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Vol. 6. Issue 1.
Pages 53-55 (January - February 2010)
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Vol. 6. Issue 1.
Pages 53-55 (January - February 2010)
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Septic arthritis in a case of hyper-IgE syndrome
Artritis séptica en un caso de síndrome de hiperinmunoglobulinemia E
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Inmaculada Macías Fernández
Sección de Reumatología, Hospital Universitario Puerta del Mar, Cádiz, Spain
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Abstract

Hyper-IgE syndrome (HIES) is characterized by recurrent skin and pulmonary infections (mainly bacterial), eczematous dermatitis and elevated serum IgE levels. Associated abnormalities in some patients include coarse facial features, failure or delay of shedding of primary teeth, recurrent fractures, hyperextensible joints, and scoliosis. Laboratory abnormalities include elevated total serum IgE levels, typically ranging from 1,000 to greater than 50,000IU/ml and variable eosynophilia. The diagnosis of HIES is based upon the presence of suggestive clinical and laboratory findings. A definitive laboratory test is not commercially available at present. Management of patients with HIES is focused on skin care, prevention of infection, prompt and complete treatment of infections that do develop, and control of pulmonary complications.

Keywords:
Hiper-IgE syndrome
Infections
Diagnosis
Septic artritis
Cutaneous infections
Resumen

El síndrome de hiperinmunoglobulinemia E (HIES) se caracteriza por infecciones recurrentes cutáneas y pulmonares (principalmente bacterianas), dermatitis eccematosa y elevados niveles séricos de IgE. Anomalías asociadas en algunos pacientes incluyen rasgos faciales toscos, fracaso o retraso de la caída de los dientes primarios, fracturas recurrentes, hiperlaxitud en las articulaciones y escoliosis. Anormalidades de laboratorio son niveles elevados de IgE total en suero, generalmente, de 1.000 a más de 50.000U/ml y eosinofilia variable. El diagnóstico de HIES se basa en la presencia de característicos hallazgos clínicos y de laboratorio. Un test diagnóstico definitivo no existe en la actualidad. El manejo de los pacientes con HIES se centra en el cuidado de la piel, la prevención de la infección, el tratamiento precoz de las infecciones que se desarrollan y el control de las complicaciones pulmonares.

Palabras clave:
Síndrome de hiperinmunoglobulinemia E
Infecciones
Diagnóstico
Artritis séptica
Infecciones cutáneas
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Copyright © 2010. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
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