Defect in Neutrophil Granulocyte Chemotaxis in Job's Syndrome of Recurrent "Cold" Staphylococcal Abscesses

Right-sided endocarditis in an adult patient with hyperimmunoglobulin-E syndrome successfully treated with high-dose daptomycin

“…HIES, which was first described in 1966 and was called Job's syndrome, is characterized by specific facial characteristics, together with the presence of increased IgE levels, eczema and recurrent infections, which are usually caused by Staphylococcus aureus [2][3][4][5] . The most characteristic facial feature is a broad nasal base and broad nasal bridge, protrusion of the forehead, wide outer canthal distances, and deep set eyes [6,7] .…”

The First C. L. Oakley Lecture: Experimental Staphylococcal Infection in Mice

“…Defects in polymorph chemotaxis (Hill et al, 1974) and intracellular killing (Quie et al, 1967) are associated with the formation of, respectively, cold abscesses and chronic granulomatous lesions. In both situations there is a failure of killing of the staphylococci; staphylococcal antigens therefore persist.…”

“…This suggested that the full expression of staphylococcal virulence required both the capacity to suppress local defences in the skin and to produce tissue damage. Hill (1968) showed that a peptidoglycan "aggressin", which was left as the residue after cell walls from log-phase skin-virulent strains of Staphylococcus aureus had been treated with deoxychol- …”

Síndrome de hiper IgE, una enfermedad poco frecuente en edad adulta: Reporte de un caso

“…Otras manifestaciones incluyen hiperextensibilidad articular, paladar hendido, pneumatoceles, anormalidades de la línea media, escoliosis, fracturas ante traumatismos de baja energía, infecciones recurrentes de la vía aérea superior, candidiasis e infecciones graves 1 . Dentro de los desórdenes del sistema inmune de estos pacientes, se ha reportado la producción disminuida de interferón gamma por las células T, defectos en la respuesta de células T helper tipo 1 (Th1) dependiente de citoquinas, un desajuste en el balance de células Th1/Th2, una disminución de linfocitos T de memoria 6 , además de una defectuosa quimiotaxis de neutrófilos 7 . Por la rareza de esta enfermedad, su baja incidencia y similitud clínica con otras enfermedades de compromiso multisistémico, pudiendo conducir a retraso o equivocación en el diagnós-tico y tratamiento, hemos considerado relevante presentar el siguiente caso clínico de un paciente diagnosticado de HIES a los 24 años que además presentó un fenómeno trombótico debido a crioglobulinemia.…”