1/7. 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 = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
2/7. A hyperimmunoglobulin E syndrome with normal chemotaxis in vitro and defective leukotaxis in vivo.A 26-yr-old male with a lifelong history of atopic dermatitis and recurrent severe staphylococcal abscesses was found to have hyperimmunoglobulinemia E. Evaluation of both the humoral and cellular aspects of chemotaxis in vitro showed both neutrophils and monocytes to be normal. However, quantitative neutrophil migration in vivo was significantly suppressed using the patient's own serum as the attractant. This defective migration in vivo was partially corrected by serum from normal donors as the attractant and also partially corrected following plasma infusion in this patient. Evaluation of quantitative leukocyte migration in vivo may be most useful in patients suspected of defects of leukocyte mobility.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
3/7. Defective neutrophil chemotaxis and bactericidal power in a child with hyperimmunoglobulinemia E.An 11-year-old boy with a life-long history of atopic-like dermatitis and recurrent staphylococcal abscesses was found to have defective neutrophil chemotaxis, impaired-T-lymphocyte functions, hyperimmunoglobulinemia E, and delayed neutrophil bactericidal power. This latter defect has never been found in such patients. The patient's mother revealed a panhypogammaglobulinemia, while his sister and maternal grandmother who had repeated infections were immunologically normal.- - - - - - - - - - ranking = 5keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
4/7. plasmapheresis treatment in patients with severe atopic keratoconjunctivitis.A 19-year-old woman with hyperimmunoglobulinemia E (the hyper-IgE syndrome [HIE]) and a 62-year-old man with atopic dermatitis experienced dramatic improvement in their chronic ocular symptoms and signs of atopic keratoconjunctivitis after plasmapheresis was instituted. Both patients had previously received topical and oral corticosteroids as well as topical cromolyn sodium without significant beneficial effect. The authors recommend a trial of plasmapheresis in selected patients with recalcitrant and debilitating atopic keratoconjunctivitis when standard therapy proves unsuccessful.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
5/7. Vernal conjunctivitis in the hyperimmunoglobulinemia E syndrome.Hyper-IgE syndrome (HIE) appears to be related to an immunoregulatory imbalance characterized by severe deficiency of suppressor T cells, elevated levels of IgE antibodies, and repeated infection of various organ systems. We report the association of HIE syndrome in two definite cases and one probable case of vernal conjunctivitis. This association suggests that T cell-mediated imbalance may be one factor in the pathogenesis of vernal conjunctivitis.- - - - - - - - - - ranking = 4keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
6/7. 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.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
7/7. Defective neutrophil chemotaxis and hyperimmunoglobulinemia E-a reversible defect?An eleven-month-old boy is presented with chronic atopic dermatitis and recurrent infections of the skin and respiratory tract, including subcutaneous abscesses. Immunological studies disclosed a neutrophil chemotactic defect, blood eosinophilia and serum hyper IgE. The clinical and analytical data are similar to those of patients previously dermatitis reversed the chemotatic defect, the blood eosinophilia and the clinical symptoms.- - - - - - - - - - ranking = 4keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |