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Sweet syndrome (acute febrile neutrophilic dermatosis): Pathogenesis, clinical manifestations, and diagnosis

Joseph F Merola, MD, MMSc, FAAD, FACR
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Sweet syndrome (acute febrile neutrophilic dermatosis) is an uncommon inflammatory disorder characterized by the abrupt appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin. Fever and leukocytosis frequently accompany the cutaneous lesions. In addition, involvement of the eyes, musculoskeletal system, and internal organs may occur.

The epidemiology, clinical features, and diagnosis of Sweet syndrome will be discussed here. Information on the treatment and prognosis of Sweet syndrome and an overview of other neutrophilic dermatoses are available separately. (See "Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis" and "Neutrophilic dermatoses".)


Sweet syndrome was first described by Dr. Robert Douglas Sweet in 1964, who documented the development of an acute inflammatory skin eruption with fever and leukocytosis in eight women, several of whom had preceding upper respiratory or gastrointestinal infections [1]. Since then, Sweet syndrome has been observed in association with a broad range of disorders. As a result, some authors divide Sweet syndrome into three subtypes based upon etiology:

Classical Sweet syndrome

Malignancy-associated Sweet syndrome


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Literature review current through: Sep 2016. | This topic last updated: Feb 12, 2015.
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  2. Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007; 2:34.
  3. Lallas A, Tzellos TG, Papageorgiou M, Mandekou-Lefaki I. Sweet's syndrome associated with upper respiratory tract streptococcal infection: "wait-and-see" strategy or anecdotal use of corticosteroids? Hippokratia 2011; 15:283.
  4. Ytting H, Vind I, Bang D, Munkholm P. Sweet's syndrome--an extraintestinal manifestation in inflammatory bowel disease. Digestion 2005; 72:195.
  5. Satra K, Zalka A, Cohen PR, Grossman ME. Sweet's syndrome and pregnancy. J Am Acad Dermatol 1994; 30:297.
  6. Cohen PR, Hongsmann H, Kurzrock R. Acute febrile neutrophilic dermatosis (Sweet syndrome). In: Fitzpatrick's Dermatology in General Medicine, 8th ed, Goldsmith LA, Katz SI, Gilchrest BA, et al.. (Eds), McGraw Hill, 2012. Vol 1, p.362.
  7. Gray PE, Bock V, Ziegler DS, Wargon O. Neonatal Sweet syndrome: a potential marker of serious systemic illness. Pediatrics 2012; 129:e1353.
  8. Cohen PR, Kurzrock R. Sweet's syndrome and cancer. Clin Dermatol 1993; 11:149.
  9. Halpern J, Salim A. Pediatric sweet syndrome: case report and literature review. Pediatr Dermatol 2009; 26:452.
  10. Bourke JF, Keohane S, Long CC, et al. Sweet's syndrome and malignancy in the U.K. Br J Dermatol 1997; 137:609.
  11. Raza S, Kirkland RS, Patel AA, et al. Insight into Sweet's syndrome and associated-malignancy: a review of the current literature. Int J Oncol 2013; 42:1516.
  12. Uihlein LC, Brandling-Bennett HA, Lio PA, Liang MG. Sweet syndrome in children. Pediatr Dermatol 2012; 29:38.
  13. Krilov LR, Jacobson M, Shende A. Acute febrile neutrophilic dermatosis (Sweet's syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia. Pediatr Infect Dis J 1987; 6:77.
  14. Miranda CV, Filgueiras Fde M, Obadia DL, et al. Sweet's Syndrome associated with Hodgkin's disease: case report. An Bras Dermatol 2011; 86:1016.
  15. Wawrzycki B, Chodorowska G, Pietrzak A, et al. Recurrent skin eruption in patient with chronic lymphocytic leukemia and lymphocytic infiltrates of the dermis resembling Sweet's syndrome. G Ital Dermatol Venereol 2011; 146:487.
  16. Ravaglia C, Poletti G, Gurioli C, et al. Sweet's syndrome associated with myelogenous leukemia and pulmonary involvement. Monaldi Arch Chest Dis 2011; 75:149.
  17. Shugarman IL, Schmit JM, Sbicca JA, Wirk B. Easily missed extracutaneous manifestation of malignancy-associated Sweet's syndrome: systemic inflammatory response syndrome. J Clin Oncol 2011; 29:e702.
  18. Kato T, Kawana S, Takezaki S, et al. Case of Sweet's syndrome with extensive necrosis and ulcers accompanied by myelodysplastic syndrome. J Nippon Med Sch 2008; 75:162.
  19. Glendenning J, Khoo V. Sweet's syndrome in prostate cancer. Prostate Cancer Prostatic Dis 2008; 11:397.
  20. Awan F, Hamadani M, Devine S. Paraneoplastic Sweet's syndrome and the pathergy phenomenon. Ann Hematol 2007; 86:613.
  21. Culp L, Crowder S, Hatch S. A rare association of Sweet's syndrome with cervical cancer. Gynecol Oncol 2004; 95:396.
  22. Dereure O, Ebrard-Charra S, Guillon F, et al. Sweet's syndrome associated with pheochromocytoma. Dermatology 2004; 208:175.
  23. Liu D, Seiter K, Mathews T, et al. Sweet's syndrome with CML cell infiltration of the skin in a patient with chronic-phase CML while taking Imatinib Mesylate. Leuk Res 2004; 28 Suppl 1:S61.
  24. Gille J, Spieth K, Kaufmann R. Sweet's syndrome as initial presentation of diffuse large B-cell lymphoma. J Am Acad Dermatol 2002; 46:S11.
  25. Zappasodi P, Corso A, del Forno C. Sweet's syndrome and myelodysplasia: two entities with a common pathogenetic mechanism? A case report. Haematologica 2000; 85:868.
  26. Suvajdzić N, Dimcić Z, Cvijetić O, Colović M. Sweet's syndrome associated with Hodgkin's disease. Haematologia (Budap) 1998; 29:157.
  27. Aubin F, Dufour MP, Angonin R, et al. Sweet's syndrome associated with cutaneous T cell lymphoma. Eur J Dermatol 1998; 8:178.
  28. Cho KH, Han KH, Kim SW, et al. Neutrophilic dermatoses associated with myeloid malignancy. Clin Exp Dermatol 1997; 22:269.
  29. Lear JT, Byrne JP. Bullous pyoderma gangrenosum, Sweet's syndrome and malignancy. Br J Dermatol 1997; 136:296.
  30. Deguchi M, Tsunoda T, Yuda F, Tagami H. Sweet's syndrome in acute myelogenous leukemia showing dermal infiltration of leukemic cells. Dermatology 1997; 194:182.
  31. Woodrow SL, Munn SE, Basarab T, Russel Jones R. Sweet's syndrome in association with non-Hodgkin's lymphoma. Clin Exp Dermatol 1996; 21:357.
  32. Colović MD, Janković GM, Novak AZ, et al. Sweet's syndrome associated with paracentric inversion of chromosome 3q in a patient with multiple myeloma. Eur J Haematol 1996; 57:188.
  33. Heer-Sonderhoff AH, Arning M, Wehmeier A, et al. Neutrophilic dermal infiltrates in granulocytopenic patients with acute leukemia. Ann Hematol 1995; 71:257.
  34. Shirono K, Kiyofuji C, Tsuda H. Sweet's syndrome in a patient with acute promyelocytic leukemia during treatment with all-trans retinoic acid. Int J Hematol 1995; 62:183.
  35. Yamamoto T, Furuse Y, Nishioka K. Sweet's syndrome with small cell carcinoma of the lung. J Dermatol 1994; 21:125.
  36. van Kamp H, van den Berg E, Timens W, et al. Sweet's syndrome in myeloid malignancy: a report of two cases. Br J Haematol 1994; 86:415.
  37. Inanç SE, Altum M, Onat H, Erseven G. Sweet's syndrome and Hodgkin's disease. Acta Oncol 1994; 33:574.
  38. Barnadas MA, Sitjàs D, Brunet S, et al. Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with prostate adenocarcinoma and a myelodysplastic syndrome. Int J Dermatol 1992; 31:647.
  39. Kramers C, Raemaekers JM, van Baar HM, et al. Sweet's syndrome as the presenting symptom of hairy cell leukemia with concomitant infection by Mycobacterium kansasii. Ann Hematol 1992; 65:55.
  40. Torralbo A, Herrero JA, del-Rio E, et al. Sweet's syndrome associated with multiple myeloma. Int J Dermatol 1992; 31:297.
  41. Uchida H, Ikari Y, Hashizume S, et al. A case of Sweet's syndrome with early gastric cancer. Dermatologica 1990; 181:224.
  42. Fischer G, Commens C, Bradstock K. Sweet's syndrome in hairy cell leukemia. J Am Acad Dermatol 1989; 21:573.
  43. Tuncer AM. Acute lymphoblastic leukemia and Sweet's syndrome. Acta Haematol 1988; 80:224.
  44. Visani G, Patrizi AL, Ricci P, et al. Sweet's syndrome: association with accelerated phase of chronic myeloid leukemia. Acta Haematol 1988; 79:207.
  45. Nguyen KQ, Hurst CG, Pierson DL, Rodman OG. Sweet's syndrome and ovarian carcinoma. Cutis 1983; 32:152.
  46. Rochet NM, Chavan RN, Cappel MA, et al. Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients. J Am Acad Dermatol 2013; 69:557.
  47. Cohen PR, Talpaz M, Kurzrock R. Malignancy-associated Sweet's syndrome: review of the world literature. J Clin Oncol 1988; 6:1887.
  48. Cohen PR, Holder WR, Tucker SB, et al. Sweet syndrome in patients with solid tumors. Cancer 1993; 72:2723.
  49. Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. J Am Acad Dermatol 1996; 34:918.
  50. Su WP, Liu HN. Diagnostic criteria for Sweet's syndrome. Cutis 1986; 37:167.
  51. Magro CM, De Moraes E, Burns F. Sweet's syndrome in the setting of CD34-positive acute myelogenous leukemia treated with granulocyte colony stimulating factor: evidence for a clonal neutrophilic dermatosis. J Cutan Pathol 2001; 28:90.
  52. White JM, Mufti GJ, Salisbury JR, du Vivier AW. Cutaneous manifestations of granulocyte colony-stimulating factor. Clin Exp Dermatol 2006; 31:206.
  53. Treton X, Joly F, Alves A, et al. Azathioprine-induced Sweet's syndrome in Crohn's disease. Inflamm Bowel Dis 2008; 14:1757.
  54. Juanola X, Nolla JM, Servitje O, Valverde J. Hydralazine induced lupus and Sweet's syndrome. J Rheumatol 1991; 18:948.
  55. Thibault MJ, Billick RC, Srolovitz H. Minocycline-induced Sweet's syndrome. J Am Acad Dermatol 1992; 27:801.
  56. Mensing H, Kowalzick L. Acute febrile neutrophilic dermatosis (Sweet's syndrome) caused by minocycline. Dermatologica 1991; 182:43.
  57. Park CJ, Bae YD, Choi JY, et al. Sweet's syndrome during the treatment of acute promyelocytic leukemia with all-trans retinoic acid. Korean J Intern Med 2001; 16:218.
  58. Arun B, Berberian B, Azumi N, et al. Sweet's syndrome during treatment with all-trans retinoic acid in a patient with acute promyelocytic leukemia. Leuk Lymphoma 1998; 31:613.
  59. Kyllo RL, Parker MK, Rosman I, Musiek AC. Ipilimumab-associated Sweet syndrome in a patient with high-risk melanoma. J Am Acad Dermatol 2014; 70:e85.
  60. Yorio JT, Mays SR, Ciurea AM, et al. Case of vemurafenib-induced Sweet's syndrome. J Dermatol 2014; 41:817.
  61. Pattanaprichakul P, Tetzlaff MT, Lapolla WJ, et al. Sweet syndrome following vemurafenib therapy for recurrent cholangiocarcinoma. J Cutan Pathol 2014; 41:326.
  62. Dickson EL, Bakhru A, Chan MP. Topotecan-induced Sweet's syndrome: A case report. Gynecol Oncol Case Rep 2013; 4:50.
  63. Zobniw CM, Saad SA, Kostoff D, Barthel BG. Bortezomib-induced Sweet's syndrome confirmed by rechallenge. Pharmacotherapy 2014; 34:e18.
  64. Pintova S, Sidhu H, Friedlander PA, Holcombe RF. Sweet's syndrome in a patient with metastatic melanoma after ipilimumab therapy. Melanoma Res 2013; 23:498.
  65. Kolb-Mäurer A, Kneitz H, Goebeler M. Sweet-like syndrome induced by bortezomib. J Dtsch Dermatol Ges 2013; 11:1200.
  66. Kim MJ, Choe YH. EPONYM. Sweet syndrome. Eur J Pediatr 2010; 169:1439.
  67. Voelter-Mahlknecht S, Bauer J, Metzler G, et al. Bullous variant of Sweet's syndrome. Int J Dermatol 2005; 44:946.
  68. Escallier F, Gaudard S, Courtois JM, et al. [Sweet's syndrome and Yersinia enterocolitica infection]. Ann Dermatol Venereol 1990; 117:858.
  69. O'Connor Reina C, Garcia Iriarte MT, Rodriguez Diaz A, et al. Tonsil cancer and Sweet's syndrome. Otolaryngol Head Neck Surg 1998; 119:709.
  70. Kawakami T, Ohashi S, Kawa Y, et al. Elevated serum granulocyte colony-stimulating factor levels in patients with active phase of sweet syndrome and patients with active behcet disease: implication in neutrophil apoptosis dysfunction. Arch Dermatol 2004; 140:570.
  71. Shinojima Y, Toma Y, Terui T. Sweet syndrome associated with intrahepatic cholangiocarcinoma producing granulocyte colony-stimulating factor. Br J Dermatol 2006; 155:1103.
  72. Tanaka N, Fujioka A, Tajima S, et al. Elafin is induced in epidermis in skin disorders with dermal neutrophilic infiltration: interleukin-1 beta and tumour necrosis factor-alpha stimulate its secretion in vitro. Br J Dermatol 2000; 143:728.
  73. Reuss-Borst MA, Müller CA, Waller HD. The possible role of G-CSF in the pathogenesis of Sweet's syndrome. Leuk Lymphoma 1994; 15:261.
  74. Loraas A, Waage A, Lamvik J. Cytokine response pattern in Sweet's syndrome associated with myelodysplasia. Br J Haematol 1994; 87:669.
  75. Reuss-Borst MA, Pawelec G, Saal JG, et al. Sweet's syndrome associated with myelodysplasia: possible role of cytokines in the pathogenesis of the disease. Br J Haematol 1993; 84:356.
  76. Giasuddin AS, El-Orfi AH, Ziu MM, El-Barnawi NY. Sweet's syndrome: is the pathogenesis mediated by helper T cell type 1 cytokines? J Am Acad Dermatol 1998; 39:940.
  77. Mijovic A, Novak A, Medenica L. Sweet's syndrome associated with inversion of chromosome 3q in a patient with refractory anemia. Eur J Haematol 1992; 49:156.
  78. Billström R, Heim S, Kristoffersson U, et al. Structural chromosomal abnormalities of 3q in myelodysplastic syndrome/acute myeloid leukaemia with Sweet's syndrome. Eur J Haematol 1990; 45:150.
  79. Kemmett D, Harrison DJ, Hunter JA. Antibodies to neutrophil cytoplasmic antigens: serologic marker for Sweet's syndrome. J Am Acad Dermatol 1991; 24:967.
  80. Takahama H, Kanbe T. Neutrophilic dermatosis of the dorsal hands: a case showing HLA B54, the marker of Sweet's syndrome. Int J Dermatol 2010; 49:1079.
  81. Mizoguchi M, Matsuki K, Mochizuki M, et al. Human leukocyte antigen in Sweet's syndrome and its relationship to Behçet's disease. Arch Dermatol 1988; 124:1069.
  82. Jo T, Horio K, Migita K. Sweet's syndrome in patients with MDS and MEFV mutations. N Engl J Med 2015; 372:686.
  83. von den Driesch P, Weber MF. Are antibodies to neutrophilic cytoplasmic antigens (ANCA) a serologic marker for Sweet's syndrome? J Am Acad Dermatol 1993; 29:666.
  84. Moschella SL, Davis M. Neutrophilic dermatoses. In: Dermatology, Bolognia JL, Jorizzo JL, Rapini RP. (Eds), Elsevier, 2008. Vol 1, p.379.
  85. Guhl G, García-Díez A. Subcutaneous sweet syndrome. Dermatol Clin 2008; 26:541.
  86. Cohen PR. Subcutaneous Sweet's syndrome: a variant of acute febrile neutrophilic dermatosis that is included in the histopathologic differential diagnosis of neutrophilic panniculitis. J Am Acad Dermatol 2005; 52:927.
  87. Walling HW, Snipes CJ, Gerami P, Piette WW. The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Arch Dermatol 2006; 142:57.
  88. Kroshinsky D, Alloo A, Rothschild B, et al. Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis. J Am Acad Dermatol 2012; 67:945.
  89. Surovy AM, Pelivani N, Hegyi I, et al. Giant cellulitis-like Sweet Syndrome, a new variant of neutrophilic dermatosis. JAMA Dermatol 2013; 149:79.
  90. Bamelis M, Boyden B, Sente F, Madoe V. Sweet's syndrome and acute myelogenous leukemia in a patient who presented with a sudden massive swelling of the tongue. Dermatology 1995; 190:335.
  91. Gottlieb CC, Mishra A, Belliveau D, et al. Ocular involvement in acute febrile neutrophilic dermatosis (Sweet syndrome): new cases and review of the literature. Surv Ophthalmol 2008; 53:219.
  92. Medenblik-Frysch S, von den Driesch P, Jonas JB, Meythaler FH. Ocular complications in Sweet's syndrome. Am J Ophthalmol 1992; 114:230.
  93. Vanourny J, Swick BL. Sweet syndrome with systemic inflammatory response syndrome. Arch Dermatol 2012; 148:969.
  94. Sawicki J, Morton RA, Ellis AK. Sweet syndrome with associated systemic inflammatory response syndrome: an ultimately fatal case. Ann Allergy Asthma Immunol 2010; 105:321.
  95. Otheo E, Ros P, Vázquez JL, et al. Systemic inflammatory response syndrome associated with Sweet's syndrome. Pediatr Crit Care Med 2002; 3:190.
  96. von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31:535.
  97. Weedon D. The vasculopathic reaction pattern. In: Weedon's Skin Pathology, 3rd ed, Elsevier Limited, 2010. p.195.
  98. Malone JC, Slone SP, Wills-Frank LA, et al. Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 2002; 138:345.
  99. Lin J, Zhang Q, Chen M. Subcutaneous histiocytoid Sweet's syndrome in a patient associated with myelodysplastic syndrome-refractory anemia. J Dermatol 2012; 39:99.
  100. Kaiser R, Connolly K, Linker C, et al. Stem cell transplant for myelodysplastic syndrome-associated histiocytoid Sweet's syndrome in a patient with arthritis and myalgias. Arthritis Rheum 2008; 59:1832.
  101. Spencer B, Nanavati A, Greene J, Butler DF. Dapsone-responsive histiocytoid Sweet's syndrome associated with Crohn's disease. J Am Acad Dermatol 2008; 59:S58.
  102. Wu AJ, Rodgers T, Fullen DR. Drug-associated histiocytoid Sweet's syndrome: a true neutrophilic maturation arrest variant. J Cutan Pathol 2008; 35:220.
  103. Chow S, Pasternak S, Green P, et al. Histiocytoid neutrophilic dermatoses and panniculitides: variations on a theme. Am J Dermatopathol 2007; 29:334.
  104. Requena L, Kutzner H, Palmedo G, et al. Histiocytoid Sweet syndrome: a dermal infiltration of immature neutrophilic granulocytes. Arch Dermatol 2005; 141:834.
  105. Chavan RN, Cappel MA, Ketterling RP, et al. Histiocytoid Sweet syndrome may indicate leukemia cutis: a novel application of fluorescence in situ hybridization. J Am Acad Dermatol 2014; 70:1021.
  106. Wasson S, Govindarajan G, Folzenlogen D. Concurrent occurrence of Sweet's syndrome and erythema nodosum: an overlap in the spectrum of reactive dermatoses. Clin Rheumatol 2006; 25:268.
  107. Titulaer MJ, Soffietti R, Dalmau J, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol 2011; 18:19.
  108. Gainon J, Bart PA, Waeber G. [Can we predict the risk of malignancy associated with dermatomyositis?]. Praxis (Bern 1994) 2003; 92:1734.