Agammaglobulinemia and lack of immunization protection in exudative atopic dermatitis
ABSTRACT Atopic dermatitis is very frequent in the first 6 months of life, and the severe exudative form of this skin disorder is by no means rare. Failure to achieve immunization protection is a potentially life-threatening complication of exudative atopic dermatitis that may go unrecognized. We report the case of a 6-month-old infant with severe exudative atopic dermatitis in whom hypoproteinemia and agammaglobulinemia were attributed to the massive exudation after exclusion of other possible causes. The patient failed to respond to standard immunization, and adequate protection with a good antibody response could be achieved only after exudation from the skin lesions had been treated by intensive topical skin therapy and multiple intravenous immunoglobulin substitution. Two otherwise similar earlier case reports did not investigate the immune status. Therefore, to the best of our knowledge, this is the first report to draw attention to absence of immunization protection in exudative atopic dermatitis. Conclusion: We hope that our case report will motivate pediatricians to check the immunization status of patients with exudative atopic dermatitis and take the necessary steps to improve their care.
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ABSTRACT: The incidence of transient hypogammaglobulinaemia of infancy (THI) detected in a major paediatric centre over a 10 year period was examined. A total of 2468 subjects less than 2 years of age had an IgG measurement taken between July 1979 and March 1990. Subjects with known immunodeficiencies were excluded. Fifteen patients were classified as having THI with an initial IgG level less than the fifth centile followed by a second measurement within the normal range. A further 24 patients were identified as having possible THI with a single low IgG concentration. There were 60,174 live births each year in Victoria in the years 1979-88. This gives an incidence of proved THI of 23 per 10(6) births, and including proved and probable THI an incidence of 61 per 10(6) live births. Of those patients with proved THI 12/15 had symptoms of either atopic disease or food allergy/intolerance and three had gastrointestinal symptoms without any evidence of atopic disease. At presentation 12/15 (80%) were IgA deficient and 9/15 had IgM concentrations less than the 20th centile for age. It is suggested that in view of the preponderance of atopic and food intolerant patients that subclinical protein loss from the bowel due to allergic inflammation may be a contributing factor to the development of THI in some patients.Archives of Disease in Childhood 04/1994; 70(3):183-6. DOI:10.1136/adc.70.3.183 · 2.91 Impact Factor
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ABSTRACT: Transient hypogammaglobulinemia of infancy (THI) is characterized by recurrent infections and one or more reduced serum immunoglobulin (Ig) levels. Usually, this clinical picture resolves spontaneously by 3 yr of age. However, hypogammaglobulinemia persists until adolescence in some patients. In recent years, those patients have been classified as undefined/unclassified hypogammaglobulinemia (UCH). We aimed to evaluate the clinical and immunologic features of patients with THI and UCH considering age of recovery and to assess relationships between hypogammaglobulinemia, infections, and allergic manifestations. We reviewed the medical records of children followed with a diagnosis of hypogammaglobulinemia from 2001 to 2007. Patients with decreased levels (<2 s.d.) of one or more major Ig isotypes (IgG, IgA, IgM) with normal antibody responses and lymphocyte subpopulations were included (n = 374). Those patients whose Igs normalized during the follow-up period were classified as THI and the others as UCH. The THI group consisted of 71 patients (27 females, 44 males) with a mean recovery age of 68.87 +/- 36.5 months. About 95% of patients with THI recovered before 10 yr of age. The UCH group consisted of 303 patients (105 females, 198 males) with a mean age at diagnosis of 52 +/- 42 months. The most common presenting manifestations in the THI and UCH groups were upper respiratory tract infections (URTIs), lower respiratory tract infections, and asthma (42%, 50%, and 52% in the THI group vs. 39%, 53%, and 55% in the UCH group, respectively). In the THI group, the prevalence of atopic disease was related to age and found to be increased markedly after 44 months. In all patients, the prevalence of asthma was independently and positively associated with family history of atopy and age, whereas it was negatively associated with recurrent URTIs. Patients with THI and UCH have similar clinical and immunologic features. The normalization of Igs may be delayed in a majority of the patients with hypogammaglobulinemia. This observation may be a contribution to the classical definition and diagnostic criteria for THI.Pediatric Allergy and Immunology 08/2010; 21(5):843-51. DOI:10.1111/j.1399-3038.2010.01010.x · 3.86 Impact Factor
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ABSTRACT: We sought to describe the clinical outcomes of eight pediatric patients diagnosed with atopic dermatitis (AD) and hypogammaglobulinemia through retrospective review of medical records. All patients presented with severe facial AD. The mean and median ages of diagnosis of hypogammaglobulinemia were 6.2 months and 6.5 months, respectively, with a mean immunoglobulin G (IgG) level of 156 mg/dL. Seven of the eight patients identified in our search demonstrated simultaneous improvement in AD and serum IgG levels within 2 years of initial presentation, suggesting a diagnosis of transient hypogammaglobulinemia. The remaining patient demonstrated normalization by age 6, but no IgG levels had been measured between initial presentation and age 6. The five patients who were tested for specific antibody response to tetanus and Haemophilus influenzae type b vaccination all produced protective responses. All eight patients initially presented with high serum IgE levels. On initial evaluation, three patients had leukocytosis (white blood cell count >18,000 cells/μL), and six had peripheral blood eosinophilia. Three patients outgrew their AD by age 5, and five had clinically good to excellent control of their AD at their last visit, coincident with normalization of IgG levels. Although severe AD and immunoglobulin deficiency may rarely be associated with complex immunodeficiency disorders, our observations suggest that, with careful immunologic monitoring and diligent skin care, most children who present with severe AD and hypogammaglobulinemia exhibit improvement in dermatitis and serum IgG levels within 2 years of onset without major complications.Pediatric Dermatology 08/2011; 29(1):73-8. DOI:10.1111/j.1525-1470.2011.01477.x · 1.52 Impact Factor