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Vol. 14. Num. 2.March - April 2018
Pages 65-124
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Vol. 14. Num. 2.March - April 2018
Pages 65-124
Case report
DOI: 10.1016/j.reumae.2016.09.005
SAPHO Syndrome in Childhood. A Case Report
Síndrome SAPHO en la infancia. Presentación de un caso clínico
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Manuel Vargas Pérez
Corresponding author
vargasperezm@gmail.com

Corresponding author.
, Belén Sevilla Pérez
Unidad de Gestión Clínica de Pediatría, Hospital Universitario San Cecilio, Granada, Spain
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Table 1. Inclusion and Exclusion Criteria for SAPHO Syndrome.
Table 2. Diagnostic Criteria Proposed by Kahn and Modified in 2003.
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Abstract

The acronym of SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) combines a cluster of cutaneous and musculoskeletal manifestations, such as hyperostosis of bones of the anterior chest wall associated with acne fulminans and hidradenitis suppurativa. There are no validated diagnostic criteria in children. Nonsteroidal anti-inflammatory drugs are not always sufficient, and the use of corticosteroids, disease-modifying agents, tumor necrosis factor-α inhibitors or bisphosphonates may be necessary.

We present the case of a child with polyarticular involvement, osteoarthritis of the sternoclavicular joint with severe inflammatory disorders and acne conglobata, with an excellent response to intravenous pamidronate.

Keywords:
SAPHO syndrome
Hyperostosis
Acne
Bisphosphonates
Resumen

El acrónimo síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis) engloba tanto manifestaciones cutáneas como muculoesqueléticas, entre ellas hiperostosis de los huesos y de las articulaciones de la pared torácica anterior, asociado a manifestaciones cutáneas; acné fulminans e hidradenitis supurativa. Los criterios diagnósticos no han sido validados en niños. El tratamiento inicial con antiinflamatorios no esteroideos en ocasiones es insuficiente y puede ser preciso asociar corticoides, fármacos modificadores de la enfermedad, antagonistas del factor de necrosis tumoral o bifosfonatos.

Presentamos el caso clínico de un escolar con afectación poliarticular, osteoartritis de articulación esternoclavicular con importante componente inflamatorio y acné conglobata, con buena respuesta al tratamiento con pamidronato por vía intravenosa.

Palabras clave:
Síndrome SAPHO
Hiperostosis
Acné
Bifosfonatos

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