Hyper Immunoglobulin E Syndrome (HIES): Report of a New Case Treated with Omalizumab and Dexametasone

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4934-4934
Author(s):  
Luis Fernando Cortázar-Benítez ◽  
R.M. Rojas Sotelo ◽  
Pablo Vargas-Viveros ◽  
Rafael Hurtado-Monroy ◽  
Catalina Romo Aguirre ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Conflicts Of Interest ◽  
Upper Airway ◽  
Severe Case ◽  
Skin Lesions ◽  
High Serum ◽  
Response To Treatment ◽  
Rapid Improvement ◽  
Serum Ige ◽  
Airway Infections

Abstract Abstract 4934 The classical triad of eczema, recurrent skin and lung infections and high serum IgE, other connective tissue, cardiac and brain abnormalities are the accepted diagnostic criteria for HIES. The treatment of HIES is not established due to the scarce number of published cases, however is limited to skin local measures and supportive care. Herein we inform a severe case with response to Omalizumab, a monoclonal anti IgE antibody in combination with Dexametasone. A 36 year old male with family history of asthma and atopic dermatitis (AD). Since child, the patient had allergies and AD. At 8 years he presented asthma and recurrent upper airway infections at 2 to 3 times per year and 7 dental pieces removal. In the last 3 years he noted important increase of AD with very intense and disabling pruritus without response to treatment and progression to generalized erithrodermia, nodes in legs and arms, and skin trasudate, axilar and inguinal lymph nodes (6×4 cm). Laboratory positive test were eosinophils 6%, serum IgE 29,280 IU/mL. Skin biopsy showed psoriasis-like dermatitis, micro abscesses and dermatopathic lymphadenopathy. We started treatment with trimetoprim/Sulfamethoxazole 80/400 mg twice a day, oral Dexametasone 20 mg weekly and Omalizumab 200 mg subcutaneously every two weeks. After 15 days of treatment there was a rapid improvement of skin derangement and IgE levels dropped to 2000 IU/mL after 16 weeks of treatment. Patient continues on treatment with no side effects and pruritus disappears. Omalizumab is a monoclonal antibody against IgE recently introduced to the treatment of HIES in a few patients reported since 2008 with excellent response, along with intensive care of skin lesions, prompt antibiotic and antimycotic treatment for infections are the mainstay of HIES management. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Topical Application of Herbal Mixture Extract Inhibits Ovalbumin- or 2,4-Dinitrochlorobenzene-Induced Atopic Dermatitis

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Soon Re Kim ◽  
Han-Seok Choi ◽  
Hye Sook Seo ◽  
Youn Kyung Choi ◽  
Yong Cheol Shin ◽  
...  
Keyword(s):  
Atopic Dermatitis ◽  
Topical Application ◽  
Immunoglobulin E ◽  
Elevated Serum ◽  
Ethanol Extract ◽  
Inflammatory Cells ◽  
Skin Lesions ◽  
Serum Ige ◽  
Beneficial Effects ◽  
Blood Eosinophils

KM110329 is four traditional herbal medicine mixtures with anti-inflammatory properties. Atopic dermatitis (AD) is an inflammatory skin disease associated with enhanced T-helper2 (Th2) lymphocyte response to allergens that results in elevated serum eosinophil and Immunoglobulin E (IgE) levels and leukocyte infiltration in atopic skin sites. In this study, we investigated the effect of topical application of KM110329 ethanol extract on the ovalbumin (OVA) or 2,4-dinitrochlorobenzene- (DNCB-) induced AD mouse models. For that purpose, we observed the effects of KM110329 on blood eosinophils, skin mast cells, production of serum IgE, and expression of cytokine mRNA in the atopic dermatitis skin lesions of OVA allergen- or DNCB-treated BALB/c mice. KM110329 significantly reduced blood eosinophils cell numbers in OVA or DNCB-treated BALB/c mice. Histological analyses demonstrated decreased mast cell count as well as dermal infiltration by inflammatory cells. In the skin lesions, mRNA expression of interleukine (IL)-4, IL-13, and IL-17 was inhibited by KM110329. KM110329 also suppressed the production of serum IgE level in both the OVA- and DNCB-induced atopic dermatitis model. Taken together, our results showed that topical application of KM110329 extracts exerts beneficial effects in AD symptoms, suggesting that KM110329 might be a useful candidate for the treatment of AD.

scholarly journals The association of absolute eosinophil count, serum immunoglobulin E and spirometry with co-morbid bronchial asthma in patients with allergic rhinitis

2019 ◽  
Vol 9 (35) ◽  
pp. 151-154
Author(s):  
Neelima Vijayan ◽  
Padmanabhan Karthikeyan ◽  
Nirmal Coumare Venkataramanujam ◽  
Ramiya Ramachandran Kaipuzha ◽  
Davis Thomas Pulimoottil
Keyword(s):  
Allergic Rhinitis ◽  
Bronchial Asthma ◽  
Eosinophil Count ◽  
Nasal Polyposis ◽  
Immunoglobulin E ◽  
Signs And Symptoms ◽  
Response To Treatment ◽  
Serum Ige ◽  
Absolute Eosinophil Count ◽  
Acute Attacks

Abstract OBJECTIVE. This study aimed to analyse the association of absolute eosinophil count (AEC), serum IgE and spirometry with co-morbid bronchial asthma in patients with allergic rhinitis. MATERIAL AND METHODS. This study involved 50 patients with signs and symptoms of allergic rhinitis who underwent a clinical examination and various tests, including spirometry, and were followed up regularly. Patients found to have bronchial asthma or nasal polyposis were treated accordingly. RESULTS. The study found the prevalence of bronchial asthma in patients with allergic rhinitis to be 58% and that the severity of bronchial asthma was reduced significantly, with lesser acute attacks and reduced hospitalizations with the effective treatment of allergic rhinitis (p=0.064). CONCLUSION. This study showed that elevated AEC and serum IgE were significantly associated with co-existing allergic rhinitis and bronchial asthma and increased the chance of co-existence of these two pathologies. Spirometry is a useful tool for observing the response to treatment.

scholarly journals Partial and Transient Clinical Response to Omalizumab in IL-21-Induced Low STAT3-Phosphorylation on Hyper-IgE Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Cesar Daniel Alonso-Bello ◽  
María del Carmen Jiménez-Martínez ◽  
María Eugenia Vargas-Camaño ◽  
Sagrario Hierro-Orozco ◽  
Mario Alberto Ynga-Durand ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
High Serum ◽  
Recurrent Infections ◽  
Dominant Form ◽  
Cutaneous Manifestations ◽  
Serum Ige ◽  
Hyper Ige Syndrome ◽  
Stat3 Phosphorylation ◽  
Eczematous Dermatitis ◽  
Pulmonary Abnormalities

Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by elevated levels of immunoglobulin E (IgE), eczematous dermatitis, cold abscesses, and recurrent infections of the lung and skin caused by Staphylococcus aureus. The dominant form is characterized by nonimmunologic features including skeletal, connective tissue, and pulmonary abnormalities in addition to recurrent infections and eczema. Omalizumab is a humanized recombinant monoclonal antibody against IgE. Several studies reported clinical improvement with omalizumab in patients with severe atopic eczema with high serum IgE level. We present the case of a 37-year-old male with HIES and cutaneous manifestations, treated with humanized recombinant monoclonal antibodies efalizumab and omalizumab. After therapy for 4 years, we observed diminished eczema and serum IgE levels.

scholarly journals Immunoglobulin E levels and pregnancy-induced hypertension: Japan Environment and Children’s Study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hyo Kyozuka ◽  
◽  
Tsuyoshi Murata ◽  
Toma Fukuda ◽  
Yuta Endo ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Late Onset ◽  
First Trimester ◽  
High Serum ◽  
Pregnancy Induced Hypertension ◽  
Serum Ige ◽  
Japanese Cohort ◽  
Increased Risk ◽  
Induced Hypertension ◽  
Hypertensive Disorders In Pregnancy

AbstractHigh serum immunoglobulin E (IgE) levels are associated with cardiovascular events. We aimed to evaluate the association between total IgE levels during the first trimester of pregnancy and pregnancy-induced hypertension (PIH) development in a large Japanese cohort. We analysed data pertaining to singleton primipara pregnancies recorded in the Japan Environment and Children’s Study involving births from 2011 to 2014. Serum IgE levels were determined using the immunonephelometric technique. High serum IgE was defined as level ≥ 170 IU/ml. Hypertensive disorders in pregnancy (HDP) were categorized into early onset (Eo) PIH (developed < 34 weeks) or late onset (Lo) PIH (developed ≧ 34 weeks). A multiple logistic regression model was used to estimate the risk of high serum IgE levels on PIH, Eo-PIH, and Lo-PIH. Overall, 32,518 participants were enrolled. The prevalence of total, Eo-, and Lo-PIH was 3.2%, 0.6%, and 2.3%, respectively. Patients with high serum IgE levels had an increased risk of Lo-HDP (adjusted odds ratio [aOR]:1.19, 95% confidence interval 1.01–1.40). No correlation was found with either PIH (total) or Eo-PIH. High serum IgE levels during the first trimester were associated with the risk of Lo-PIH. Our results could influence and shape further research regarding the pathogenesis of Lo hypertension.

scholarly journals Topical Application ofChrysanthemum indicum L.Attenuates the Development of Atopic Dermatitis-Like Skin Lesions by Suppressing Serum IgE Levels, IFN-γ, and IL-4 in Nc/Nga Mice

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Sunmin Park ◽  
Jung Bok Lee ◽  
Suna Kang
Keyword(s):  
Atopic Dermatitis ◽  
Topical Application ◽  
Immunoglobulin E ◽  
Clinical Symptoms ◽  
Secondary Infection ◽  
Skin Lesions ◽  
Mrna Levels ◽  
Dorsal Skin ◽  
Gene Expressions ◽  
Serum Ige

Chrysanthemum indicum L. (CIL) is widely used as an anti-inflammatory agent in Asia and our preliminary study revealed that CIL reduced interleukin (IL)-4 and IL-13 in 2,4-dinitrochlorobenzene (DNCB)-treated HaCaT cells, a human keratinocyte cell line. We investigated the atopic dermatitis (AD) effect of topically applied CIL in mice with AD-like symptoms. After topical application of 1,3-butylen glycol (control), CIL-Low (5%), CIL-High (30%), or 0.1% hydrocortisone (HC) on the AD-like skin lesions in DNCB-treated NC/Nga mice for 5 weeks, the ear thickness, mast cell infiltration, and serum immunoglobulin E (IgE), IgG1, IL-4 and interferon (IFN)-γwere measured. The gene expressions of IL-4, IL-13, and IFN-γin the dorsal skin were assayed. CIL treatment dosedependently reduced severity of clinical symptoms of dorsal skin, ear thickness, and the number of mast cells and eosinophils. CIL-High significantly decreased serum IgE, IgG1, IL-4, and IFN-γlevels and reduced mRNA levels of IFN-γ, IL-4, and IL-13 in dorsal skin lesion. The improvement by CIL-High was similar to HC, but without its adverse effects such as skin atrophy maceration, and secondary infection. In conclusion, CIL may be an effective alternative substance for the management of AD.

scholarly journals Two Sisters with Idiopathic Pulmonary Hemosiderosis

2007 ◽  
Vol 14 (8) ◽  
pp. 490-493 ◽  
Author(s):  
Mehmet Gencer ◽  
Erkan Ceylan ◽  
Muharrem Bitiren ◽  
Ahmet Koc
Keyword(s):  
Chest Radiography ◽  
Immunoglobulin E ◽  
High Serum ◽  
Hemoglobin Level ◽  
Unknown Etiology ◽  
Total Serum ◽  
Diffuse Infiltration ◽  
Serum Ige ◽  
Pulmonary Hemosiderosis ◽  
Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE) level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%. Her chest radiography revealed diffuse infiltration. She died due to respiratory failure. The second patient was 18 years of age. She had also suffered from dry cough and gradually increasing dyspnea for two years. She had partial pneumothorax in the right lung and diffuse infiltration in other pulmonary fields on chest radiography. Her hemoglobin level was 99 g/L, total serum IgE level was 1200 U/mL and eosinophilia was 8%. IPH was diagnosed by open lung biopsy. All these findings suggested that familial or allergic factors, as well as immunological factors, might have contributed to the etiology of IPH.

scholarly journals Phenotypic distinctions of BLM- and RMI1-associated Bloom syndrome

2021 ◽  
Author(s):  
Ipek Ilgin Gonenc ◽  
Nursel Huriye Elcioglu ◽  
Carolina Martinez Grijalva ◽  
Seda Aras ◽  
Nadine Grossmann ◽  
...  
Keyword(s):  
Upper Airway ◽  
Skin Lesions ◽  
Bloom Syndrome ◽  
Growth Delay ◽  
Homozygous Mutation ◽  
Loss Of Function ◽  
Primary Microcephaly ◽  
Whole Exome ◽  
Airway Infections ◽  
Turkish Family

Bloom syndrome (BS) is an autosomal recessive disease with characteristic clinical features of primary microcephaly, growth deficiency, skin lesions, cancer predisposition, and immunodeficiency. Here, we report the clinical and molecular findings of eight patients from six families diagnosed with BS. We identified causative mutations in all families, three different homozygous mutations in BLM and one causative homozygous mutation in RMI1. The homozygous c.581_582delTT (p.Phe194*) and c.3164G>C (p.Cys1055Ser) mutations in BLM have already been reported in BS patients, while the c.572_573delGA (p.Arg191Lysfs*4) is novel. Interestingly, whole-exome sequencing revealed a homozygous loss-of-function mutation in RMI1 in two BS patients of a consanguineous Turkish family. All BS patients had primary microcephaly, intrauterine growth delay, and short stature, presenting the phenotypic hallmarks of BS. However, a narrow face, skin lesions, and upper airway infections were observed only in some of the patients. Overall, patients with homozygous BLM mutations had a more severe BS phenotype compared to patients carrying the homozygous RMI1 mutation, especially in terms of immunodeficiency and associated recurrent infections. Low-level immunoglobulins were observed in all BLM-mutated patients, emphasizing the immunodeficiency profile of the disease, which should be considered as an important phenotypic characteristic of BS, especially in the current Covid-19 pandemic era.

scholarly journals Study of serum IgE levels in childhood asthma in Barabanki region, India

2018 ◽  
Vol 5 (5) ◽  
pp. 1755
Author(s):  
Ekansh Rathoria ◽  
Utkarsh Bansal ◽  
Abhishek Gupta ◽  
Nyay Bhai Gupta ◽  
Ravindra Ahuja ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Elevated Serum ◽  
Allergic Diseases ◽  
Normal Subjects ◽  
High Serum ◽  
Serum Immunoglobulin ◽  
Age Group ◽  
Mean Values ◽  
Serum Ige ◽  
Asthmatic Patients

Background: Elevated Serum Immunoglobulin E (IgE) levels are characteristic of most of the allergic diseases including asthma. Most of the asthma patients are allergic to inhaled antigens and chemical antigens, which lead to their sensitization and induce a state of hypersensitivity that is IgE mediated hypersensitivity reaction. The aim of this study was to estimate and compare serum IgE levels in childhood asthmatics and in normal subjects and to obtain the relationship between serum IgE levels and severity of asthma.Methods: A stratified sample of 58 patients within the age group of 5-15 years including 36 male and 22 female asthmatic patients and 58 healthy controls within the same age group were included in this study and classified according to GINA classification 2016. Serum IgE levels were estimated by using ELISA kit.Results: Mean IgE levels ranged from 163.82 IU/mL in normal subjects to 881.81 IU/mL in asthmatics. The mean values of Serum IgE levels in mild, moderate and severe asthmatic children were 625.25 IU/mL, 871.77 IU/mL, 1225.05 IU/mL respectively.Conclusions: High Serum Immunoglobulin E levels were found in childhood asthmatics as compared to normal subjects. Serum IgE levels were found to increase as the severity of asthma increased. The variability in each grade of asthma was very large so we could not find any statistically significant correlation.

scholarly journals Immunoglobulin E Levels and Hypertensive Disorders of Pregnancy: Japan Environment and Children’s Study

2020 ◽  
Author(s):  
Hyo Kyozuka ◽  
Tuyoshi Murata ◽  
Toma Fukuda ◽  
Yuta Endo ◽  
Akiko Yamaguchi ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Early Onset ◽  
Immunoglobulin E ◽  
Late Onset ◽  
First Trimester ◽  
High Serum ◽  
Hypertensive Disorders Of Pregnancy ◽  
Serum Ige ◽  
Hypertensive Disorders ◽  
Increased Risk

Abstract High serum immunoglobulin E (IgE) levels are associated with cardiovascular events. We aimed to evaluate the association between total IgE levels during the first trimester of pregnancy and hypertensive disorders of pregnancy (HDP) development in a large Japanese cohort. We analysed data pertaining to singleton primipara pregnancies recorded in the Japan Environment and Children’s Study involving births in 2011–2014. First trimester’s serum IgE levels were determined using the immunonephelometric technique. High serum IgE was defined as IgE levels ≥170 IU/ml. A multiple logistic regression model was used to estimate the risk of high serum IgE levels on HDP, comprising early-onset and late-onset hypertension. A total of 32,518 participants were enrolled. The prevalence of total, early-onset, and late-onset HDP was 3.2%, 0.6%, and 2.3%, respectively. Patients with high serum IgE levels had an increased risk of late-onset hypertension (adjusted odds ratio [aOR]: 1.19, 95% confidence interval: 1.01–1.40). No correlation was found with either HDP (total) or early-onset hypertension (aOR: 1.15 and 0.85, 95% confidence interval: 0.99–1.32 and 0.60–1.21, respectively). High serum IgE levels during the first trimester are associated with late-onset hypertension. Our results could influence and shape further research regarding the pathogenesis of late-onset hypertension.

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