Makis et al. Journal of Medical Case Reports 2010, 4:281 http://www.jmedicalcasereports.com/content/4/1/281 Antibiotic and antiviral medications were discontinued and intravenous prednisone (1 mg/kg body weight/24 h) was administered for 10 days with immediate cessation of the fever and gradual improvement of the skin lesions, the hip complaints and the laboratory findings (Table 1). Thereafter, prednisone was continued orally and tapered off slowly over a period of 40 days. After six months of follow-up no recurrence has been observed and the child is thriving. Discussion We report on the case of an atypical vesicobullous var- iant of Sweet’s syndrome in a child with a serologically documented preceding rotavirus infection. Although gastroenteritis often precedes an ‘idiopathic’ Sweet’s syn- drome, this is the first, to the best of our knowledge, documented case of an associated rotavirus infection and this syndrome. Given (a) the high prevalence of rotavirus infections, (b) the frequency of gastroenteritis as a concomitant disease of the Sweet’s syndrome and (c) the wide suspicion in the literature that this syn- drome is still under-diagnosed, this is a remarkable dis- crepancy, which calls for further exploration in future studies. Sweet’s syndrome was originally described in 1964 by Douglas Sweet as an ‘acute febrile neutrophilic dermato- sis’ [1]. Since then several cases have been reported in the literature, most of them in adults but also in chil- dren of all age groups [6]. In children the syndrome is usually of the classic, ‘para-infectious’ subtype and in most cases follows a respiratory or a gastrointestinal infection. The most frequent nosologic entities that have been associated with this syndrome in children are shown in the Appendix. Classical or idiopathic (’para-infectious’) Sweet’s syn- drome is characterized by fever (higher than 38°C), blood leukocytosis and neutrophilia, which can precede the cutaneous manifestations for several days, character- istic skin lesions (tender erythematous papules or nodules which can develop into erythematous plaques with a characteristic papillomatous surface). Skin biopsy is the laboratory test that usually confirms the clinical diagnosis of Sweet’s syndrome. Edema and a diffuse and perivascularly attenuated inflammatory infiltrate of mature neutrophils with neutrophil fragmentation in the upper dermis, yet without microscopical evidence of vasculitis are the hallmarks of the histopathological pic- ture [7]. The most often affected areas are the upper extremities, the face and the neck. Sometimes the lesions may resemble blisters, while less often, may mimic lesions of pyoderma gangrenosum (Figure 2). Another characteristic clinical feature is the Köbner phenomenon, that is, appearance of ‘specific’ skin lesions at sites of minor cutaneous trauma (biopsy, Page 5 of 6 venipuncture, and so on.). Skin lesions in Sweet’s syndrome usually resolve without scarring. Apart from the involvement of the integument, as a result of an extensive, multiorganic, sterile neutrophilic inflammatory process other organic systems can be involved as well, as the hip involvement in our patient, probably the result of a sterile arthritis [8]. Also, mucosal involvement is not unusual; it presents as ede- matous and aphthous lesions of the upper aero-diges- tive tract in the mouth and pharynx that can lead to airway obstruction, as happened to our patient too [9]. Systemic symptoms that may coexist are headache, myalgias and arthralgias. The syndrome may resolve either automatically or after medication. Recurrence is considered as the most common complication and can take place in various time points. Quite typical is the immediate response to systemic corticosteroids, though after tapering off it recurs in at least one third of the cases. Corticosteroids (prednisone, 1 mg/kg body weight/24 h) are the treatment of choice, usually admi- nistered for 10 days and then tapered down slowly to avoid recurrence. However, in children the syndrome is considered more resistant to corticosteroids than in adults and sometimes protracted treatment for up to five months is required in order to avoid recurrences [10]. Topical corticosteroids can be used in patients with localized lesions either as monotherapy or con- currently to systemic prednisone. Potassium iodide and colchicine have also been used with good results as monotherapies or in combination with corticosteroids, while indomethacin, clofazimine, cyclosporine and dap- sone are considered as second-line modalities [11]. In malignancy associated cases cure or remission of the underlying disease is followed by the clearance of the symptoms, while in drug induced cases spontaneous improvement is achieved by stopping the suspected medication. The pathogenesis of Sweet’s syndrome is still unknown. Its epidemiological characteristics and the related conditions (infections, malignancies, systemic autoimmune conditions, inflammatory bowel diseases and female predominance) classify this disorder among the diseases that relate to hypersensitivity reactions to bacterial, viral or tumor antigens or to drugs. Circulat- ing autoantibodies, immune complexes and aberrant expression of different cytokines have all been postu- lated to explain the pathogenesis of this syndrome [12,13]. According to the most recent view, the syn- drome reflects a skin-confined or systemic disorder of the homeostasis of the cytokine network leading to abundance of pro-inflammatory cytokines in target tis- sues, especially of IL-1, G-CSF, GM-CSF and IFN-a. It is worth mentioning that rotavirus infections elicit an increased cytokine production in the intestinal