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In the anamnesis, he reported a history of atopic dermatitis and eczema since childhood, as well as recurrent otitis, bronchial asthma and delayed tooth eruption. Physical examination revealed coarse facies, oropharyngeal candidiasis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), multiple folliculitis lesions and pustulosis in abdomen (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B) and limbs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C), and several skin cold abscesses (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D). The additional tests, showed increased immunoglobulin E (IgE) levels up to 988.6<span class="elsevierStyleHsp" style=""></span>IU/ml (0–100). Given the suspicion of a primary immune deficiency such as hyper-immunoglobulin E syndrome (HIES), a specific genetic study was requested, which showed heterozygous mutation of STAT3 gene, which confirmed the diagnosis of autosomal dominant HIES. Antibiotic prophylaxis with trimethoprim–sulfamethoxazole significantly improved his symptomatology.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">HIES or Job syndrome is a rare primary immunodeficiency first described in 1966, clinically characterized by dermatitis and recurrent bacterial infections in skin and lungs. It also occurs with abnormalities in dental, musculoskeletal and facial development, neurological and vascular problems, along with an increased risk of lymphoma.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Vizán-Caravaca JR, García-Castro JM, López-García S. Síndrome de Job. Med Clin (Barc). 2019;152:331.</p>" ] ] "multimedia" => array:1 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 979 "Ancho" => 1400 "Tamanyo" => 163209 ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015200000008/v1_201904100623/S2387020619300968/v1_201904100623/en/main.assets" "Apartado" => array:4 [ "identificador" => "51820" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images in medicine" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015200000008/v1_201904100623/S2387020619300968/v1_201904100623/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020619300968?idApp=UINPBA00004N" ]
Journal Information
Vol. 152. Issue 8.
Pages 331 (April 2019)
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Vol. 152. Issue 8.
Pages 331 (April 2019)
Image in medicine
Job syndrome
Síndrome de Job
Visits
4
José Ramón Vizán-Caravaca
, José Miguel García-Castro, Sara López-García
Corresponding author
Servicio de Medicina Interna, Hospital Santa Ana, Motril, Granada, Spain
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