1/16. Defective neutrophil chemotaxis with variant ichthyosis, hyperimmunoglobulinemia E, and recurrent infections.Host defense mechanisms were evaluated in a 4-1/2-year-old boy with recurrent pyogenic infections and a unique hyperkeratotic skin disorder. The patient's neutrophils were consistently defective in chemotactic responsiveness but had normal NBT reduction, glucose oxidation, and iodination. serum concentrations of IgE were markedly elevated and the secondary antibody response was abnormal. No T-cell dysfunction was detected. These findings suggest a relationship between this patient and patients with other syndromes associated with recurrent infections, cutaneous disease, defective chemotaxis, immunodeficiency, and hyperimmunoglobulinemia E.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
2/16. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.- - - - - - - - - - ranking = 0.8keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
3/16. Recurrent eosinophilic cystitis with peripheral eosinophilia and hyperimmunoglobulinemia E.A case of recurrent eosinophilic cystitis with peripheral eosinophilia and hyperimmunoglobulinemia E, which responded successfully to initial and secondary steroid therapies is reported.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
4/16. 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.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
5/16. Recurrent staphylococcus aureus chalazia in hyperimmunoglobulinemia E (Job's) syndrome.PURPOSE: To report a case of recurrent staphylococcus aureus chalazia in a patient with hyperimmunoglobulinemia E syndrome (Job's Syndrome). DESIGN: Case report. methods: Three separate surgical incisions and curettages of multiple, recurrent chalazia of the right upper eyelid were performed over a course of 3 months. Cultures and pathologic specimens were obtained. Postoperative treatment consisted of oral erythromycin and amoxicillin/clavulonate, topical tobramycin/dexamethasone ointment, and warm compresses. RESULTS: pathology of the tarsus confirmed the diagnosis of multiple chalazia. Cultures of the chalazia contents were positive for staphylococcus aureus. No further recurrence was observed following the third surgical procedure over a 3-month follow-up period. CONCLUSION: Characteristic staphylococcus aureus skin infections in immunodeficient patients with hyperimmunoglobulinemia E syndrome can involve the eyelids and may be recurrent despite appropriate medical and surgical therapy.- - - - - - - - - - ranking = 1.2keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
6/16. A probable case of Muckle-Wells syndrome.Muckle-Wells syndrome is a rare autosomally dominant disorder belonging to the group of periodic fever syndromes. Three main features of the disease are: (i) urticarial eruptions; (ii) progressive perceptive deafness; and (iii) amyloid nephropathy. A 26-year-old Japanese woman had suffered at birth from an urticarial rash and episodic fever. The fever was frequently associated with chills and ill-defined malaise. There was no familial history of urticarial rash or fever. Although she did not recognize hearing loss, audiometry revealed perceptive deafness. She also had hepatosplenomegaly and hyperimmunoglobulinemia, but did not have persistent arthritis, or any neurological or gastrointestinal disorder. No growth retardation was observed. skin biopsy specimens from her buttock showed a sparse perivascular and interstitial infiltrate of neutrophils in the papillary dermis. Periodic fever syndrome was diagnosed. Muckle-Wells syndrome was most likely, although no amyloid nephropathy was observed and no gene mutations of CIAS1 (T785C, C778T, G907A, G1315A, G1075C) were detected. We treated her with prednisolone, which had a partial effect. Previous treatment with colchicines, antihistamines, dapsone, clarithromycin, minocycline hydrochloride and loxoprofen sodium had been unsuccessful. Muckle-Wells syndrome may go undiagnosed for many years or be misdiagnosed as refractory urticaria. Therefore, we should consider the possibility of periodic fever syndrome when we see patients with refractory urticaria and episodic fever.- - - - - - - - - - ranking = 0.2keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
7/16. Variant of hyper-IgE syndrome: the differentiation from atopic dermatitis is important because of treatment and prognosis.The hyper-IgE syndrome is characterized clinically by recurrent staphylococcal abscesses of the skin, lungs and other sites from infancy. Affected patients also have a pruritic dermatitis that differs in character and distribution from lesions of atopic dermatitis. Most lack other signs of atopic disease, develop persistent pneumatoceles and have osteopenia. Laboratory abnormalities include the consistent presence of marked hyperimmunoglobulinemia E and eosinophilia of blood, sputum and tissues. They may have other inconsistent abnormalities of humoral and cellular immune responses and sometimes of phagocytic cell chemotactic responsiveness. Other clinical problems reported in such patients have included lymphomas, cryptococcal meningitis and cutaneous fungal disease. An 18-year-old male patient with a variant of the hyper-IgE syndrome, which he had acquired after a measles attack at the age of 5 years, suffered from recurrent ulcerative dermatitis and lymph node abscesses. Immunological investigation revealed an excessively elevated total serum IgE level (46,850 IU/ml), the presence of specific IgE to staphylococci, and quantitative and functional deficiency of IgG2. skin and serological (radioallergosorbent) tests to inhalant and nutritive allergens were negative. Differentiation from atopic dermatitis should be made, because a long-term antistaphylococcal regime not only improves skin lesions but hinders the occurrence of lung abscesses and pneumatoceles.- - - - - - - - - - ranking = 0.2keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
8/16. 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.- - - - - - - - - - ranking = 0.2keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
9/16. Hyperimmunoglobulinemia E syndrome of neonatal onset.A case of hyperimmunoglobulinemia IgE with recurrent infections of neonatal onset and unfavourable evolution is described. The first clinical finding was the appearance of maculopapular rash in the skin when the child was fifteen days old. From this period onwards, respiratory and cutaneous infections were continuous, leading to a progressive deterioration of the general condition, and malnutrition, which ended fatally at the age of three years. IgE levels were measured on several occasions, this being 17 IU/ml at the onset of the symptoms; afterwards, the mean value was 17.680 IU/ml (Range: 14.9-32.710 IU/ml).- - - - - - - - - - ranking = 0.2keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
10/16. Anti-staphylococcus aureus IgE antibodies for diagnosis of hyperimmunoglobulinemia E-recurrent infection syndrome in infancy.Four infants with hyperimmunoglobulinemia E presented with a persistent papulovesicular rash and eosinophilia. serum IgE levels and specific anti-staphylococcus aureus IgE antibodies were studied during the first year of life. Increased anti-S aureus IgE antibodies were an early indicator of the disease; they appeared as soon as 7 weeks of age in patient 1 and before 1 year of age for the other patients. These antibodies were detected before the development of deeper staphylococcal infections. Prompt diagnosis and treatment are important, as they may prevent long-term infectious complications.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
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