European Journal of Pediatrics

, Volume 168, Issue 1, pp 1–9 | Cite as

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases

  • T. Hospach
  • P. von den Driesch
  • G. E. Dannecker



The objectives of this study were to analyse the literature on Sweet’s syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet’s syndrome are reported.


A literature search was performed on Pub med using search terms “sweet* syndrome*” and neutrophil* dermatos*. Patients were subdivided into the following groups: classic/idiopathic, paraneoplastic, and parainflammatory Sweet’s syndrome.


The literature search revealed 64 patients (including our two patients) who were diagnosed with Sweet’s syndrome in childhood and adolescence; 27 (42%) patients were categorized as “classic/idiopathic Sweet’s syndrome”. In 37 patients (58%) chronic associated diseases were reported. Out of these, 21 (33%) patients were categorized as “parainflammatory Sweet’s syndrome” including chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, recurrent infections due to immunodeficiencies, arthritis, and systemic lupus erythematosus. Sixteen (25%) patients were categorized as “paraneoplastic Sweet’s syndrome” comprising both malignant and premalignant diseases like leukemia, aplastic anaemia, and Fanconi anaemia. As all five (8%) patients treated with drugs (granulocyte-colony stimulating factor, retinoid acid) suffered from malignant, premalignant, or parainflammatory diseases, these patients were categorized according to the underlying disease. Two new children with Sweet’s syndrome and associated diseases are presented here, one of them suffering from recurrent infections and trisomy 21, while the other was diagnosed with CNS vasculitis 5 1/2 years after the primary diagnosis.


Sweet’s syndrome should be considered in differential diagnosis of prolonged fever with cutaneous involvement. As most cases of pediatric Sweet’s syndrome are associated with other diseases we suggest careful screening and monitoring of these patients especially concerning malignant/premalignant diseases, immunodeficiencies, cardiovascular involvement, autoimmune diseases, and drug associations.


Sweet’s syndrome Febrile neutrophilic dermatosis 



Aplastic anemia


Acute myelogenous leukemia


Acute lymphoblastic leukemia


All-trans retinoic acid


Bone marrow transplantation


Congenital dyserythropoeitic anemia


Chronic Granulomatous disease


Chronic recurrent multifocal osteomyelitis


Fanconi anemia


Granulocyte colony stimulating factor


Human immunodeficiency virus infection


Juvenile chronic myelogenous leukemia




Myelodysplastic syndrome




Systemic inflammatory response syndrome


  1. 1.
    Al-Saad K, Khanani MF, Naqvi A, Krafchik B, Grant R, Pappo A (2004) Sweet syndrome developing during treatment with all-trans retinoic acid in a child with acute myelogenous leukemia. J Pediatr Hematol Oncol 26:197–199. doi:10.1097/00043426-200403000-00011 PubMedCrossRefGoogle Scholar
  2. 2.
    Astudillo L, Loche F, Reynish W, Rigal-Huguet F, Lamant L, Pris J (2002) Sweet’s syndrome associated with retinoic acid syndrome in a patient with promyelocytic leukemia. Ann Hematol 81:111–114. doi:10.1007/s00277-001-0416-5 PubMedCrossRefGoogle Scholar
  3. 3.
    Attili RV, Hira SK, Dube MK (1983) Sweet’s syndrome in a child (a case report). Med J Zambia 17:92–93PubMedGoogle Scholar
  4. 4.
    Bajwa RP, Marwaha RK, Garewal G, Rajagopalan M (1993) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in myelodysplastic syndrome. Pediatr Hematol Oncol 10:343–346. doi:10.3109/08880019309029513 PubMedCrossRefGoogle Scholar
  5. 5.
    Baron F, Sybert VP, Andrews RG (1989) Cutaneous and extracutaneous neutrophilic infiltrates (Sweet syndrome) in three patients with Fanconi anemia. J Pediatr 115:726–729. doi:10.1016/S0022-3476(89)80649-3 PubMedCrossRefGoogle Scholar
  6. 6.
    Boatman BW, Taylor RC, Klein LE, Cohen BA (1994) Sweet’s syndrome in children. South Med J 87:193–196. doi:10.1097/00007611-199402000-00009 PubMedCrossRefGoogle Scholar
  7. 7.
    Brady RC, Morris J, Connelly BL, Boiko S (1999) Sweet’s syndrome as an initial manifestation of pediatric human immunodeficiency virus infection. Pediatrics 104:1142–1144. doi:10.1542/peds.104.5.1142 PubMedCrossRefGoogle Scholar
  8. 8.
    Burnham JM, Cron RQ (2005) Sweet syndrome as an initial presentation in a child with systemic lupus erythematosus. Lupus 14:974–975. doi:10.1191/0961203305lu2236xx PubMedCrossRefGoogle Scholar
  9. 9.
    Callen JP (2002) Neutrophilic dermatoses. Dermatol Clin 20:409–419. doi:10.1016/S0733-8635(02)00006-2 PubMedCrossRefGoogle Scholar
  10. 10.
    Campos LM, Castellanos AL, Afiune JY, Kiss MH, Silva CA (2005) Takayasu’s arteritis with aortic aneurysm associated with Sweet’s syndrome in childhood. Ann Rheum Dis 64:168–169. doi:10.1136/ard.2003.006353 PubMedCrossRefGoogle Scholar
  11. 11.
    Christensen CC, Gonzalez-Crussi F (1983) Postinflammatory elastolysis and cutis laxa: report of a case with aortitis. Pediatr Pathol 1:199–210. doi:10.3109/15513818309040658 PubMedCrossRefGoogle Scholar
  12. 12.
    Collins P, Rogers S, Keenan P, McCabe M (1991) Acute febrile neutrophilic dermatosis in childhood (Sweet’s syndrome). Br J Dermatol 124:203–206. doi:10.1111/j.1365-2133.1991.tb00435.x PubMedCrossRefGoogle Scholar
  13. 13.
    Dunn TR, Saperstein HW, Biederman A, Kaplan RP (1992) Sweet syndrome in a neonate with aseptic meningitis. Pediatr Dermatol 9:288–292. doi:10.1111/j.1525-1470.1992.tb00350.x PubMedCrossRefGoogle Scholar
  14. 14.
    Edwards TC, Stapleton FB, Bond MJ, Barrett FF (1986) Sweet’s syndrome with multifocal sterile osteomyelitis. Am J Dis Child 140:817–818PubMedGoogle Scholar
  15. 15.
    Eghrari-Sabet JS, Hartley AH (1994) Sweet’s syndrome: an immunologically mediated skin disease. Ann Allergy 72:125–128PubMedGoogle Scholar
  16. 16.
    Elliott SP, Mallory SB (1999) Sweet syndrome: an unusual presentation of chronic granulomatous disease in a child. Pediatr Infect Dis J 18:568–570. doi:10.1097/00006454-199906000-00025 PubMedCrossRefGoogle Scholar
  17. 17.
    Garewal G, Marwaha RK, Ray R, Marwaha N (1993) Clinico-hematological profile and natural history of childhood myelodysplastic syndromes. Indian J Pediatr 60:573–581. doi:10.1007/BF02751436 PubMedCrossRefGoogle Scholar
  18. 18.
    Garty BZ, Levy I, Nitzan M, Barak Y (1996) Sweet syndrome associated with G-CSF treatment in a child with glycogen storage disease type Ib. Pediatrics 97:401–403PubMedGoogle Scholar
  19. 19.
    Goh CL, Alora M, Kohar Y (1996) Sweet’s syndrome in a skin clinic in Singapore: an epidemiology study. Ann Acad Med Singapore 25:222–227PubMedGoogle Scholar
  20. 20.
    Gray LC, Abele DC (1998) Annular erythematous plaques and tibial pain in a child. Sweet syndrome. Arch Dermatol 134:625–626, 628–629. doi:10.1001/archderm.134.5.625-a Google Scholar
  21. 21.
    Guia JM, Frias J, Castro FJ, Gracian M (1999) Cardiovascular involvement in a boy with Sweet’s syndrome. Pediatr Cardiol 20:295–297. doi:10.1007/s002469900468 PubMedCrossRefGoogle Scholar
  22. 22.
    Gyorfy A, Kovacs T, Szegedi I, Olah E, Kiss C (2003) Sweet syndrome associated with 13-cis-retinoic acid (isotretinoin) therapy. Med Pediatr Oncol 40:135–136. doi:10.1002/mpo.10089 PubMedCrossRefGoogle Scholar
  23. 23.
    Haliasos E, Soder B, Rubenstein DS, Henderson W, Morrell DS (2005) Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin. Pediatr Dermatol 22:530–535. doi:10.1111/j.1525-1470.2005.00133.x PubMedCrossRefGoogle Scholar
  24. 24.
    Hassouna L, Nabulsi-Khalil M, Mroueh SM, Zaynoun ST, Kibbi AG (1996) Multiple erythematous tender papules and nodules in an 11-month-old boy. Sweet syndrome (SS) (acute febrile neutrophilic dermatosis). Arch Dermatol 132:1507–1510Google Scholar
  25. 25.
    Havaldar PV, Kumar H, Koppad AM, Bellad R, Mogale KD (1998) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Indian Pediatr 35:1017–1020PubMedGoogle Scholar
  26. 26.
    Hazen PG, Kark EC, Davis BR, Carney JF, Kurczynski E (1983) Acute febrile neutrophilic dermatosis in children. Report of two cases in male infants. Arch Dermatol 119:998–1002. doi:10.1001/archderm.119.12.998 PubMedCrossRefGoogle Scholar
  27. 27.
    Herron MD, Coffin CM, Vanderhooft SL (2005) Sweet syndrome in two children. Pediatr Dermatol 22:525–529. doi:10.1111/j.1525-1470.2005.00132.x PubMedCrossRefGoogle Scholar
  28. 28.
    Heyl TSIW, Cronje RE (1971) Post-inflammatory cutis laxa and aortitis (acquired systemic elastolysis). Br J Dermatol 85:37. doi:10.1111/j.1365-2133.1971.tb14107.x CrossRefGoogle Scholar
  29. 29.
    Itami S, Nishioka K (1980) Sweet’s syndrome in infancy. Br J Dermatol 103:449–451. doi:10.1111/j.1365-2133.1980.tb07270.x PubMedCrossRefGoogle Scholar
  30. 30.
    Jain KK (1996) Sweet’s syndrome associated with granulocyte colony-stimulating factor. Cutis 57:107–110PubMedGoogle Scholar
  31. 31.
    Keller J, Spira I (1986) Sweet syndrome with immune complex vasculitis in a child. Z Hautkr 61:1351–1360Google Scholar
  32. 32.
    Kibbi AG, Zaynoun ST, Kurban AK, Najjar SS (1985) Acute febrile neutrophilic dermatosis (Sweet’s syndrome): case report and review of the literature. Pediatr Dermatol 3:40–44. doi:10.1111/j.1525-1470.1985.tb00484.x PubMedCrossRefGoogle Scholar
  33. 33.
    Klock JC, Oken RL (1976) Febrile neutrophilic dermatosis in acute myelogenous leukemia. Cancer 37:922–927. doi:10.1002/1097-0142(197602)37:2<922::AID-CNCR2820370245>3.0.CO;2-P PubMedCrossRefGoogle Scholar
  34. 34.
    Kourtis AP (2002) Sweet syndrome in infants. Clin Pediatr (Phila) 41:175–177. doi:10.1177/000992280204100308 Google Scholar
  35. 35.
    Krilov LR, Jacobson M, Shende A (1987) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia. Pediatr Infect Dis J 6:77–79. doi:10.1097/00006454-198701000-00025 PubMedCrossRefGoogle Scholar
  36. 36.
    Laissaoui K, Amal S, Hocar O, Benadra F, Alatawna H, Belaabidia B (2005) Sweet’s syndrome in a child. Ann Dermatol Venereol 132:32–34. doi:10.1016/S0151-9638(05)79192-4 Google Scholar
  37. 37.
    Larsson LG, Baum J (1985) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Successful treatment with short term corticosteroids. J Rheumatol 12:1000–1003PubMedGoogle Scholar
  38. 38.
    Lear JT, Atherton MT, Byrne JP (1997) Neutrophilic dermatoses: pyoderma gangrenosum and Sweet’s syndrome. Postgrad Med J 73:65–68PubMedGoogle Scholar
  39. 39.
    Levin DL, Esterly NB, Herman JJ, Boxall LB (1981) The Sweet syndrome in children. J Pediatr 99:73–78. doi:10.1016/S0022-3476(81)80960-2 PubMedCrossRefGoogle Scholar
  40. 40.
    Lipp KE, Shenefelt PD, Nelson RP Jr, Messina JL, Fenske NA (1999) Persistent Sweet’s syndrome occurring in a child with a primary immunodeficiency. J Am Acad Dermatol 40:838–841. doi:10.1053/jd.1999.v40.a95650 PubMedCrossRefGoogle Scholar
  41. 41.
    Litt R, Branski D, Gross-Kieselstein E, Abrahamov A, Rubinow A (1986) Sweet syndrome in early childhood. Eur J Pediatr 145:303–305. doi:10.1007/BF00439405 PubMedCrossRefGoogle Scholar
  42. 42.
    Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H et al (2006) Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med 355:788–798. doi:10.1056/NEJMoa055695 PubMedCrossRefGoogle Scholar
  43. 43.
    Majeed HA, Kalaawi M, Mohanty D, Teebi AS, Tunjekar MF, al-Gharbawy F et al (1989) Congenital dyserythropoietic anemia and chronic recurrent multifocal osteomyelitis in three related children and the association with Sweet syndrome in two siblings. J Pediatr 115:730–734. doi:10.1016/S0022-3476(89)80650-X PubMedCrossRefGoogle Scholar
  44. 44.
    Maleville J, Taieb A (1993) Acute febrile neutrophilic dermatosis in childhood (Sweet syndrome). Pediatr Dermatol 10:298–299. doi:10.1111/j.1525-1470.1993.tb00386.x PubMedCrossRefGoogle Scholar
  45. 45.
    Malone JC, Slone SP, Wills-Frank LA, Fearneyhough PK, Lear SC, Goldsmith LJ et al (2002) Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 138:345–349. doi:10.1001/archderm.138.3.345 PubMedCrossRefGoogle Scholar
  46. 46.
    McDermott MB, Corbally MT, O ’Marcaigh AS (2001) Extracutaneous Sweet syndrome involving the gastrointestinal tract in a patient with Fanconi anemia. J Pediatr Hematol Oncol 23:59–62. doi:10.1097/00043426-200101000-00015 PubMedCrossRefGoogle Scholar
  47. 47.
    Munoz Hoyos A, Loscertales Abril M, Caballero MT, Hernandez Gomez MV, Delgado V, Ruiz Extremera A et al (1983) Sweet’s syndrome in an infant. Pediatrie 38:253–259Google Scholar
  48. 48.
    Muster AJ, Bharati S, Herman JJ, Esterly NB, Gonzales-Crussi F, Holbrook KA (1983) Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis. J Pediatr 102:243–248. doi:10.1016/S0022-3476(83)80529-0 PubMedCrossRefGoogle Scholar
  49. 49.
    Nurre LD, Rabalais GP, Callen JP (1999) Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review. Pediatr Dermatol 16:214–216. doi:10.1046/j.1525-1470.1999.00058.x PubMedCrossRefGoogle Scholar
  50. 50.
    Ohga S, Nomura A, Takada H, Terao H, Harada N, Hara T (2002) Expansion of trisomy 8 and Sweet syndrome in a prolonged course of aplastic anemia. J Pediatr Hematol Oncol 24:64–68. doi:10.1097/00043426-200201000-00017 PubMedCrossRefGoogle Scholar
  51. 51.
    Oshitari K, Hida T, Takahashi M, Okada AA, Shiobara T (2004) Sweet syndrome presenting as orbital cellulitis. Nippon Ganka Gakkai Zasshi 108:162–165Google Scholar
  52. 52.
    Otheo E, Ros P, Vazquez JL, Carrillo R, Moreno R, Maldonado S et al (2002) Systemic inflammatory response syndrome associated with Sweet’s syndrome. Pediatr Crit Care Med 3:190–193. doi:10.1097/00130478-200204000-00020 PubMedCrossRefGoogle Scholar
  53. 53.
    Parsapour K, Reep MD, Gohar K, Shah V, Church A, Shwayder TA (2003) Familial Sweet’s syndrome in 2 brothers, both seen in the first 2 weeks of life. J Am Acad Dermatol 49:132–138. doi:10.1067/mjd.2003.328 PubMedCrossRefGoogle Scholar
  54. 54.
    Rogers M, Greenacre T, Dalla-Pozza L (1987) Neutrophilic dermatosis of myeloproliferative disease in a 10-year-old. Pediatr Dermatol 4:305–312. doi:10.1111/j.1525-1470.1987.tb00798.x PubMedCrossRefGoogle Scholar
  55. 55.
    Saxe N, Gordon W (1978) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Four case reports. S Afr Med J 53:253–256PubMedGoogle Scholar
  56. 56.
    Schneider DT, Schuppe HC, Schwamborn D, Koerholz D, Lehmann P, Goebel U (1998) Acute febrile neutrophilic dermatosis (Sweet syndrome) as initial presentation in a child with acute myelogenous leukemia. Med Pediatr Oncol 31:178–181. doi:10.1002/(SICI)1096-911X(199809)31:3<178::AID-MPO12>3.0.CO;2-T PubMedCrossRefGoogle Scholar
  57. 57.
    Sedel D, Huguet P, Lebbe C, Donadieu J, Odievre M, Labrune P (1994) Sweet syndrome as the presenting manifestation of chronic granulomatous disease in an infant. Pediatr Dermatol 11:237–240. doi:10.1111/j.1525-1470.1994.tb00593.x PubMedCrossRefGoogle Scholar
  58. 58.
    Shimizu T, Yoshida I, Eguchi H, Takahashi K, Inada H, Kato H (1996) Sweet syndrome in a child with aplastic anemia receiving recombinant granulocyte colony-stimulating factor. J Pediatr Hematol Oncol 18:282–284. doi:10.1097/00043426-199608000-00009 PubMedCrossRefGoogle Scholar
  59. 59.
    Sirvent N, Olivier V, Benet L, Maillotte AM, von Bueltzingsloewen A, Mariani R et al (2000) Sweet syndrome in an infant. Arch Pediatr 7:501–504. doi:10.1016/S0929-693X(00)89005-3 Google Scholar
  60. 60.
    Sweet RD (1964) An acute febrile neutrophilic dermatosis. Br J Dermatol 76:349–356. doi:10.1111/j.1365-2133.1964.tb14541.x PubMedCrossRefGoogle Scholar
  61. 61.
    Tuerlinckx D, Bodart E, Despontin K, Boutsen Y, Godding V, Ninane J (1999) Sweet’s syndrome with arthritis in an 8-month-old boy. J Rheumatol 26:440–442PubMedGoogle Scholar
  62. 62.
    Tuncer AM (1988) Acute lymphoblastic leukemia and Sweet’s syndrome. Acta Haematol 80:224PubMedCrossRefGoogle Scholar
  63. 63.
    van Kamp H, van den Berg E, Timens W, Kraaijenbrink RA, Halie MR, Daenen SM (1994) Sweet’s syndrome in myeloid malignancy: a report of two cases. Br J Haematol 86:415–417. doi:10.1111/j.1365-2141.1994.tb04757.x PubMedCrossRefGoogle Scholar
  64. 64.
    von den Driesch P (1994) Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 31:535–560Google Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • T. Hospach
    • 1
  • P. von den Driesch
    • 2
  • G. E. Dannecker
    • 1
  1. 1.Department of Pediatrics and Pediatric RheumatologyKlinikum Stuttgart, OlgahospitalStuttgartGermany
  2. 2.Department of DermatologyKlinikum StuttgartStuttgartGermany

Personalised recommendations