Cardiovascular Involvement in a Boy with Sweet's Syndrome

Abstract

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a rare disease in infancy. It may present in an isolated manner or be associated with diverse conditions. Only two children with postinflammatory slack skin who developed cardiovascular disease have been described to date, both of whom died from coronary artery occlusion. We report a boy with Sweet's syndrome and diffuse vascular disease involving the aorta and the supraaortic vessels, the pulmonary trunk, and the right coronary artery but without signs of coronary obstruction.

Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis

“…Other first-line systemic treatments for Sweet's syndrome are potassium iodide and colchicine (Table 10) [10,12,17,20,23,30,49,70,143,184,198,203,221,223,231,240,245,250,259,261,281,284,294,296,329,359-363,368-384,397,410]. …”

“…Second-line agents for treating Sweet's syndrome include indomethacin, clofazimine, cyclosporin, and dapsone (Table 11) [1,12,17,20,30,203,221,231,245,259,261,284,294,296,372,378-384,421]. All of these agents have been used as monotherapy either in the initial management of the patient or after first-line therapies have failed.…”

“…The initial oral dose of cyclosporin ranged from 2 mg/kg/day [380] to 10 mg/kg/day [12,381]; for the patient who was receiving 10 mg/kg/day, the dose was reduced by 2 mg/kg/day every 2 days and discontinued on day 21 [12,381]. The initial oral dose of dapsone ranged from 100 mg per day to 200 mg per day [17,20,30,203,221,245,284,372,382-384,421]. …”

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases

“…Despite the long interval of up to 7 years between Sweet's syndrome and cardiological diagnosis, some authors suggest an acquired (auto-) inflammatory process leading to a parallel inflammation of cutis and vessels with cutis laxa representing the postinflammatory state of the skin [48,21]. Alternatively, and in view of the phenotypic overlap between Loeys-Dietz syndrome, a recently described autosomal dominant aortic-aneurysm syndrome with widespread systemic involvement due to mutations in the genes encoding transforming growth factor beta receptors 1 and 2 [42] and vascular Ehlers-Danlos syndrome, a genetic defect could also come to mind.…”

A case of Sweet's syndrome with slack skin and pathergy phenomenon

“…Hwang's case had coexisting α 1 ‐antitrypsin deficiency, suggesting that the lack of natural protease inhibitors may allow inflammatory processes to produce the elastic tissue destruction 10 . Review of the literature (Table 1) revealed that there were at least three other cases of acquired cutis laxa following Sweet's syndrome 11–13 . As shown in the table, all of these patients were below the age of 2 years and had extensive skin lesions.…”