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PubMedDate not supplied

Moderate-dose intravenous immunoglobulin treatment of Job's syndrome. Case report.

Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic "cold" abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after IVIG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.

Adult↗
PubMed2004

Disseminated pulmonary candidiasis complicating hyperimmunoglobulin E (Job's) syndrome.

Hyperimmunoglobulin E (Job's) syndrome is a rare disease characterized by recurrent pneumonia and sinusitis, pneumatoceles, chronic dermatitis, and elevated serum levels of immunoglobulin E. In this report, the author presents a case of disseminated pulmonary candidiasis in a young man with hyperimmunoglobulin E syndrome. His chest radiograph showed reticulonodular opacities in both lungs. Computed tomography scans revealed multiple pulmonary nodules and hilar and mediastinal enlarged lymph nodes.

Adult↗
PubMed2003

Job's syndrome--a case report.

Job's syndrome is a rare immune disorder characterized by atopic dermatitis-like skin lesions, elevated serum IgE-levels, repeated occurrence of skin and respiratory tract infections, and skeletal abnormalities. We report on a 12-year-old boy with Job's syndrome from Gujarat State, India. He disclosed the characteristic face, eczematous skin reactions and skin and lung infections. Long-term chemoprophylaxis was realized with oral penicillins that dramatically improved the course of his disease. Other treatment options are discussed but not all meet the needs of developing countries.

Administration, Oral↗
PubMed2006

Peripheral T-cell lymphoma and Job's syndrome: a rare association.

Job's syndrome or hyper-immunoglobulin E syndrome is a primary immunodeficiency characterized by recurrent staphylococcal abscesses and markedly elevated serum immunoglobulin E concentrations. Here we report a case of a woman with hyper-immunoglobulin E syndrome with a diagnosis of peripheral T-cell lymphoma, and we review the pertinent literature.

Adult↗
PubMed1983

Abnormalities of lymphocyte locomotion in immunodeficiency disease.

Lymphocyte and neutrophil locomotion were studied in 23 patients with well defined, primary immunodeficiencies. These included eight patients with common variable immune deficiency, three patients with X-linked agammaglobulinaemia, two patients with the Wiskott-Aldrich syndrome, three patients with ataxia telangiectasia, three patients with immunodeficiency and normal serum immunoglobulin concentrations, one patient with immune deficiency and hyper-IgM syndrome, two patients with Job syndrome and one patient with a granulocyte adherence defect. Random and stimulated lymphocyte and neutrophil migration were evaluated. C5a and casein were used to stimulate lymphocyte migration and C5a and formyl-methionyl-leucyl-phenylalanine (f-MLP) were used to stimulate neutrophil migration. Significantly depressed lymphocyte migration in response to casein and C5a was observed in patients with common variable immune deficiency, patients with immune deficiency and normal immunoglobulin concentration, and patients with Job syndrome. No consistent defect in lymphocyte locomotion was observed in the other patients studied. Neutrophil migration in response to C5a and f-MLP was depressed in Job syndrome, the patient with a granulocyte adherence defect, one of the six patients with common variable immune deficiency and none of the remaining patients. No significant correlation of skin test reactivity and lymphocyte migration was noted, but a correlation between the degree of lymphocyte proliferation in response to phytohaemagglutinin and lymphocyte migration in response to casein was observed. The results presented indicate that aberrations in lymphocyte migration occur in several types of immunodeficiency diseases and that defects in lymphocyte and neutrophil migration can occur simultaneously or totally independent of each other.

Adolescent↗
PubMed1977

Atopic dermatitis and impaired neutrophil chemotaxis in Job's syndrome.

A 22-year-old white woman with Job's syndrome was found to have atopic dermatitis and impaired neutrophil chemotaxis in vitro. Major clinical features of Job's syndrome included large, "cold" and recurrent staphylococcal abscesses, and intermittent bacterial and yeast infections. Evidence for atopic disease included infantile eczema progressing to flexural dermatitis, a family history of atopy, positive immediate hypersensitivity skin tests, and hyperimmunoglobulinemia E. Defective erythema responses to histamine, methyl niacinate, and methacholine (Mecholyl) chloride may explain the lack of redness, heat, or pain signalling the development of abscesses (hence the term "cold"). Impaired chemotaxis was probably due to an intrinsic neutrophil defect since patient's serum generated normal amounts of chemotactic factors and did not contain an inhibitor of neutrophil chemotaxis. A delay in neutrophil exudation in vivo may explain the abscess formations and the atopic diathesis may explain the absence of clinical signs of inflammation that have been described in this and other patients with Job's syndrome.

Adult↗
PubMedDate not supplied

The development of pulmonary adenocarcinoma in a patient with Job's syndrome, a rare immunodeficiency condition.

The hyperimmunoglobulin E (HIE) (Job's) syndrome often has it onset in childhood and is characterized by markedly elevated serum IgE levels, chronic dermatitis and recurrent pyogenic infections. Lymphoid malignancies have most commonly been associated with this syndrome while the first case in the literature of carcinoma associated with HIE syndrome was a squamous cell carcinoma of the vulva, described by Clark et al. in 1998. We observed a male patient with Job's syndrome diagnosed at age three who presented with bone pain and a metastatic epithelial tumor of the bone revealed by biopsy. Diagnostic procedures aimed at detecting the primary site showed multiple mediastinal lymph nodes with lung and liver metastases on computed tomography scans and an extradural spinal metastasis at the upper thoracic level on magnetic resonance imaging. Although the patient refused a bronchoscopic procedure, a diagnosis of pulmonary adenocarcinoma was established on the basis of sputum cytology and the clinical aspects of tumor extent. Intravenous corticosteroids and palliative radiotherapy were given for the spinal metastasis. Palliative chemotherapy could not be started because of the patient's poor performance status as well as nosocomial fungal pneumonia and pseudomonal urogenital infection with bacteremia. Despite the antifungal and broad-spectrum antimicrobial treatments, the patient died of pseudomonal sepsis.

Adenocarcinoma↗
PubMedDate not supplied

Giant chalazia in the hyperimmunoglobulinemia E (hyper-IgE) syndrome.

PURPOSE: To report the occurrence of recurrent multiple giant chalazia in the hyperimmunoglobulin E syndrome (hyper-IgE syndrome or Job syndrome). METHODS: Two patients with hyperimmunoglobulinemia E (>500 IU/ml) had ophthalmologic examination and surgical treatment for chalazia of the eyelids. RESULTS: The hyper-IgE syndrome is a rare immunodeficiency and multisystem disorder characterized by recurrent skin and pulmonary abscesses, connective tissue abnormalities, and elevated levels of serum IgE. In two patients with the hyper-IgE syndrome, multiple giant chalazia were seen in upper and lower eyelids. Despite surgical incision new giant chalazia arose. CONCLUSIONS: Recurrent multiple giant chalazia may occur as an ophthalmic feature of the hyper-IgE syndrome.

Adolescent↗
PubMed1994

Bronchial artery aneurysm in hyperimmunoglobulinemia E syndrome.

A 2-year-old girl presented with a large right middle lobe cavitating lesion, complicated by life-threatening hemoptysis secondary to hyperimmunoglobulinemia E (hyper IgE) syndrome (Job's syndrome). This proved to be a bronchial artery pseudoaneurysm which was successfully embolized.

Aneurysm↗
PubMed2004

Non-Hodgkin's lymphoma in Job's syndrome: a case report and literature review.

Job's or hyper immunoglobulin E recurrent infection syndrome (Hyper-IgE syndrome) is a rare, often inherited multisystem disorder, characterized by cutaneous abscesses, pneumonia, elevated IgE levels and skeletal defects. We report a case of a 22-year-old man with Job's syndrome who presented with back pain. He was found to have diffuse large B-cell lymphoma involving his second lumbar vertebrae and spleen. Treatment with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) chemotherapy achieved a complete remission after 4 cycles. A review of reported cases of lymphoma in Job's syndrome indicates an increase in relative risk of 259 (95% confidence interval 102, 416). The cause of the increased risk has yet to be defined but has similarities to a pathogenetic model of AIDS related lymphoma. In previous reports of lymphoma in Job's syndrome, patients presented with extranodal disease and had poor outcomes. With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission.

Adult↗
PubMed1984

Esophageal cryptococcosis in a patient with the hyperimmunoglobulin E-recurrent infection (Job's) syndrome.

Patients with the hyperimmunoglobulin E-recurrent infection (Job's) syndrome, which is characterized by an elevated immunoglobulin E level, recurrent staphylococcal infections, and an abnormality of neutrophil chemotaxis, have been reported to have visceral Candida infections in addition to their more frequent pyogenic infections. We report a patient with Job's syndrome who presented with massive hematemesis secondary to esophageal cryptococcosis. A thorough evaluation for an occult neoplasm or extraesophageal cryptococcosis was negative. The patient received a 6-wk course of amphotericin B (970 mg) and 5-fluorocytosine with complete radiographic and endoscopic resolution of the lesion. He is doing well 18 mo after therapy. The patient was not anergic, and his response to T-cell mitogens, helper-to-suppressor T-cell ratio, total number of T cells, and immunoglobulin-producing capability were all normal. This case is unusual in that it is the first documentation of a cryptococcoma of the esophagus and underscores the importance of culturing abnormal specimens for unsuspected pathogens in unusual clinical circumstances.

Adult↗
PubMed2000

Hyperimmunoglobulinaemia E syndrome: pulmonary imaging features.

Hyperimmunoglobulinaemia E (hyper-IgE) syndrome (Job's syndrome) is a rare disease that presents with recurrent cutaneous and sinopulmonary infections, and which begins in infancy and is associated with extreme hyper-IgE. The pulmonary imaging features typically consist of recurrent alveolar lung infections, pneumatocoeles and, occasionally, pneumothorax. Various other causes of pneumatocoeles in children can be excluded on the basis of clinical history and other data. Computed tomography may add valuable information for the management of these patients.

Child↗
PubMed1999

Regulation of immunoglobulin production in hyper-IgE (Job's) syndrome.

BACKGROUND: The hyper-IgE (HIE), or Job's, syndrome is a rare, complex disorder characterized by high levels of serum IgE in childhood and chronic dermatitis with recurrent, often severe sinopulmonary and skin infections. Although the etiology of HIE syndrome is unknown, there is evidence that patients with HIE have abnormalities in cellular immune responses, as well as in the production of polyclonal and antigen-specific antibodies. Furthermore, there appears to be a common (but still undefined) mechanism underlying the regulation of IgE and IgG4 in this condition. OBJECTIVE: We sought to assess the role of cytokines or cytokine receptor blockade in regulating IgE and IgG4 production in HIE. METHODS: PBMCs were isolated from patients with HIE (n = 9) and normal individuals (n = 8), and IgE and IgG4 production was assessed spontaneously, in the presence of recombinant IL-4, IL-13, IL-6, IL-8, IL-12, and IFN-gamma, under conditions in which the IL-4R was blocked or when these cytokines were neutralized by specific monoclonal or polyclonal antibodies. RESULTS: In PBMCs from patients with HIE, a significant (P <.01) reduction in the spontaneously produced IgE (and IgG4) was induced by either IFN-gamma or IL-12, although neither cytokine could totally abrogate the immunoglobulin production. Whereas spontaneous IgE (and IgG4) production was not affected by exogenous IL-4 and IL-13, neutralizing antibodies to IL-4 and IL-13 also significantly (P <.01) reduced the production of IgE and IgG4, a finding supported by the observation of increased expression of IgE germline transcripts in these patients. In contrast to the neutralization of IL-4 and IL-13 protein, anti-IL-4R antibodies or soluble IL-4R completely suppressed IgE and IgG4 production in HIE. Similarly, IL-8 or antibodies to IL-6 and TNF-alpha, cytokines known to affect IL-4-dependent IgE production, completely inhibited both IgE and IgG4 production. CONCLUSION: These data show that overproduction of IgE and IgG4 can be regulated by a number of cytokines affecting the IL-4-dependent pathway of IgE/IgG4 production in HIE and suggest new targets for therapeutic intervention.

Cytokines↗
PubMed1997

Pneumatocele complicated by fungal lung abscess in Job's syndrome. Successful lobectomy with the aid of videothoracoscopy.

Hyperimmunoglobulin E (Job's) syndrome, is a complex immune disorder characterized by complications involving, pulmonary and cutaneous infections. An 11-year-old girl presented with a pneumatocele superinfected by aspergillosis and occupying almost the entire right lower lobe. Lobectomy was performed with the aid of videothoracoscopic instruments, and 9 months later the patient is doing well.

Aspergillosis↗
PubMed1986

Ileocecal histoplasmosis mimicking Crohn's disease in a patient with Job's syndrome.

A 16-years-old male with a history of Job's syndrome and a recent diagnosis of Crohn's disease was admitted to the hospital because of abdominal pain, nonbloody diarrhea, weakness, and fever. Due to failure to respond to medical therapy it was decided that an operation was indicated, and resection of the terminal ileum and right colon was performed. Cecum and ileum were inflamed, and pathologic studies revealed the presence of budding yeasts which on special stain were diagnostic of Histoplasma species. Successful management was accomplished with oral ketoconazole.

Adolescent↗
PubMed1984

Deficiency in C3b receptors on neutrophils of patients with chronic granulomatous disease and hyperimmunoglobulin-E recurrent infection (Job's) syndrome.

C3b receptor (CR1) expression by neutrophils (PMNs) and erythrocytes (Es) from patients with chronic granulomatous disease (CGD) or with hyper-IgE, frequent infection (Job's) syndrome was compared with that of control subjects. The control subjects consisted of one group of patients with infections and a second group of normal, healthy individuals. Three quantitative assays were used: rosette formation with C3b-coated cellular intermediates (EAC43b), binding of radiolabeled monoclonal anti-CR1 ([125I]anti-CR1) to PMN surfaces, and binding of the antibody to nonidet P-40 (NP-40) extracts of PMNs and Es in an immunoradiometric assay. Rosette formation by the PMNs of five male CGD patients was about 50% of that of paired normal control subjects, whereas the rosette formation of three female CGD patients was similar to that of the control subjects. Surface binding of [125I]anti-CR1 to PMNs of 10 CGD patients was about half that of the normal subjects (mean percent binding was 2.33% for the CGD patients vs. 3.86% for the normal subjects, giving a difference of -1.53 +/- 0.22%, P less than 0.001 by the paired-sample t test). The degree of PMN binding was similarly low for both the male and the female CGD patients. Conversely, the binding of anti-CR1 to the PMNs of 11 infected control patients appeared to be similar to that of the normal subjects (4.51% for the patient vs. 4.21% for the paired normal subjects). The infected control group originally included four Job's syndrome patients, and when this subgroup was analyzed separately, their PMNs were shown to bind significantly less anti-CR1 than did the PMNs of the normal subjects (P less than 0.01 by the paired-sample t test). In contrast, the other infected control patients showed higher-than-normal levels of anti-CR1 binding (P less than 0.05). When compared to that of the normal subjects, the total CR1 quantitated in PMN extracts was also lower than normal in CGD patients (P less than 0.01 and in the PMN extracts of eight Job's syndrome patients tested (P less than 0.01). The PMNs of the other infected control subjects were not significantly different from those of the normal subjects in total CR1 expression. Extracts of Es from Job's syndrome patients also had fewer than normal CR1 (P less than 0.02). On the other hand, CR1 levels in E extracts from the CGD patients and the other control patients were similar to those in the normal control subjects. Quantitations of C3, C4, and factor B were normal in CGD.(ABSTRACT TRUNCATED AT 400 WORDS)

Antigen-Antibody Complex↗
PubMed1990

Job's syndrome: a rare cause of recurrent lung abscess in childhood.

A clinical syndrome characterized by recurrent staphylococcal infection of the skin and respiratory tract from birth was described in 1966 and referred to as Job's syndrome. Marked hyperimmunoglobulinemia E was later found to be associated with this syndrome. This article describes a case of Job's syndrome as a cause of recurrent lung abscess during childhood necessitating lung resection.

Bronchiectasis↗