Le syndrome de Job-Buckley : à propos d’un cas et revue de la littérature

A phenotypic variant of Job-Buckley syndrome

“…It affects both sexes, in the first weeks of life but sometimes at a later age as the case of our patient. This pathology is caused by the lack of chemotaxis of neutrophils [5] which leads to an immunodeficiency responsible for the generalized infectious manifestations characteristic of this syndrome [1,6]. These are bacterial and are found mainly in the skin as abscesses, pneumonia.…”

“…These are bacterial and are found mainly in the skin as abscesses, pneumonia. The offending bacterial species are, staphylococcus aureus in 60% of cases, hemophilus influenzae in 10% of cases and, infrequently, streptoccoccus, pneumoniae, cryptococcus neoformans, aspergillus fumigatus [1,2]. Chronic candida infections, mainly related to Candida albicans [1].…”

“…This syndrome was secondarily named by Buckley to a hyper-immunoglobulinemia E in 1972. It was in 1973 by Clark, then in 1974 when Hill showed that these two syndromes initially described rest on a defect of the chemotaxis of the neutrophils [1,2]. The physiopathology incriminates the mutation of the STAT3 gene.…”

“…This pathology affects a female subject, but especially both sexes are reached, without ethnic factor. The familial character has only rarely been described [1]. This syndrome is associated with particular clinical characteristics of immunological and morphological order [1][2][3].…”

“…The familial character has only rarely been described [1]. This syndrome is associated with particular clinical characteristics of immunological and morphological order [1][2][3]. The usual diagnostic criteria of SHIE include [9]: Hyper IGE greater than 2000 IU/ml, moderate hyper eosinophilia, eczematous dermatitis, recurrent bacterial infections, specially staphylococcus aureus, with a cutaneous seat, pulmonary, Ear Nose and Throat.…”