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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 3  |  Page : 179-181

Sweet’s syndrome in the pediatric population: Two case reports


Department of Dermatology, Venereology, Leprosy, Rajah Muthiah Medical College and Hospital, Chidambaram, Tamil Nadu, India

Date of Web Publication10-Jul-2015

Correspondence Address:
P Swetha
Department of Dermatology, Venereology, Leprosy, Rajah Muthiah Medical College and Hospital, Chidambaram - 608 002, Tamil Nadu
India
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Source of Support: Nil, Conflict of Interest: None declared.


DOI: 10.4103/2319-7250.160668

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  Abstract 

Sweet’s syndrome (SS) is characterized by a constellation of clinical symptoms and physical findings, which include fever, blood, and tissue neutrophilia, leading to the development of tender, erythematous plaques, histopathologically characterized by the presence of abundant mature neutrophils. Here, we present two cases of SS. First, a 4-year-old girl, who presented with fever, features of upper respiratory infection, abdominal pain, and multiple skin lesions of 10 days duration. She had similar illness 6 months ago. Second, an 8-month-old male baby who presented with fever and multiple skin lesions of 20 days duration. On examination, both children had papules and plaques with elevated borders all over the body. Laboratory investigations revealed raised inflammatory markers with neutrophilic predominance. Skin biopsy of the lesions showed dense neutrophilic infiltration of the dermis. In view of recurrent SS of the first case, underlying malignancy was ruled out. All these fit into the diagnostic criteria of SS classical type probably para-infectious etiology. These case reports bring to light that SS should be borne in mind when dealing with children with fever of unknown etiology with tender plaques, blood, and tissue neutrophilia.

Keywords: Neutrophilic dermatoses, recurrent Sweet’s syndrome, Sweet’s syndrome


How to cite this article:
Swetha P, Prasad P, Kaviarasan P. Sweet’s syndrome in the pediatric population: Two case reports. Indian J Paediatr Dermatol 2015;16:179-81

How to cite this URL:
Swetha P, Prasad P, Kaviarasan P. Sweet’s syndrome in the pediatric population: Two case reports. Indian J Paediatr Dermatol [serial online] 2015 [cited 2019 Dec 13];16:179-81. Available from: http://www.ijpd.in/text.asp?2015/16/3/179/160668


  Introduction Top


Sweet’s syndrome (SS) is also known as acute febrile neutrophilic dermatosis first described by Robert Douglas Sweet in 1964.[1] SS is clinically characterized by fever, blood, and tissue neutrophilia, leading to the development of tender, erythematous inflammatory skin lesions (papules, nodules, plaques) and histopathologically by the presence of abundant mature neutrophils.[2] It presents in three clinical settings: “Classical” (para-infectious) SS, representing a hypersensitivity reaction preceding infection; malignancy-associated (para-neoplastic) SS (in children usually associated with acute myelogenous leukemia); and as adverse drug reaction, sometimes in connection with certain underlying diseases (drug induced SS).[3]

Diagnostic criteria for SS were proposed by Su and Liu in 1986 and modified by von den Driesch in 1994[4] [Table 1].
Table 1: Diagnostic criteria for sweet syndrome[4]

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Histologically, the disease is characterized by the presence of a dense dermal neutrophilic inflammatory infiltrate associated with subepidermal edema of variable intensity and nuclear dust which was seen in our biopsy too.

Apart from the involvement of the integument, as a result of an extensive, multiorganic, sterile neutrophilic inflammatory process other organic systems can be involved. Mucosal involvement is common; it presents as edematous and aphthous lesions of the upper aerodigestive tract that can lead to airway obstruction. Systemic symptoms that may coexist are headache, myalgias, and arthralgias.[5] The syndrome may resolve either automatically or after medication. Recurrence is considered as the most common complication.

Systemic corticosteroids are considered as the “gold standard” treatment for SS. However, in children, the syndrome is considered more resistant to corticosteroids than in adults and sometimes protracted treatment for up to 5 months is required to avoid recurrences. Topical or intralesional corticosteroids may be effective in patients with localized lesions either as monotherapy or adjuvant therapy. Oral therapy with potassium iodide results in rapid resolution of symptoms; therefore, in patients with SS, who have a potential systemic infection or in whom corticosteroids have been contra-indicated, it is reasonable to initiate these agents as a first line therapy. However, indomethacin, clofazimine, cyclosporine, and dapsone are considered as second-line modalities.[5],[6]


  Case reports Top


Case 1

A 4-year-old girl presented with a history of sudden onset of skin lesions of 10 days duration. The lesions initially appeared over the face and progressed to involve the rest of the body. There was a history of upper respiratory tract infection 7 days prior to the onset of lesions. There was no history of drug intake prior to the onset of skin lesions and no history suggesting systemic involvement. There was a history of similar complaints 6 months back, for which she was treated with antibiotics and finally settled.

On general examination, child was febrile (102°F) with palpable left submandibular lymph nodes and bilateral conjunctivitis. On cutaneous examination over the face, limbs, and abdomen there were multiple, tender papules and plaques with central hyperpigmentation and elevated borders and a few resolved lesions showed hypopigmented patches with no atrophic scarring [Figure 1]a] and [Figure 1]b]. The lesions were typically “liquid to look, solid to touch.” There was no mucous membrane involvement. Systemic examination was normal. Investigation revealed a microcytic hypochromic anemia with hemoglobin of 9.8 mg/dl, a total count of 26,000 cells/dl (no abnormal cells) and a differential count was neutrophilic predominant (80%) and bone marrow study was normal. Erythrocyte sedimentary rate was 40 mm/1st h, and C-reactive protein was positive (40 mg/L). The histopathologic section revealed a dense infiltrate in the upper dermis, consisted mainly of neutrophils with no vasculitis.
Figure 1: (a) Multiple papules and plaques with central hyperpigmentation distributed over the face, limbs, and abdomen. (b) Bilateral conjunctivitis

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The child was treated with oral prednisolone at a dose of 1 mg/kg body weight, saturated solution of oral potassium iodide, five drops, 3 times daily and a course of oral azithromycin. There was a good response to steroids with resolution of fever and skin lesions. At present, the child is on follow-up and no further episode since then.

Case 2

An 8-month-old male baby presented with a history of fever followed by skin lesions after 5 days. The lesions initially appeared over trunk, face and later involved the rest of the body. There was no history of drug intake prior to the onset of lesions and no history suggestive of systemic involvement.

On general examination, child was febrile (102°F). On cutaneous examination, there were multiple, tender papules and plaques with elevated borders over the face, trunk, and limbs [Figure 2]. There was no mucous membrane involvement. Systemic examination was normal. Investigations revealed microcytic hypochromic anemia, hemoglobin of 10.4 mg/dl, a total count of 30,000 cells/dl (no abnormal cells) and a differential count was neutrophilic predominant (90%) and bone marrow study was normal. Erythrocyte sedimentary rate was 35 mm/1st h and C-reactive protein was positive (40 mg/L). The histopathologic section revealed a dense infiltrate in the upper dermis, consisted mainly of neutrophils with no vasculitis.
Figure 2: Multiple, tender papules and plaques with elevated borders over the face, trunk, and limbs

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The child was treated with oral prednisolone at a dose of 1 mg/kg body weight, oral potassium iodide (100%), two drops, 3 times daily and a course of oral cefixime.

These clinical features and histopathology reports for both cases were consistent with SS-probably classical type. There was a good response to steroids and potassium iodide with resolution of fever and lesions which was in favor of SS.


  Discussion Top


Sweet’s syndrome, is a rare disease in the pediatric population and accounts for 8% of cases and generally gender nonspecific.[2]

It occurs in 2 forms: Idiopathic and malignancy associated. There have been at least 10 reported cases of children with hematologic disorders in whom SS developed and three cases in whom the syndrome was associated with granulocyte colony-stimulating factor. Malignancies that are associated with SS are usually hemoproliferative disorders or solid tumors and account for only 20% of the cases. Relapses are noted most commonly in the malignancy-associated rather than the idiopathic variety (70% vs. 30%). Other associated conditions include infections, inflammatory bowel disease, rheumatologic diseases, pregnancy, primary immunodeficiency, and drug- or vaccine-induced conditions.[7]

In this case report, we specifically focus on SS that is rare in the pediatric population and with the presence of recurrent history which it is not malignancy associated. As for eye symptoms which are present in 30–75% of the patients with SS,[8] our first case manifested with bilateral conjunctivitis. The predisposing factor being upper respiratory tract infection is more prevalent and consistent with the literature. The diagnosis is based on clinical and histopathologic findings which is in accordance with the data reported in the literature. We also noted rapid response with no history of recurrence after treating with corticosteroids and potassium iodide.

Hence, SS should be borne in mind when considering a constellation of prolonged fever, skin lesions, and raised inflammatory markers. SS also presents in the pediatric age group with the majority para-infectious in etiology. Though the diagnostic criteria are confirmatory, a skin biopsy is necessary. In the pediatric age group with recurrence, malignancy should be ruled out. SS has been shown to not only respond rapidly to steroids when combined with potassium iodide but also helps in the prevention of recurrence and the side effects of long-term use of corticosteroids as seen in our case.



 
  References Top

1.
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56.  Back to cited text no. 1
    
2.
Wojcik AS, Nishimori FS, Santamaría JR. Sweet’s syndrome: A study of 23 cases. An Bras Dermatol 2011;86:265-71.  Back to cited text no. 2
    
3.
Prasad PV, Ambujam S, Priya K, Padma K, Rehana T. Sweet’s syndrome in an infant – Report of a rare case. Int J Dermatol 2002;41:928-30.  Back to cited text no. 3
    
4.
Su WP, Liu HN. Diagnostic criteria for Sweet’s syndrome. Cutis 1986;37:167-74.  Back to cited text no. 4
    
5.
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. 5
    
6.
Cohen PR. Neutrophilic dermatoses: A review of current treatment options. Am J Clin Dermatol 2009;10:301-12.  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.
Vignon-Pennamen MD. The extracutaneous involvement in the neutrophilic dermatoses. Clin Dermatol 2000;18:339-47.  Back to cited text no. 8
    


    Figures

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    Tables

  [Table 1]



 

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