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Molluscum contagiosum and Job's syndrome.
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Hyperimmunoglobulin E recurrent infection (Job's) syndrome.
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Job's syndrome or hyperimmunoglobulin-E.
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Recurrent colonic histoplasmosis after standard therapy with amphotericin B in a patient with Job's syndrome.
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Levamisole is inferior to placebo in the hyperimmunoglobulin E recurrent-infection (Job's) syndrome.
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Anesthetic management of a pregnant patient with the hyperimmunoglobulin E (Job's) syndrome.
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Importance of life-long continuous antimicrobial prophylaxis to prevent infections in patients with Job's syndrome.
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Job's syndrome: an unusual response to a common drug.
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Eye involvement in hyperimmunoglobulinemia E (Job's) syndrome.
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Job's syndrome. A case report.
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The development of invasive vulvar cancer in a patient with Job's syndrome, a rare immunodeficient condition.
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Staphylococcal botryomycosis and hyperimmunoglobulin E (Job's) syndrome in an infant.
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Job's syndrome associated with HIV infection improved with immune globulin therapy.
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Cranial synostosis in Job's syndrome.
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Colon perforation in a patient with hyperimmunoglobulin E (Job's) syndrome.
A Taiwanese boy was diagnosed as having hyperimmunoglobulin E syndrome at the age of 4 years. At age 18 he was admitted to the hospital because of pneumonia in the left lower lobe. Abdominal pain developed 9 days later, and his condition progressed to peritonitis. Colon perforation, 10 cm distal to the ileocecal valve, was found. Double-barrel ileostomy was performed, and reanastomosis was done 1 1/2 months later. Afterward, he was fine, and he had no significant gastrointestinal problems after being discharged. To the author's knowledge, this is the first reported case of hyperimmunoglobulin E syndrome complicated by colon perforation.
Immunoglobulins in the hyperimmunoglobulin E and recurrent infection (Job's) syndrome. Deficiency of anti-Staphylococcus aureus immunoglobulin A.
Patients with the hyperimmunoglobulin E and recurrent infection syndrome (HIE) characteristically have frequent skin and respiratory infections caused by Staphylococcus aureus. We have developed a set of enzyme-linked immunosorbent assays that use whole S. aureus (Wood's strain) immobilized on 0.22-micrometers filters and highly specific, affinity-purified enzyme conjugates of goat anti-human IgE, anti-human IgD, anti-human IgG, anti-human IgA, and anti-human IgM. These reagents were used to determine S. aureus-specific immunoglobulin (Ig) levels. As previously published, 10 patients with HIE had markedly higher levels of anti-S. aureus IgE than did 5 patients with eczema and recurrent superficial S. aureus infections (P less than 0.001). The HIE patients were also found to have a deficit of anti-S. aureus serum IgA as compared with 12 normal subjects, 12 patients with chronic granulomatous disease, 5 patients with chronic eczema and recurrent superficial S. aureus infections, and 3 patients with the Chediak-Higashi syndrome (P less than 0.01 for each comparison). In addition the HIE patients had an excess of anti-S. aureus IgM as compared with normal subjects (P less than 0.01). An expected excess of anti-S. aureus IgG was absent. These abnormalities cannot be explained by variations of total serum Ig levels or by a general inability to produce antigen-specific IgA because levels of naturally occurring IgA antibody against Escherichia coli lipopolysaccharide and the antigens of the pneumococcal vaccine are normal. Parotid saliva from patients with HIE contained less salivary IgA per milligram of protein (P less than 0.01) and less salivary anti-S. aureus IgA per milligram of protein (P less than 0.05) than did normal controls. The incidence of infection at mucosal surfaces and adjacent lymph nodes correlated inversely with serum anti-S. aureus IgA (r = -0.647, P = 0.034), serum anti-S. aureus IgE (r = -0.731, P = 0.016), total serum IgE (r = -0.714, P = 0.020), and total serum IgD (r = -0.597, P = 0.049). These findings are evidence of a previously undescribed immunoregulatory defect in patients with HIE, which may contribute to the increased susceptibility to infection in this syndrome.