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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 1  |  Page : 29-31

Sweet's syndrome in a 1-year-old child


Department of Dermatology Venereology and Leprosy, IGMC, Shimla, Himachal Pradesh, India

Date of Web Publication16-Jan-2015

Correspondence Address:
Renu Rattan
Department of Dermatology Venereology and Leprosy, IGMC, Shimla 171 001, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.149426

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  Abstract 

Sweet's syndrome or acute febrile neutrophilic dermatosis is characterized by tender polymorphous skin lesions, accompanied by fever and peripheral neutrophilia. Although it may be seen at any age, it most commonly occurs in women in the fourth and fifth decade. It is a relatively rare diagnosis in children. We report a 1-year-old boy with Sweet's syndrome following an upper respiratory tract infection.

Keywords: Lesions, neutrophilic dermatosis, Sweet′s syndrome, upper respiratory tract infection


How to cite this article:
Sharma A, Rattan R, Shankar V, Tegta GR, Verma GK. Sweet's syndrome in a 1-year-old child. Indian J Paediatr Dermatol 2015;16:29-31

How to cite this URL:
Sharma A, Rattan R, Shankar V, Tegta GR, Verma GK. Sweet's syndrome in a 1-year-old child. Indian J Paediatr Dermatol [serial online] 2015 [cited 2017 Sep 25];16:29-31. Available from: http://www.ijpd.in/text.asp?2015/16/1/29/149426


  Introduction Top


Sweet's syndrome or acute febrile neutrophilic dermatosis is characterized by fever, blood and tissue neutrophilia, leading to the development of tender, erythematous skin lesions with presence of abundant mature neutrophils on histopathology. [1] It presents in three clinical settings:

  • "Classical" ("para-infectious") Sweet's syndrome, representing a hypersensitivity reaction to preceding infection
  • Malignancy-associated ("para-neoplastic") Sweet's syndrome (in children usually associated with acute myelogenous leukemia)
  • And less frequently as an adverse drug reaction, sometimes in connection with certain underlying diseases (drug-induced Sweet's syndrome). [2] Irrespective of the cause it shows prompt response to systemic corticosteroids.


Sweet's syndrome is rarely diagnosed in children. [3]


  Case report Top


A 1-year-old male child presented with 15 days history of fever and red raised skin lesions on both cheeks, scalp and extremities. There was a history of sore throat around 1 week prior to onset of lesions. There was no history of diarrhea, pain abdomen, loss of weight and appetite, night sweats, recurrent infections or bleeding tendencies. He had been given azithromycin for 3 days before presenting to us. He was febrile (101°F) at the time of presentation. There were multiple, well-defined, erythematous, edematous, shiny plaques of size 1 cm × 1-4 cm × 4 cm on the scalp, cheeks [Figure 1], and both feet. Systemic examination was unremarkable.
Figure 1: Nodulo-plaque lesions over the cheek at the time of presentation

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Investigations showed leukocytosis (white blood cells count = 15000), with neutrophilia (82%). Hb was 9 g/dl. Platelet count was within normal range. The peripheral blood smear revealed mild microcytic hypochromic anemia, but no atypical cells/blasts. Erythrocyte sedimentation rate was raised, C-reactive protein was positive and Antistreptolysin O was negative. Urine routine examination was within the normal limits. His chest X-ray and ultrasound abdomen were normal. Histopathology revealed edema of the papillary dermis along along with dense neutrophilic infiltration [Figure 2]. Based on the clinical and laboratory findings, diagnosis of Sweet's syndrome was established, and he was started on oral prednisolone (1 mg/kg). There was a rapid response with flattening of lesions [Figure 3] within a week. Prednisolone was tapered gradually over the next 8 weeks and then stopped.
Figure 2: Histopathology showing dermal infiltrate

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Figure 3: Flattening of lesions after 4 weeks of therapy

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  Discussion Top


Sweet's syndrome was originally described in 1964. Since then several cases have been reported in the literature, most of them being in adults. It is a relatively rare pediatric diagnosis, with fewer than 70 cases reported in the literature. [3] Pediatric cases account for approximately 5% of all cases. [4]

In adults, there is an overall female preponderance with a female: male ratio of approximately 4:1, [5] whereas in the pediatric population, equal sex distribution has been reported but with a male predominance in children younger than 3 years. [3],[4],[6] Pediatric cases have an average age of onset of 5 years. [6] The youngest reported patient developed lesions at 10 days of age. [7]

Compared with adult disease, pediatric cases are less frequently associated with malignancy and are more likely postinfectious. [8] In the literature, it has been described that cases occurring under the age of 3 years had a male predominance and no association with malignancy, whereas cases over the age of 3 years had an equal sex distribution and were strongly associated with myeloid line hematologic malignancies. [6]

The exact pathogenesis of Sweet's syndrome is still unknown, but it has been suggested that it results from a hypersensitivity reaction to a bacterial, viral or tumor antigen. Most patients have a febrile upper respiratory tract infection (URTI) that precedes their skin lesions by 1-3 weeks. [9]

In our patient, there was a history of sore throat 1 week before the onset of skin lesions and thus URTI was the most likely cause. Investigations to find out other causes were noncontributory. However in a significant group of patients, including children, the syndrome either accompanies or precedes the development of myelogenous leukemia. [10]

Therefore, it is important to keep the possibility of Sweet's syndrome in children presenting with a febrile papulo-nodular or nodulo-plaque lesions. A thorough search for causes should be done and after confirming the diagnosis, investigations to rule out underlying malignancy is warranted.

 
  References Top

1.
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56.  Back to cited text no. 1
[PUBMED]    
2.
Cohen PR. Sweet's syndrome - A comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;2:34.  Back to cited text no. 2
    
3.
Hospach T, von den Driesch P, Dannecker GE. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: Two new patients and review of the literature on associated diseases. Eur J Pediatr 2009;168:1-9.  Back to cited text no. 3
    
4.
Fitzgerald RL, McBurney EI, Nesbitt LT Jr. Sweet's syndrome. Int J Dermatol 1996;35:9-15.  Back to cited text no. 4
    
5.
von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56.  Back to cited text no. 5
    
6.
Halpern J, Salim A. Pediatric sweet syndrome: Case report and literature review. Pediatr Dermatol 2009;26:452-7.  Back to cited text no. 6
    
7.
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;49:132-8.  Back to cited text no. 7
    
8.
Herron MD, Coffin CM, Vanderhooft SL. Sweet syndrome in two children. Pediatr Dermatol 2005;22:525-9.  Back to cited text no. 8
    
9.
Cohen PR, Hönigsmann H, Kurzrock R. Acute febrile neutrophilic dermatosis (Sweet's syndrome). In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ. editors. Fitzpatrick's Dermatology in General Medicine. 7 th ed. New York: McGraw-Hill; 2008. p. 289-95.  Back to cited text no. 9
    
10.
Caughman W, Stern R, Haynes H. Neutrophilic dermatosis of myeloproliferative disorders. Atypical forms of pyoderma gangrenosum and Sweet's syndrome associated with myeloproliferative disorders. J Am Acad Dermatol 1983;9:751-8.  Back to cited text no. 10
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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