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Sweet Syndrome

Sweet's Syndrome


Presentation

  • OBJECTIVE: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome.[ncbi.nlm.nih.gov]
  • We present a patient with a bullous presentation of Sweet syndrome who developed an aseptic splenic abscess.[ncbi.nlm.nih.gov]
  • Abstract Neutrophilic dermatoses are a spectrum of diseases with varying presentations and similar clinical and histologic features. The association of neutrophilic dermatosis with celiac disease has not previously been described.[ncbi.nlm.nih.gov]
  • Abstract We present a rare case of acute febrile neutrophilic dermatosis, also known as Sweet syndrome, associated with recurrence of metastatic cervical cancer.[ncbi.nlm.nih.gov]
  • OBJECTIVE & METHOD: To present a case highlighting a rare clinical presentation and to review the causes of Sweet syndrome with an emphasis on drug-induced etiologies.[ncbi.nlm.nih.gov]
Fever
  • We describe a case of a woman aged 57 years, with recently diagnosed stage III rectal cancer, developed painful erythematous rash over her face along with fever and headache.[ncbi.nlm.nih.gov]
  • Both diagnoses are characterized by an acute onset of fever, leukocytosis, and tender, erythematous infiltrated plaques.[ncbi.nlm.nih.gov]
  • 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]
  • Sweet's syndrome (or acute febrile neutrophilic dermatosis) is a rare inflammatory disease that is characterized by fever, neutrophilia, and painful erythematous plaques that histologically show a dense dermal infiltrate of neutrophils without associated[ncbi.nlm.nih.gov]
  • Author information 1 Louisiana State University School of Medicine, New Orleans, USA. stephenklinger@hotmail.com Abstract Sweet syndrome is a rare disorder characterized by fever and painful erythematous plaques containing dense dermal neutrophilic infiltrates[ncbi.nlm.nih.gov]
High Fever
  • fever, neutrophilia and typical skin lesions.[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]
  • […] 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]
  • Classical Sweet's syndrome is characterized by high fever, elevated neutrophil count, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils typically located in the upper[histopathology-india.net]
Anemia
  • 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]
  • It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy. Copyright 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • Tolerance of anemia has to be evaluated preoperatively and should require transfusion.[accessanesthesiology.mhmedical.com]
  • Diagnostic Tests: Laboratory testing abnormalities include anemia and elevated ESR, WBC counts, and alkaline phosphatase. Proteinuria is seen in 15%. Positive P-ANCA test results have been reported.[rheumaknowledgy.com]
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]
Papule
  • Abstract Sweet syndrome is a cutaneous lesion characterized by tender, red inflammatory nodules or papules. We describe a pediatric case of Sweet syndrome presenting 10 days after treatment with azathioprine.[ncbi.nlm.nih.gov]
  • Abstract Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by tender, red inflammatory nodules or papules that occur in association with infection, malignancy, connective tissue disease, or following exposure[ncbi.nlm.nih.gov]
  • In addition, erythematous papules and macules were noted on his face, neck, and extremities. Cultures, a biopsy, and laboratory tests yielded a diagnosis of Sweet syndrome.[ncbi.nlm.nih.gov]
  • Herein, we describe an unusual case of Sweet syndrome in a 5-year-old child who suffered from recurrent papules, plaques, and blisters on his face and trunk after the initial onset of fever without an obvious cause. Two skin biopsies were performed.[ncbi.nlm.nih.gov]
  • Abstract A 45-year-old white woman presented with fever, arthalgias, and widespread erythematous papules after a recent Parvovirus B19 infection. Biopsy findings were consistent with classic Sweet syndrome.[ncbi.nlm.nih.gov]
Erythema
  • 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]
  • Abstract A 73-year-old man was admitted to our hospital because of a 2-year history of repeated painful edematous erythemas on the skin and pancytopenia for the last 8 months.[ncbi.nlm.nih.gov]
  • Sweet's syndrome Crohn's disease.gif 935 700; 362 KB Sweet-syndrome skin.png 1 337 586; 574 KB Estratto da " " Categorie : Erythemas Diseases and disorders of the skin Syndromes Rare diseases Categorie nascoste: Uses of Wikidata Infobox with no image[commons.wikimedia.org]
  • 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]
Arthritis
  • It may be associated with hematologic diseases, including leukemia, with immune diseases as rheumatoid arthritis, or can occur in isolation.[ncbi.nlm.nih.gov]
  • 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]
  • Extracutaneous manifestations include alveolitis, sterile osteomyelitis, and arthritis. We present a patient with a bullous presentation of Sweet syndrome who developed an aseptic splenic abscess.[ncbi.nlm.nih.gov]
  • 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]
  • Arthritis tends to parallel skin lesions and usually affects the hands, wrists, ankles, or knees.[rheumaknowledgy.com]
Arthralgia
  • 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]
  • 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]
  • 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]
  • […] with malignancy in multivariate logistic regression analysis were age (OR 1.08 for each increasing year, P 0.01), anaemia (OR 9.38, P 0.001), thrombocytopenia (OR 16.10, P CONCLUSIONS: Patients with older age, anaemia or thrombocytopenia, and without arthralgia[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]

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]
  • All 3 patients were morbidly obese, and workup revealed underlying cancer in 2 cases: myeloma and breast carcinoma. Management of the underlying disease resulted in long-term remission of the skin disease.[ncbi.nlm.nih.gov]
  • Awareness of this diagnosis is important not only to avoid unnecessary medical and surgical therapy and to expediently initiate the administration of steroids for this highly responsive dermatosis, but also to conduct an appropriate workup to exclude[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]
  • Initial workup showed normal complete blood count with hemoglobin level of 90 g/L (normal 16.5-12.5), there was evidence of rouleaux on the peripheral blood smear. There was evidence of hypercalcemia with level up to 2.73 mm/L (normal 2.10-2.55).[jahjournal.org]
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]

Treatment

  • Since azathioprine has historically been known to cause SS, this case highlights a previously unreported treatment response for the histiocytoid variant.[ncbi.nlm.nih.gov]
  • 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]
  • We discuss the different treatment of these diseases because in most cases glucocorticoids, which are the treatment of choice in Sweet's syndrome, may be insufficient. Copyright 2011 Elsevier España, S.L. All rights reserved.[ncbi.nlm.nih.gov]
  • 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. Copyright 2015 AEDV. Published by Elsevier España, S.L.U.[ncbi.nlm.nih.gov]
  • OBJECTIVE: We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • Prognosis The symptoms and lesions of Sweet's syndrome may resolve spontaneously, without any therapeutic intervention; however, recurrence may follow either spontaneous remission or therapy-induced clinical resolution.[orpha.net]
  • Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis. UpToDate, Inc. 2013 Oct 8. Available at: Accessed on: September 10, 2015. Bae YS, Salter SA. Acute Neutrophilic Dermatosis. Emedicine Journal, June 18 2015.[rarediseases.org]
  • Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis. . Accessed Sept. 24, 2015. Merola, JF. Sweet syndrome (acute febrile neutrophilic dermatosis): Pathogenesis, clinical manifestations, and diagnosis. .[mayoclinic.org]
  • Prognosis With or without treatment there is usually complete resolution without any residual scar. There is usually a single episode but, in about a third of cases, it is recurrent. These tend to be associated with malignancy.[patient.info]

Etiology

  • BACKGROUND: Sweet syndrome (SS) is a rare skin condition that is classically idiopathic in etiology, but can also be triggered by malignancy, drug reaction, or infection.[ncbi.nlm.nih.gov]
  • Abstract 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]
  • OBJECTIVE & METHOD: To present a case highlighting a rare clinical presentation and to review the causes of Sweet syndrome with an emphasis on drug-induced etiologies.[ncbi.nlm.nih.gov]
  • Screening for underlying etiologies, including autoimmune disease, malignancy, and offending drugs, is important for targeted therapy.[ncbi.nlm.nih.gov]
  • The disease is divided into several categories based on the underlying etiology, with the drug-induced variant comprising a rising number of the total cases and being reported in association with an increasing number of medications.[ncbi.nlm.nih.gov]

Epidemiology

  • OBJECTIVES: The aim of this study was to evaluate the clinical, epidemiological, laboratory, and histopathological findings and associations of patients with SS.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • Epidemiology It usually occurs in middle-aged women, but men, children and the elderly may also be affected. It is very uncommon. There were six case reports in world literature in 2008-2009.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • CONCLUSION: 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]
  • Less than 35 cases reported with associated multiple myeloma. [5] The pathophysiology has not been clear.[jahjournal.org]

Prevention

  • Early recognition and aggressive therapeutic treatment could prevent fatal cardiac complications.[ncbi.nlm.nih.gov]
  • […] 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]
  • Alongside medication they need education to prevent lesions and help them manage the condition. A lack of information has led many to rely on online information and support-group websites.[nursingtimes.net]

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