ABC | Volume 115, Nº1, Suplement, July 2020

Case Report Graça-Santos et al. Myocardial involvement in Sweet Syndrome Arq Bras Cardiol 2020; 115(1Suppl.1):6-9 were fulfilled for SS 3 and a high likelihood for cardiovascular involvement presenting as acute myocarditis (AM) 3 was considered. On D9, there had been no fever relapse, skin lesions were mostly healed, and CRP and TnI levels almost normalized (9.9mg/L and 0.32ng/dL respectively). The patient was discharged on a tapering corticosteroid regimen. Four days later, the patient presented completely asymptomatic with no skin lesions and both systemic inflammatory and myocardial injury biomarkers have normalized. A cardiovascular magnetic resonance (CMR) was scheduled six days after discharge and showed findings suggestive of myocarditis (Figure 4). Both LVEF and GLPSS improved up to 63% and -22.4%, respectively, three months after the initial assessment (Figure 2.B). The patient did not wish to undergo a second CMR study. During a two-year follow-up, the patient remained completely asymptomatic with no signs or symptoms of cardiovascular or malignant disease. Discussion We present a case where the diagnosis of SS was established as two major and three minor Driesch criteria were identified. 4 The idiopathic type was assumed since no recent drug intake was reported and no signs of malignant disease were present. Extracutaneous manifestations may occur, particularly in association with malignancy. 1,2 Cardiovascular involvement is extremely rare and up to this date, only two cases of myocarditis have been reported in the idiopathic type, to our best knowledge. 2,5,6 Both manifestations typically respond well to corticosteroids. 1 In this patient, the presence of transient chest discomfort associated with TnI elevation raised the suspicion of cardiovascular involvement. Both AM and acute myocardial infarction have been previously described as cardiovascular manifestations. 2 Coronary angiogram remains the gold standard for the diagnosis of CAD 7 or for its exclusion in case of suspected AM 8 and was normal in this case. There is some evidence that two-dimensional speckle tracking echocardiography (2D-STE) may help support the diagnosis of AM since GLPSS correlates with the presence of fibrosis and oedema on CMR and with lymphocytic infiltrates on endomyocardial biopsy (EMB). 9-12 In our case, the presence of GLPSS reduction mainly at the expense of the mid-basal segments, instead of the mid-apical segments (typical Figure 2 – Global and segmental longitudinal strain analysis; “bull-eye” plot (General Electric®). (A) Global strain is reduced (-16.4%) at admission; (B) and normalized (-22.4%) three months after corticosteroid treatment. Figure 3 – Histology of the cervical skin lesion (haematoxylin-eosin stain). Predominant neutrophilic dermal infiltrate and oedema (left). Zoom over the dermal area showing some lymphocytes, histiocytes, and absence of vasculitis (right). 7