ABC | Volume 115, Nº1, Suplement, July 2020

Case Report Myocardial Involvement in Sweet Syndrome: A Rare Finding in a Rare Condition Luís Graça-Santos, 1 Katarina Kieselova, 2 Fernando Montenegro-Sá, 1 J oana Guardado, 2 João Morais 2 Department of Cardiology, Leiria Hospital Centre, 1 Leiria - Portugal Department of Dermatology, Leiria Hospital Centre, 2 Leiria - Portugal Introduction Sweet Syndrome is an acute febrile neutrophilic dermatosis characterized by an association of fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), as well as pathologic findings consisting predominantly of mature neutrophils typically located in the upper dermis. 1 It is a rare condition with a worldwide distribution which can present as one of three main clinical types: idiopathic, malignancy- associated, or drug-induced. 1-3 Extracutaneous manifestations may occur but cardiovascular involvement is extremely rare. 1,2 Case presentation A previously healthy 41-year-old male presents to the emergency department with a 48-hour history of mild fever and worsening widespread skin lesions. He denied recent drug intake, known allergies, relevant personal or familial diseases, as well as suspicious epidemiological context. The patient was febrile (38.3ºC) and heart rate, blood pressure, and oxygen saturation were all normal. The chest and abdominal examination were both unremarkable. Skin examination revealed painful pseudovesiculate, erythematous papules, and plaques on the nape, neck, shoulders, and arms, as well as painful hyperpigmented subcutaneous nodules ( erythema nodosum-like ) on the legs (Figure 1). Blood tests showed slight leucocytosis (10800/ uL) with 81.4% of neutrophils, erythrocyte sedimentation rate was 89mm/h (normal value (NV) <10) and C-reactive protein (CRP) 128.5mg/L (NV<5.0). Electrolytes, renal and hepatic profiles were normal. A few hours later, the patient complained of transient chest discomfort at rest. The electrocardiogram showed sinus rhythm at 58 per minute with first-degree atrioventricular block plus incomplete right bundle branch block. Troponin I (TnI) was 1.89ng/mL (NV<0.05) and raised up to 10.82ng/mL six hours later. The repeated electrocardiogram was identical to the previous one. Transthoracic echocardiogram (TTE) was normal, demonstrating preserved left ventricular ejection fraction (LVEF; 53% Simpson’s biplane method) with no major wall motion abnormalities. However, global longitudinal peak systolic strain (GLPSS) was reduced, especially at the expense of the mid-basal segments being the apex relatively spared (Figure 2.A). Coronary angiogram excluded obstructive coronary artery disease (CAD). The patient was admitted to the internal medicine ward with the presumptive diagnosis of acute febrile neutrophilic dermatosis. On the second day (D2), skin biopsy was performed and oral prednisolone (PDN) 1mg/Kg/day was initiated, taking into consideration the persistence of both fever and skin lesions as well as the increase of the CRP value (242mg/L). Despite no chest discomfort relapse, TnI reached a peak level of 15.01ng/mL on D2. After initiating PDN, the patient remained afebrile, and both systemic inflammatory and myocardial injury biomarkers started to decrease. Complementary laboratorial tests (such as electrophoretic proteinogram, autoimmunity screening, thyroid hormones, blood cultures and serology tests) were normal. The histological skin analysis revealed subepithelial oedema, dermal inflammatory infiltrate with polymorphonuclear predominance and absence of vasculitis (Figure 3). Based on this information, diagnostic criteria Mailing Address: Luís Graça Santos • Leiria Hospital Centre - Rua Santo Andre Pousos. 2410-196, Leiria – Portugal E-mail: luismscp1@gmail.com Manuscript received April 13, 2019, revised manuscript July 27, 2019, accepted August 18, 2019. Keywords Sweet Syndrome/physiopathology; Erythema Multiforme; Neutrophils; Myocarditis; Adrenal Cortex Hormones/ therapeutic use. DOI: https://doi.org/10.36660/abc.20190249 Figure 1 – Skin examination. Pseudovesiculate, erythematous papules and plaques on the nape (top); hyperpigmented subcutaneous nodules on the legs (bottom). 6