Sweet Syndrome in Children

Abstract

The objective of this study was to describe the clinical features of Sweet syndrome in children. Our study population consisted of seven children diagnosed with Sweet syndrome over a 22-year period. Age, sex, appearance and location of lesions, associated signs and symptoms, past medical history, pathology, and subsequent disease course were documented for each patient. Fever and typical lesions were reported in most of patients in our study. The majority of patients presented with less-typical findings, such as pustules, vesicles, bullae, oral ulcerations, atrophic scars, and evidence of pathergy. Of the seven children in our study, four were found to have a preceding nonspecific upper respiratory or gastrointestinal infection, and two were diagnosed with an underlying hematologic malignancy. Our results suggest that atypical lesions are relatively common in children with Sweet syndrome and that underlying malignancy is associated with a minority of cases of pediatric Sweet syndrome.

Síndrome de Sweet en Pediatría. A proposito de un caso

“…Se han descrito tres escenarios clínicos en los que se presenta el SS: clásico o idiopático, paraneoplásico e inducido por drogas (7) . En pediatría se ha descrito hasta un 45% de casos idiopáticos, posterior a infecciones gastrointestinales o respiratorias transitorias, un 30% asociado a condiciones inflamatorias crónicas y un 25% de los casos paraneoplásicos, siendo la mayor asociación con leucemia mieloide aguda, osteosarcoma y síndrome mielodisplásico (4,6,10,11) . Debido a estas asociaciones con diversas patologías se han propuesto esquemas de tamizaje de patologías asociadas al SS, que incluye aspectos clínicos como medición de presión arterial, pruebas de laboratorio básico: Hemograma-VHS, LDH y ácido úrico, además de detección de VIH, ANA y niveles de inmunoglobulinas séricas.…”

“…Pese a que el tratamiento de primera línea corresponde a corticoides en dosis equivalente de prednisona 1-2 mg/kg/día, se ha visto resolución espontánea de las lesiones cutáneas sin mediar tratamiento (4) . En nuestro caso hemos visto una buena respuesta terapeútica inicial con el uso de corticoides sistémicos, por lo que se indicó reducción progresiva de la dosis, con control clínico estricto.…”

“…No se han reportado casos de SS en niños o adolescentes en la literatura nacional. La patogenia no está del todo clara, aunque se ha asociado con infecciones, inmunodeficiencia, fármacos, neoplasias y otras enfermedades sistémicas (4) . Algunos autores especulan que una predisposición genética puede determinar variaciones geográficas en la incidencia, ya que casi la mitad de todos los casos notificados han ocurrido en Japón, encontrandose una asociación con el antígeno leucocitario humano (HLA) BW 54 en estos pacientes, el cual podría jugar un rol (2) .…”

Sweet Syndrome in a Patient With Systemic Lupus Erythematosus Flare.

“…SS has been treated with a variety of therapeutic interventions; which are primarily based on case series and individual reports of clinical experience. Corticosteroid therapy is considered first-line treatment for SS [16]. In addition, there are some reports suggesting the use of colchicine and potassium Iodide as first-line treatment options [17].…”

Sudden Eruption of Tender Nodules and Plaques in a Three-year-old girl: A Quiz

“…It is estimated that approximate 20% of cases are associated with cancer (1). In children, less than 80 cases of Sweet's syndrome have been reported worldwide so far (2,3,(5)(6)(7). In a recent review evaluating 64 cases of Sweet's syndrome in childhood and adolescence, 21 cases (33%) were categorized as "parainflammatory" and associated with diseases such as chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, immunodeficiencies, arthritis, and systemic lupus erythematosus, while 16 cases (25%) were categorized as "paraneoplastic", comprising associations such as leukaemia, aplastic anaemia, and Fanconi anaemia (2).…”