[Job's syndrome (hyperimmunoglobulin E and recurrent infection syndrome)].
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Female↗
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An adult patient with hyperimmunoglobulemia E (Job's) syndrome, end-stage renal disease and repeated episodes of peritonitis.
We report the first case of an adult patient with hyperimmunoglobulemia E (Job's) syndrome, end-stage renal disease and repeated episodes of Staphylococcus peritonitis. A 42-year-old black female with end-stage renal disease was treated with hemodialysis, and later switched to peritoneal dialysis because of severe vascular access problems. On CAPD, she had numerous (> 15) episodes of staphylococcal peritonitis. Past history included chronic atopic eczema and repeated sinopulmonary infections since childhood. She was found to have serum IgE levels greater than 10,000 units/ml. This combination of repeated infections, chronic skin condition and phenomenal elevation of IgE levels establish the diagnosis of hyperimmunoglobulemia E (Job's) syndrome. The cause of repeated episodes of Staphylococcal peritonitis are presumed to be related to impaired host defense mechanisms known to be present in patients with this disorder. As nephrologists, we should be aware that patients with impaired host defense mechanisms are highly susceptible to peritonitis in the presence of an indwelling peritoneal catheter.
Adult↗
Neonatal Job's syndrome featuring a vesicular eruption.
A newborn infant who developed a vesicular eruption, clinically indistinguishable from herpetic lesions, eventually developed the classic features of Job's syndrome. The initial ares of involvement included the hands and feet, then the scalp, face, and suprapubic skin. The clear, tense vesicles varied only slightly in size and appeared as isolated, grouped, or confluent lesions on inflamed skin. Many eventually became umbilicated. The more typical eczematous component appeared over the course of the next several months. Although the child initially had an elevated white blood cell count and eosinophilia, his IgE level did not become dramatically elevated until after 1 year of age. Job's syndrome should be considered as part of the differential diagnosis of a vesicular eruption in the newborn.
Chronic Disease↗
[Hyperimmunoglobulinemia E, recurring staphylococcal infections and a defect in granulocyte chemotaxis in adults. A variant of Job's syndrome].
Two cases in adults with recurrent staphylococcal infections associated with abnormal granulocytic chemotaxis and hyperimmunoglobulinaemia E (Job's syndrome) are described. The pathophysiological mechanisms seems to consist of an abnormal IgE reaction against staphylococcal antigens causing secondary abnormality of granulocyte function. Abnormal cellular immune function was demonstrated in vitro and in vivo. Corticosteroid administration at first proved effective in both patients. One patient developed Hodgkin's disease of the mixed type in the course of the disease.
Adrenal Cortex Hormones↗
Atopiform dermatitis.
It is proposed to introduce the term 'atopiform dermatitis' to describe patients who have dermatitis with many of the characteristics of true atopic dermatitis, but who are not atopic. Atopy should be defined as the genetically determined and environmentally influenced syndrome in which the primary immunological abnormality is the production of allergen-specific IgE. It is suggested that by making a distinction between atopiform dermatitis and true atopic dermatitis, subsequent genetic, immunological and therapeutic studies will be improved. Furthermore, atopiform dermatitis would be a more appropriate diagnosis for the atopic dermatitis-like skin diseases that may occur in syndromes such as phenylketonuria, Schwachman's syndrome, Wiskott-Aldrich syndrome, Netherton's syndrome, Job's syndrome, selective IgA deficiency, agammaglobulinaemia and ataxia telangiectasia. In contrast to patients with true atopy, patients with atopiform dermatitis can logically be advised that allergen avoidance is not required, as they have no allergen-specific IgE.
Dermatitis, Atopic↗
Invasive aspergillosis in primary immunodeficiencies.
Primary immunodeficiencies are rare and usually first manifest during childhood. Invasive aspergillosis is the leading cause of mortality in chronic granulomatous disease (CGD), reflecting the key role of the phagocyte NADPH oxidase in host defense against opportunistic fungi. Despite interferon-gamma prophylaxis, invasive filamentous fungal infections are a persistent problem in CGD. Key principles of management of fungal infections involve early recognition and aggressive treatment and appropriate surgical debridement of localized disease. Because CGD is a disorder of phagocyte stem cells in which the gene defects are well defined, it is a model disease to evaluate immune reconstitution through stem cell transplantation and gene therapy. Patients with the hyper-IgE syndrome with recurrent infections (Job syndrome) are prone to colonization of lung cavities (pneumatoceles) by Aspergillus species leading to local invasion and rarely disseminated infection. Other primary phagocytic disorders, T-cell disorders, and mitochondrial disorders are uncommonly associated with invasive aspergillosis. Taken together, these rare primary immunodeficiencies highlight the complex coordination of both innate and acquired pathways mediating host defense against Aspergillus infection.
Animals↗
[Chemotaxis and cellular migration in respiratory pathology].
Numerous types of cells have a capacity for movement in physiological or pathological situations. For example, this is the case for inflammatory cells in the lung, during acute lobar pneumonia, sarcoidosis and idiopathic pulmonary fibrosis. Cellular migration is a general process which rests on the interaction between different chemotactic factors and specific receptors which are present on the target cells. On the other hand the addition of inhibitors can significantly decrease the cellular migration in the presence of chemotactic factors. In respiratory pathology, congenital chemotactic defects are exceptional (and the Chediak-Higashi syndrome and Job syndrome are examples). In contrast during the course of lung cancer, circulating monocytes often show a significant decrease in their chemotactic responsiveness.
Chemotactic Factors↗
Leukocyte functions.
Knowledge concerning leukocyte functions has increased enormously over the last two decades, largely because techniques have become available to assess them. It has provided insights into many disease entities, as well as on immunologic functioning as a whole. In addition to some classical clinical entities, such as severe combined immunodeficiency disease and chronic granulomatous disease, new entities have been discovered and described, in which subtle disturbances in white blood cell function are sufficient to cause disease. Furthermore, much has been learned about the effect of drugs and disease conditions on leukocyte functions. This review is not intended as a comprehensive description of white blood cell function testing, rather, it is meant to provide a practical guide for clinical laboratories. Therefore only those functional tests are presented which have a direct impact on patient management. These are primarily those functional derangements in white cells which either define a disease entity or without which a diagnosis cannot be made. Granulocytes, monocytes, and lymphocytes will be discussed separately. A short review of their physiology and function is presented to provide a context for the further discussion of their dysfunctions. This discussion of dysfunction and the techniques for laboratory testing thereof includes the evaluation of diagnostic usefulness and implications for patient management. Clinically, the most important granulocyte defects involve chemotaxis, respiratory burst, and intracellular microbial killing. Defects of granulocyte chemotaxis define the lazy leukocyte syndrome, Job's syndrome, and a series of relatively rare idiopathic neutrophil disorders. In addition, chemotaxis is a relatively easily measured sign of actin dysfunction. Defective generation of the respiratory burst with consequent impaired microbial killing defines chronic granulomatous disease and its variants as well as glutathione peroxidase deficiency. The most reliable clinical laboratory testing of granulocyte chemotaxis is performed by using granulocyte migration in response to a chemoattractant, either across a filter or in agarose. For clinical testing of respiratory burst, nitroblue tetrazolium (NBT) reduction is simple, quantitative, and has recently been adapted to automated measurement. Direct measurement of microbial killing is generally not indicated, since clinically important defects are recognized by measurement of respiratory burst generation. Although the monocyte's functions are numerous, they are somewhat less well characterized than those of granulocytes and lymphocytes.(ABSTRACT TRUNCATED AT 400 WORDS)
Animals↗
Cryptococcosis of the colon resembling Crohn's disease in a patient with the hyperimmunoglobulinemia E-recurrent infection (Job's) syndrome.
A 29-yr-old woman presenting with granulomatous colitis and a chronic perirectal abscess was found to have localized cryptococcosis associated with the hyperimmunoglobulinemia E-recurrent infection (Job's) syndrome. Similarity to previous cases of esophageal cryptococcosis and ileocecal histoplasmosis suggests an association between the hyperimmunoglobulinemia E-recurrent infection syndrome and localized fungal infections of the alimentary tract. To our knowledge, this is the first well-documented case of cryptococcosis confined to the colon and perirectal tissues.
Adult↗
[A case of Job's syndrome].
An 8-year-old boy had been followed in our clinic for recurrent abscesses of his thigh and buttocks. Serum levels of IgE greater than 2000 IU/ml and impaired leukocyte chemotactic response led to the diagnosis of Job's syndrome (hyperimmunoglobulin E syndrome), which is characterized by markedly elevated serum IgE, impaired neutrophil chemotaxis, chronic dermatitis, and recurrent pyogenic infections. Children with this syndrome are much more likely to develop opportunistic infections. An accurate etiological diagnosis and treatment of this specific infection in these children is mandatory. We report the first successful treatment of this syndrome with cyclosporin A. We therefore suggest that to diagnose this syndrome, quantitative IgE levels be routinely performed in children with recurrent skin or other suppurative infections.
Child↗
Generalized lymphadenopathy caused by Trichosporon asahii in a patient with Job's syndrome.
Of the six species of Trichosporon known to cause human infections, T. asahii is the main agent of invasive trichosporonosis. We describe an unusual case of generalized lymphadenopathy due to T, asahii in a 10-year-old boy with Job's syndrome (markedly elevated IgE with eosinophilia). The diagnosis was based on the presence of blastic conidia and hyphal elements breaking into arthroconidia in biopsied tissue of the cervical lymph node and isolation of the causal agent T, asahii in pure culture. The patient responded initially to amphotericin B therapy, but the infection recurred within 4 weeks and did not respond to therapy of liposomal amphotericin B and 5-fluorocytosine for 10 days. The patient left the hospital against medical advice.
Child↗
Case of the season. Job's syndrome with superimposed lymphoma.
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Adult↗
[Images in medicine. Job's Syndrome].
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Adult↗
Job's syndrome (granulomatous disease variant).
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Eponyms↗
[28-Year-Old Patient with Elevated Serum IgE Levels and Multiple Refractory Epidural and Paravertebral Abscesses. Clinical manifestation of a Job's Syndrome?].
BACKGROUND: The hyper-IgE syndrome is a rare primary immunodeficiency characterized by recurrent staphylococcal infections and high levels of serum IgE. CASE REPORT: The case of a 28-year-old man with epidural and paravertebral abscesses and a markedly elevated serum IgE level of 2,609 U/ml is reported. There was no allergic diathesis, nor had the patient a history of other diseases, which might explain the high serum IgE level. In a blood culture, Staphylococcus aureus was detected. Since early childhood, the patient has suffered from recurrent skin infections. CONCLUSION: The presented study case discusses the diagnosis of a Job's syndrome in this patient and reviews previously published cases. It also summarizes the current knowledge about pathogenesis and diagnostic criteria of this rare syndrome.
Adult↗