Journal Information
Vol. 6. Issue 1.
Pages 53-55 (January - February 2010)
Share
Share
Download PDF
More article options
Vol. 6. Issue 1.
Pages 53-55 (January - February 2010)
Full text access
Septic arthritis in a case of hyper-IgE syndrome
Artritis séptica en un caso de síndrome de hiperinmunoglobulinemia E
Visits
4888
Inmaculada Macías Fernández
Sección de Reumatología, Hospital Universitario Puerta del Mar, Cádiz, Spain
This item has received
Article information
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
Full text is only aviable in PDF
References
[1.]
R.H. Buckley, B.B. Wray, E.Z. Belmaker.
Extreme hyperimmunoglobulinemia E and undue susceptibility to infection.
Pediatrics, 49 (1972), pp. 59-70
[2.]
H.R. Hill, P.G. Quie, H.F. Pabst, H.D. Ochs, R.A. Clark, S.J. Klebaroff.
Defect in neutrophil granulocyte chemotaxis in Job's syndrome of recurrent “cold” staphylococcal abscesses.
Lancet, 2 (1974), pp. 617-619
[3.]
S.M. Holland, F.R. De Leo, H.Z. Elloumi, A.P. Hsu, G. Uzel, N. Brodsky, et al.
STAT3 mutations in the hiper-IgE syndrome.
N Engl J Med, 357 (2007), pp. 1608-1619
[4.]
M.L. Paulson, A.F. Freeman, S.M. Holland.
Hyper IgE syndrome: An update on clinical aspects and the role of signal transducer and activator of transcription 3.
Curr Opin Allergy Clin Immunol, 8 (2008), pp. 527-533
[5.]
G.C. Pien, J.S. Orange.
Evaluation and clinical interpretation of hypergammaglobulinemia E: Differentiating atopy from immunodeficiency.
Ann Allergy Asthma Immunology, 100 (2008), pp. 392-395
[6.]
D.L. Domingo, A.F. Freeman, J. Davis, J.M. Puck, W. Tianxia, S.M. Holland, et al.
Novel intraoral phenotypes in hyperinmunoglobulin E syndrome.
[7.]
M. Trendelenburg, J.A. Schifferli.
Rituximab in a patient with hiper-IgE syndrome.
Arch Dermatology, 143 (2007), pp. 807-808
[8.]
B. Grimbacher, S.M. Holland, J.M. Puck.
Hyper-IgE syndromes.
Immunol Rev, 203 (2005), pp. 244-250
[9.]
G. Guhl, B. Diaz-Ley, J. Fernandez-Herrera.
Uso de fármacos biológicos en dermatosis fuera de la indicación probada.
Actas Dermosifiliográficas, 99 (2008), pp. 5-33
[10.]
Immunologic disorders in infants and children, 5st ed., pp. 550
Copyright © 2010. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
Idiomas
Reumatología Clínica (English Edition)
Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?