue, arthralgia, myalgias, decreased appetite, and diarrhea, whereas its cutaneous adverse reactions are local injection site reactions, Sweet's syndrome, and deep morphea. [medical-dictionary.thefreedictionary.com]

    Her course of disease was complicated by arthralgias, episcleritis, headaches, and erythema nodosum-like subcutaneous involvement. There was marked improvement with discontinuation of the inciting antibiotics and initiation of systemic steroids. [ncbi.nlm.nih.gov]

    Additional symptoms may include headache, arthralgia, conjunctivitis, and episcleritis. Febrile upper respiratory tract infection, tonsillitis, or influenza like illness often precede the appearance of skin lesions. [whonamedit.com]

  • Agitation

    […] osteonecrosis, hypocalcemia Gastrointestinal: peptic ulcer disease, bowel perforation, fatty liver changes, esophageal reflux, nausea, vomiting Ocular: cataracts, glaucoma, infections (especially Staphylococcal), refraction changes Psychiatric: psychosis, agitation [cancertherapyadvisor.com]

Workup

Additionally, we discuss the typical presentation of the syndrome, the proper workup and treatment, and a common pitfall encountered in the diagnosis of Sweet syndrome. [ncbi.nlm.nih.gov]

Extensive workup did not find any evidence of an underlying solid tumor or lymphoproliferative disorder. The patient was given prednisone, 1 mg/kg/d, with dose reduction across several weeks. [jamanetwork.com]

Serum

  • Neutrophilia

    […] monoclonal gammopathies, rheumatoid arthritis, erythema nodosum, Sjögren syndrome, lupus erythematosus, vaccinations and various infections Management Systemic steroids Sweet syndrome Febrile neutrophilic dermatosis A rare condition characterized by fever, neutrophilia [medical-dictionary.thefreedictionary.com]

    BACKGROUND: Sweet syndrome (SS) is an infrequent skin disease characterised by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous plaques infiltrated by neutrophils. [ncbi.nlm.nih.gov]

    Although patients with neutrophilia are most likely to be diagnosed with Sweet’s syndrome, so, too, may patients who are neutropenic post-cytotoxic chemotherapy. [bloodjournal.org]

    Our patient presented with systemic upset, fever, neutrophilia and typical cutaneous, and histological features of Sweet syndrome. Surprisingly, IVIG resulted in a prompt and dramatic response in fever and neutrophilia. [hindawi.com]

    Larger series of patients showed that fever and neutrophilia are not consistently present. [en.wikipedia.org]

HLA Type

  • HLA-Bw54

    They found that the frequency of HLA-Bw54 was significantly higher in patients with Sweet syndrome than in controls, while there was no significant difference in the incidence of HLA-Bw54 between patients with Behcet disease and controls. [omim.org]

    Some authors speculate that a genetic predisposition may determine geographical variations in the incidence, since almost half of all reported ca ses have occurred in Japan, finding an association with human leukocyte antigen BW 54 (HLA-Bw54) in these [scielo.conicyt.cl]

    […] syndrome patients received G-CSF/GM-CSF, interferon-γ and all-trans retinoic acid (ATRA) and subsequently developed Sweet's syndrome.11 Although there is no consistent evidence of a genetic predisposition for Sweet's syndrome, a higher frequency of HLA-Bw54 [actasdermo.org]

Treatment

Surgical wound debridement was performed on the fingers and, because of immunosuppressive treatment (glucocorticoids, methotrexate, and azathioprine), systemic antibiotic treatment was started even in the absence of detectable systemic infection. [ard.bmj.com]

Severe ulcerative cases associated with malignancy persist, despite treatment. [dermnetnz.org]

The most common treatment for Sweet's syndrome is corticosteroid pills, such as prednisone. Signs and symptoms often disappear just a few days after treatment begins, but recurrence is common. [mayoclinic.org]

Treatment The treatment of choice in malignancy-associated Sweet’s syndrome is treatment of the underlying malignancy, which can result in complete resolution of the individual’s Sweet’s syndrome ( 2 ). [spandidos-publications.com]

Prognosis

Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis. UpToDate, Inc. 2013 Oct 8. [rarediseases.org]

In general, prognosis is good, but recurrences occur and second-line treatment may be needed. © 2014 The International Society of Dermatology. [ncbi.nlm.nih.gov]

Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis. http://www.uptodate.com/home. Accessed Sept. 24, 2015. Merola, JF. [mayoclinic.org]

Etiology

Sweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. [ncbi.nlm.nih.gov]

Etiology 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. [orpha.net]

Epidemiology

We evaluated clinical, histopathological, and immunohistochemical findings of a case categorized as idiopathic histiocytoid Sweet syndrome in which clinical-epidemiological data raised the possibilities of Sweet syndrome, leprosy, and drug reaction. [ncbi.nlm.nih.gov]

Synonym: acute febrile neutrophilic dermatosis Epidemiology Manifests at any age, classic peaks at 30 to 60 years of age Often based on subtype Classic: 80% are women; 75–90% have a prior upper respiratory infection (URI); 30% recurrence Drug-induced: [unboundmedicine.com]

CONCLUSION: The clinical and epidemiological data found in our study are mostly similar to those found in the literature. [scielo.br]

Summary Epidemiology Several hundred cases of Sweet's syndrome have been published. Clinical description Sweet's syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. [orpha.net]

Pathophysiology

The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease. [ncbi.nlm.nih.gov]

Etiology and Pathophysiology Pathogenesis is multifactorial. Theorized factors include hypersensitivity reactions, cytokine dysregulation, and infection. [unboundmedicine.com]

In this report, we will discuss pathophysiology, diagnosis and challenges in the treatment associated with malignancy-associated Sweet’s syndrome. [spandidos-publications.com]

Prevention

[…] facilitating the early diagnosis and appropriate management of unique conditions such as necrotizing Sweet syndrome, anesthesiologists can not only play a more visible role as leaders in the emerging perioperative surgical home model, but they may also prevent [ncbi.nlm.nih.gov]

Taking good care of your skin by protecting it from the sun may also help to prevent it from returning. [healthline.com]

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