Generic placeholder image

Current Pediatric Reviews

Editor-in-Chief

ISSN (Print): 1573-3963
ISSN (Online): 1875-6336

Review Article

Sweet’s Syndrome: An Update

Author(s): Amit Agrawal*, Salahaldin Hafud Arif, Krithika Kumarasan and Dalwinder Janjua

Volume 18, Issue 4, 2022

Published on: 18 April, 2022

Page: [265 - 273] Pages: 9

DOI: 10.2174/1573396318666220223100208

Price: $65

Abstract

Sweet’s syndrome is a serious dermatological disorder characterized by a rapid onset of tender plaques or nodules, fever, joint pain, headache, and oral and genital lesions. According to the clinical features and underlying causes, Sweet’s syndrome is divided into three categories, i.e., classical (or idiopathic), malignancy-associated Sweet's syndrome, and drug-induced Sweet's syndrome. It is multifactorial in etiology, and the exact cause is still undetermined. The diagnosis can be confirmed by the routine histopathologic evaluation of skin biopsy from the lesions. The first-line treatment options are topical and systemic steroids. Multiple databases, like Medline/PubMed, Scopus, and Google, were used to identify resources for this literature review. The relevant information was collected from various case reports, case series, reviews, meta-analyses, and large clinical trials reporting clinical description, etiology, diagnosis, and management of Sweet’s syndrome. This narrative review aimed to discuss recent understandings related to Sweet's syndrome, both in terms of clinical presentation and management approach.

Keywords: Sweet’s syndrome, dermatosis, drug-induced, dermatological disorder, steroids, papules.

Graphical Abstract

[1]
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964; 76(8-9): 349-56.
[http://dx.doi.org/10.1111/j.1365-2133.1964.tb14541.x] [PMID: 14201182]
[2]
Cohen PR. Sweet’s syndrome-a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007; 2(1): 34.
[http://dx.doi.org/10.1186/1750-1172-2-34] [PMID: 17655751]
[3]
Su WP, Liu HN. Diagnostic criteria for Sweet’s syndrome. Cutis 1986; 37(3): 167-74.
[PMID: 3514153]
[4]
von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31(4): 535-56.
[http://dx.doi.org/10.1016/S0190-9622(94)70215-2] [PMID: 8089280]
[5]
Paydas S. Sweet’s syndrome: A revisit for hematologists and oncologists. Crit Rev Oncol Hematol 2013; 86(1): 85-95.
[http://dx.doi.org/10.1016/j.critrevonc.2012.09.005] [PMID: 23046529]
[6]
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(5 Pt 2): 918-23.
[http://dx.doi.org/10.1016/S0190-9622(96)90080-8] [PMID: 8621829]
[7]
Vashisht P, Bansal P, Goyal A, Hearth Homes MP. Sweet syndrome. Treasure Island, FL: StatPearls Publishing 2021. Updated 2021 Sep 14 Available from: https://www.ncbi.nlm.nih.gov/books/NBK431050/
[8]
Vignon-Pennamen MD. The extracutaneous involvement in the neutrophilic dermatoses. Clin Dermatol 2000; 18(3): 339-47.
[http://dx.doi.org/10.1016/S0738-081X(99)00125-X] [PMID: 10856666]
[9]
Cohen PR, Kurzrock R. Sweet’s syndrome and cancer. Clin Dermatol 1993; 11(1): 149-57.
[http://dx.doi.org/10.1016/0738-081X(93)90112-P] [PMID: 8339190]
[10]
Parsapour K, Reep MD, Gohar K, Shah V, Church A, Shwayder TA. Familial Sweet’s syndrome in 2 brothers, both seen in the first 2 weeks of life. J Am Acad Dermatol 2003; 49(1): 132-8.
[http://dx.doi.org/10.1067/mjd.2003.328] [PMID: 12833027]
[11]
Haverstock C, Libecco JF, Sadeghi P, Maytin E. Tender erythematous plaques in a woman with acute myelogenous leukemia. Arch Dermatol 2006; 142(2): 235-40.
[http://dx.doi.org/10.1001/archderm.142.2.235-b] [PMID: 16490855]
[12]
Disel U, Paydas S, Yavuz S, Tuncer I, Alpay R. Bilateral ear Sweet’s syndrome in a case with relapse acute myeloblastic leukemia. Leuk Res 2006; 30(3): 364.
[http://dx.doi.org/10.1016/j.leukres.2005.08.003] [PMID: 16157374]
[13]
Sudhakar MK, Kallarakkal JT, Damodharan J, Sahib K, Mahajan A, Kannan R. Sweet’s syndrome preceding carcinoma of the adrenal cortex. J Indian Med Assoc 2005; 103(8): 433-5.
[PMID: 16363199]
[14]
Dereure O, Ebrard-Charra S, Guillon F, Baldet P, Guilhou JJ. Sweet’s syndrome associated with pheochromocytoma. Dermatology 2004; 208(2): 175.
[http://dx.doi.org/10.1159/000076499] [PMID: 15057014]
[15]
Culp L, Crowder S, Hatch S. A rare association of Sweet’s syndrome with cervical cancer. Gynecol Oncol 2004; 95(2): 396-9.
[http://dx.doi.org/10.1016/j.ygyno.2004.06.050] [PMID: 15491764]
[16]
Sáez M, García-Bustínduy M, Noda A, et al. Drug-induced Sweet’s syndrome. J Eur Acad Dermatol Venereol 2004; 18(2): 233.
[http://dx.doi.org/10.1111/j.1468-3083.2004.00866.x] [PMID: 15009320]
[17]
Neoh CY, Tan AWH, Ng SK. Sweet’s syndrome: A spectrum of unusual clinical presentations and associations. Br J Dermatol 2007; 156(3): 480-5.
[http://dx.doi.org/10.1111/j.1365-2133.2006.07677.x] [PMID: 17300237]
[18]
Stenzel W, Frosch PJ, Schwarz M. Sweet’s syndrome associated with acute benign encephalitis. A drug induced aetiology. J Neurol 2003; 250(6): 770-1.
[http://dx.doi.org/10.1007/s00415-003-1092-2] [PMID: 12862039]
[19]
Al-Saad K, Khanani MF, Naqvi A, Krafchik B, Grant R, Pappo A. Sweet syndrome developing during treatment with all-trans retinoic acid in a child with acute myelogenous leukemia. J Pediatr Hematol Oncol 2004; 26(3): 197-9.
[http://dx.doi.org/10.1097/00043426-200403000-00011] [PMID: 15125614]
[20]
Govindarajan G, Bashir Q, Kuppuswamy S, Brooks C. Sweet syndrome associated with furosemide. South Med J 2005; 98(5): 570-2.
[http://dx.doi.org/10.1097/01.SMJ.0000157530.63614.9B] [PMID: 15954518]
[21]
Kazlouskaya V, Junkins-Hopkins JM. Lymphocytes in sweet syndrome: A potential diagnostic pitfall. J Cutan Pathol 2018; 45(3): 217-22.
[http://dx.doi.org/10.1111/cup.13096] [PMID: 29265393]
[22]
Marzano AV, Cugno M, Trevisan V, et al. Inflammatory cells, cytokines and matrix metalloproteinases in amicrobial pustulosis of the folds and other neutrophilic dermatoses. Int J Immunopathol Pharmacol 2011; 24(2): 451-60.
[http://dx.doi.org/10.1177/039463201102400218] [PMID: 21658319]
[23]
Meyer V, Schneider SW, Bonsmann G, Beissert S. Experimentally confirmed induction of Sweet’s syndrome by phototesting. Acta Derm Venereol 2011; 91(6): 720-1.
[http://dx.doi.org/10.2340/00015555-1139] [PMID: 21681361]
[24]
Natkunarajah J, Gordon K, Chow J, Sarkany R, Millington GW, Marsden RA. Photoaggravated Sweet’s syndrome. Clin Exp Dermatol 2010; 35(3): e18-9.
[http://dx.doi.org/10.1111/j.1365-2230.2009.03329.x] [PMID: 20500161]
[25]
Touraud JP, Dutronc Y, Tsan P, Collet E, Lambert D. Cutaneous manifestations of Yersinia enterocolitica infection. Ann Dermatol Venereol 2000; 127(8-9): 741-4.
[PMID: 11011167]
[26]
Abramovits W, Stevenson LC. Sweet’s syndrome associated with Staphylococcus aureus. Int J Dermatol 2004; 43(12): 938-41.
[http://dx.doi.org/10.1111/j.1365-4632.2004.02000.x] [PMID: 15569025]
[27]
Evans AV, Sabroe RA, Liddell K, Russell-Jones R. Lymphocytic infiltrates as a presenting feature of Sweet’s syndrome with myelodysplasia and response to cyclophosphamide. Br J Dermatol 2002; 146(6): 1087-90.
[http://dx.doi.org/10.1046/j.1365-2133.2002.04701.x] [PMID: 12072085]
[28]
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(5): 570-4.
[http://dx.doi.org/10.1001/archderm.140.5.570] [PMID: 15148101]
[29]
von den Driesch P, Simon M Jr, Djawari D, Wassmuth R. Analysis of HLA antigens in Caucasian patients with acute febrile neutrophilic dermatosis (Sweet’s syndrome). J Am Acad Dermatol 1997; 37(2 Pt 1): 276-8.
[http://dx.doi.org/10.1016/S0190-9622(97)80142-9] [PMID: 9270521]
[30]
Nesterovitch AB, Gyorfy Z, Hoffman MD, et al. Alteration in the gene encoding protein tyrosine phosphatase nonreceptor type 6 (PTPN6/SHP1) may contribute to neutrophilic dermatoses. Am J Pathol 2011; 178(4): 1434-41.
[http://dx.doi.org/10.1016/j.ajpath.2010.12.035] [PMID: 21406173]
[31]
Cohen PR, Kurzrock R. Sweet’s syndrome: A neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol 2000; 18(3): 265-82.
[http://dx.doi.org/10.1016/S0738-081X(99)00129-7] [PMID: 10856659]
[32]
Bielsa S, Baradad M, Martí RM, Casanova JM. Sweet’s syndrome with bullous lesions. Actas Dermosifiliogr 2005; 96(5): 315-6.
[http://dx.doi.org/10.1016/S0001-7310(05)75062-6] [PMID: 16476395]
[33]
Voelter-Mahlknecht S, Bauer J, Metzler G, Fierlbeck G, Rassner G. Bullous variant of Sweet’s syndrome. Int J Dermatol 2005; 44(11): 946-7.
[http://dx.doi.org/10.1111/j.1365-4632.2004.02287.x] [PMID: 16336530]
[34]
Masmoudi A, Chaaben H, Hamdouni K, et al. Sweet syndrome: Retrospective study of 54 cases. Presse Med 2007; 36(3 Pt 1): 419-24.
[http://dx.doi.org/10.1016/j.lpm.2006.07.010] [PMID: 17321363]
[35]
Marie I, Levesque H, Joly P, et al. Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis. J Am Acad Dermatol 2001; 44(1): 137-9.
[http://dx.doi.org/10.1067/mjd.2001.110903] [PMID: 11148492]
[36]
Jordaan HF. Acute febrile neutrophilic dermatosis. A histopathological study of 37 patients and a review of the literature. Am J Dermatopathol 1989; 11(2): 99-111.
[http://dx.doi.org/10.1097/00000372-198911020-00001] [PMID: 2653084]
[37]
Villarreal-Villarreal CD, Ocampo-Candiani J, Villarreal-Martínez A. Sweet syndrome: A review and update. Actas Dermosifiliogr 2016; 107(5): 369-78.
[http://dx.doi.org/10.1016/j.adengl.2016.05.021] [PMID: 26826881]
[38]
Ghoufi L, Ortonne N, Ingen-Housz-Oro S, et al. Histiocytoid Sweet syndrome is more frequently associated with myelodysplastic syndromes than the classical neutrophilic variant: A comparative series of 62 patients. Medicine (Baltimore) 2016; 95(15): e3033.
[http://dx.doi.org/10.1097/MD.0000000000003033] [PMID: 27082547]
[39]
Christ E, Linka A, Jacky E, Speich R, Marincek B, Schaffner A. Sweet’s syndrome involoving the musculoskeletal system during treatment of promyelocytic leukemia with all-trans retinoic acid. Leukemia 1996; 10(4): 731-4.
[PMID: 8618455]
[40]
Casarin Costa JR, Virgens AR, de Oliveira Mestre L, et al. Sweet syndrome: Clinical features, histopathology, and associations of 83 cases. J Cutan Med Surg 2017; 21(3): 211-6.
[41]
Khan U, Rizvi H, Ali F, Lebovic D. Sweet syndrome: A painful reality. BMJ Case Rep 2016; 2016: bcr2016217606.
[http://dx.doi.org/10.1136/bcr-2016-217606] [PMID: 28011887]
[42]
Silfvast-Kaiser A, Napoli E, Stockton L, Bautista I, Lopez L, Mirkes C. Sweet’s syndrome in a patient with Crohn’s disease. Proc Bayl Univ Med Cent 2018; 31(4): 460-1.
[http://dx.doi.org/10.1080/08998280.2018.1496670] [PMID: 30948979]
[43]
Corbeddu M, Pilloni L, Pau M, Pinna AL, Rongioletti F, Atzori L. Treatment of Sweet’s syndrome in pregnancy. Dermatol Ther 2018; 31(4): e12619.
[http://dx.doi.org/10.1111/dth.12619] [PMID: 30043469]
[44]
Wallach D, Vignon-Pennamen MD. Pyoderma gangrenosum and Sweet syndrome: The prototypic neutrophilic dermatoses. Br J Dermatol 2018; 178(3): 595-602.
[http://dx.doi.org/10.1111/bjd.13955] [PMID: 26202386]
[45]
Wallach D, Vignon-Pennamen MD. From acute febrile neutrophilic dermatosis to neutrophilic disease: Forty years of clinical research. J Am Acad Dermatol 2006; 55(6): 1066-71.
[http://dx.doi.org/10.1016/j.jaad.2006.07.016] [PMID: 17097401]
[46]
Bush JW, Wick MR. Cutaneous histiocytoid Sweet syndrome and its relationship to hematological diseases. J Cutan Pathol 2016; 43(4): 394-9.
[http://dx.doi.org/10.1111/cup.12659] [PMID: 26694973]
[47]
Kazmi SM, Pemmaraju N, Patel KP, et al. Characteristics of Sweet Syndrome in patients with acute myeloid leukemia. Clin Lymphoma Myeloma Leuk 2015; 15(6): 358-63.
[http://dx.doi.org/10.1016/j.clml.2014.12.009] [PMID: 25630528]
[48]
Yang J, Li M. Sweet’s syndrome. BMJ 2018; 360: k1189.
[http://dx.doi.org/10.1136/bmj.k1189]
[49]
Smith RA, Cokkinides V, Eyre HJ. Cancer screening in the United States, 2007: A review of current guidelines, practices, and prospects. CA Cancer J Clin 2007; 57(2): 90-104.
[http://dx.doi.org/10.3322/canjclin.57.2.90] [PMID: 17392386]
[50]
Morgan KW, Callen JP. Sweet’s syndrome in acute myelogenous leukemia presenting as periorbital cellulitis with an infiltrate of leukemic cells. J Am Acad Dermatol 2001; 45(4): 590-5.
[http://dx.doi.org/10.1067/mjd.2001.119032] [PMID: 11568752]
[51]
Cohen PR, Kurzrock R. Sweet Syndrome Fitzpatrick's Dermatology. 9. McGraw Hill 2019. Available from: https://accessmedicine.mhmedical.com/content.aspx?bookid=2570&sectionid=210423071 (Accessed January 02, 2022).
[52]
Magro CM, Crowson AN. A distinctive vesiculopustular eruption associated with hepatobiliary disease. Int J Dermatol 1997; 36(11): 837-44.
[http://dx.doi.org/10.1046/j.1365-4362.1997.00010.x] [PMID: 9427076]
[53]
Cohen PR. Paraneoplastic dermatopathology: Cutaneous paraneoplastic syndromes. Adv Dermatol 1996; 11: 215-52.
[PMID: 8718480]
[54]
Requena L, Sánchez Yus E. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 2001; 45(3): 325-61.
[http://dx.doi.org/10.1067/mjd.2001.114735] [PMID: 11511831]
[55]
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(5): 927-8.
[http://dx.doi.org/10.1016/j.jaad.2005.03.001] [PMID: 15858502]
[56]
Cohen PR, Rapini RP, Beran M. Infiltrated blue-gray plaques in a patient with leukemia. Chloroma (granulocytic sarcoma) Arch Dermatol 1987; 123(2): 251-254, 254.
[http://dx.doi.org/10.1001/archderm.1987.01660260121027] [PMID: 2434033]
[57]
Cohen PR, Kurzrock R. Sweet’s syndrome: A review of current treatment options. Am J Clin Dermatol 2002; 3(2): 117-31.
[http://dx.doi.org/10.2165/00128071-200203020-00005] [PMID: 11893223]
[58]
Drago F, Ciccarese G, Agnoletti AF, Sarocchi F, Parodi A. Neuro sweet syndrome: A systematic review. A rare complication of Sweet syndrome. Acta Neurol Belg 2017; 117(1): 33-42.
[http://dx.doi.org/10.1007/s13760-016-0695-1] [PMID: 27659797]
[59]
Khatri ML, Taha M. Sweet’s syndrome associated with myelodysplastic syndrome presenting as periorbital cellulitis. Int J Dermatol 2007; 46(5): 496-9.
[http://dx.doi.org/10.1111/j.1365-4632.2006.03019.x] [PMID: 17472681]
[60]
Horio T, Imamura S, Danno K, Furukawa F, Ofuji S. Treatment of acute febrile neutrophilic dermatosis (Sweet’s Syndrome) with potassium iodide. Dermatologica 1980; 160(5): 341-7.
[http://dx.doi.org/10.1159/000250516] [PMID: 7364144]
[61]
Tangtatco JAA, Ho N, Drucker A, Forse C. Potassium iodide in refractory, recurrent pediatric Sweet syndrome: Guidance in dosing and monitoring. Pediatr Dermatol 2018; 35(2): 271-3.
[http://dx.doi.org/10.1111/pde.13391] [PMID: 29314271]
[62]
Cohen PR, Kurzrock R. Treatment of Sweet’s syndrome. Am J Med 1990; 89(3): 396.
[http://dx.doi.org/10.1016/0002-9343(90)90362-H] [PMID: 2203266]
[63]
Suehisa S, Tagami H. Treatment of acute febrile neutrophilic dermatosis (Sweet’s syndrome) with colchicine. Br J Dermatol 1981; 105(4): 483.
[http://dx.doi.org/10.1111/j.1365-2133.1981.tb00785.x] [PMID: 7295564]
[64]
Maillard H, Leclech C, Peria P, Avenel-Audran M, Verret JL. Colchicine for Sweet’s syndrome. A study of 20 cases. Br J Dermatol 1999; 140(3): 565-6.
[http://dx.doi.org/10.1046/j.1365-2133.1999.02747.x] [PMID: 10233302]
[65]
Hoffman GS. Treatment of Sweet’s syndrome (active febrile neutrophilic dermatosis) with indomethacin. J Rheumatol 1977; 4(2): 201-6.
[PMID: 881698]
[66]
Jeanfils S, Joly P, Young P, Le Corvaisier-Pieto C, Thomine E, Lauret P. Indomethacin treatment of eighteen patients with Sweet’s syndrome. J Am Acad Dermatol 1997; 36(3 Pt 1): 436-9.
[http://dx.doi.org/10.1016/S0190-9622(97)80222-8] [PMID: 9091476]
[67]
Banet DE, McClave SA, Callen JP. Oral metronidazole, an effective treatment for Sweet’s syndrome in a patient with associated inflammatory bowel disease. J Rheumatol 1994; 21(9): 1766-8.
[PMID: 7799365]
[68]
Amichai B, Lazarov A, Halevy S. Sweet’s syndrome. J Am Acad Dermatol 1995; 33(1): 144-5.
[PMID: 7601939]
[69]
Joshi RK, Atukorala DN, Abanmi A, al Khamis O, Haleem A. Successful treatment of Sweet’s syndrome with doxycycline. Br J Dermatol 1993; 128(5): 584-6.
[http://dx.doi.org/10.1111/j.1365-2133.1993.tb00242.x] [PMID: 8504055]
[70]
Case JD, Smith SZ, Callen JP. The use of pulse methylprednisolone and chlorambucil in the treatment of Sweet’s syndrome. Cutis 1989; 44(2): 125-9.
[PMID: 2758862]
[71]
Singhi EK, Li BC, Denny JC. Simmering below the surface: sweet’s syndrome with multiple myeloma. Am J Med 2018; 131(9): 1058-60.
[http://dx.doi.org/10.1016/j.amjmed.2018.04.014] [PMID: 29729238]
[72]
Haliasos E, Soder B, Rubenstein DS, Henderson W, Morrell DS. Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin. Pediatr Dermatol 2005; 22(6): 530-5.
[http://dx.doi.org/10.1111/j.1525-1470.2005.00133.x] [PMID: 16354255]
[73]
Bianchi L, Masi M, Hagman JH, Piemonte P, Orlandi A. Systemic interferon-alpha treatment for idiopathic Sweet’s syndrome. Clin Exp Dermatol 1999; 24(6): 443-5.
[http://dx.doi.org/10.1046/j.1365-2230.1999.00527.x] [PMID: 10606943]
[74]
Altomare G, Capella GL, Frigerio E. Sweet’s syndrome in a patient with idiopathic myelofibrosis and thymoma-myasthenia gravis-immunodeficiency complex: Efficacy of treatment with etretinate. Haematologica 1996; 81(1): 54-8.
[PMID: 8900854]
[75]
Rahier JF, Lion L, Dewit O, Lambert M. Regression of Sweet’s syndrome associated with Crohn’s disease after anti-Tumour Necrosis Factor therapy. Acta Gastroenterol Belg 2005; 68(3): 376-9.
[PMID: 16268426]
[76]
Kluger N, Gil-Bistes D, Guillot B, Bessis D. Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret®) in a case of refractory Sweet’s syndrome. Dermatology 2011; 222(2): 123-7.
[http://dx.doi.org/10.1159/000326112] [PMID: 21464561]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy