RESUMO
BACKGROUND: Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized. OBJECTIVE: We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement. METHODS: We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013. Skin phenotype, histopathology, presence of KIT mutation in the skin, and assessment of systemic involvement according to World Health Organization (WHO) criteria were prospectively investigated. RESULTS: Of 243 patients with cutaneous mastocytosis, 34 (14%) were given a diagnosis of TMEP. The diagnosis of systemic mastocytosis was established in 16 patients (47%) with TMEP. Three patients (9%) had aggressive systemic mastocytosis (C-findings according to WHO). In all, 32 patients (94%) exhibited at least 1 mast cell activation-related symptom. LIMITATIONS: Patient recruitment was undertaken at 2 referral centers with expertise in the diagnosis and treatment of mastocytosis so that the clinical findings and incidence of systemic involvement may be overestimated in comparison with the overall population of patients with TMEP. CONCLUSION: TMEP accounts for about 14% of patients with cutaneous mastocytosis. The disease manifests as mast cell activation symptoms in almost all patients and can be associated with systemic involvement in about 50% of cases.
Assuntos
Mastocitose Sistêmica/patologia , Telangiectasia/patologia , Urticaria Pigmentosa/patologia , Adulto , Fatores Etários , Biópsia por Agulha , Estudos de Coortes , Progressão da Doença , Feminino , França , Testes Hematológicos , Humanos , Imuno-Histoquímica , Masculino , Mastocitose Sistêmica/epidemiologia , Mastocitose Sistêmica/fisiopatologia , Pessoa de Meia-Idade , Prognóstico , Encaminhamento e Consulta , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Telangiectasia/fisiopatologia , Urticaria Pigmentosa/epidemiologia , Urticaria Pigmentosa/fisiopatologiaAssuntos
Anestesia Geral/efeitos adversos , Espasmo Brônquico/etiologia , Sobrepeso/complicações , Adulto , Idoso , Alérgenos/imunologia , Anestésicos/imunologia , Espasmo Brônquico/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Sobrepeso/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Testes CutâneosAssuntos
Anafilaxia/diagnóstico , Mastócitos/fisiologia , Mastocitose/diagnóstico , Mutação/genética , Proteínas Proto-Oncogênicas c-kit/genética , Pele/patologia , Adulto , Biomarcadores/metabolismo , Estudos de Coortes , Análise Mutacional de DNA , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Triptases/metabolismoRESUMO
BACKGROUND: The clinical significance of newly available platforms for specific IgE measurement must be evaluated. However, data are lacking for NOVEOS (Hycor), especially for food allergens. OBJECTIVE: We compared the technical and clinical performance of two platforms (ImmunoCAP and NOVEOS) to measure specific IgE to 10 food allergens. METHODS: Sera from 289 clinically characterized patients were tested for IgE specific for six food allergen extracts (egg white, cow's milk, peanut, hazelnut, fish, and shrimp) and four molecular allergens (Gal d 1, Bos d 8, Ara h 2, and Cor a 14). Specific IgE measurements were carried out using ImmunoCAP and NOVEOS methods. Food allergy diagnoses were established according to international guidelines. RESULTS: A strong correlation (ρ > 0.9) was present between the two platforms whereas specific IgE concentrations measured with NOVEOS were consistently lower (mean, -15%) than with ImmunoCAP. NOVEOS and ImmunoCAP provided similar overall odds ratios and relative risks for food allergy diagnosis with both allergen extracts and molecular allergens. When all 10 allergens were considered, NOVEOS provided better receiver operating characteristic curves (P = .04). Finally, we found that the most discordant results were observed with hazelnut and peanut extracts and were related to cross-reactive carbohydrate determinants for these two with ImmunoCAP. CONCLUSIONS: Specific IgE determination by either ImmunoCAP or NOVEOS (odds ratios of allergy, 25.1 or 33.0, respectively) is highly informative regarding the risk of allergy in the selected population. The NOVEOS platform presents the advantage of being less affected by unwanted reactivity owing to carbohydrate determinant-specific IgE while requiring a 10-fold lower test sample volume.
Assuntos
Alérgenos , Hipersensibilidade Alimentar , Imunoglobulina E , Humanos , Hipersensibilidade Alimentar/diagnóstico , Hipersensibilidade Alimentar/imunologia , Imunoglobulina E/sangue , Alérgenos/imunologia , Feminino , Masculino , Adulto , Adolescente , Criança , Pré-Escolar , Pessoa de Meia-Idade , Adulto Jovem , Lactente , Curva ROC , Sensibilidade e Especificidade , Imunoensaio/métodos , IdosoAssuntos
Venenos de Artrópodes/uso terapêutico , Dessensibilização Imunológica , Himenópteros , Mastocitose Sistêmica/imunologia , Mastocitose Sistêmica/terapia , Anafilaxia/diagnóstico , Anafilaxia/imunologia , Anafilaxia/terapia , Animais , Venenos de Artrópodes/efeitos adversos , Venenos de Artrópodes/imunologia , Dessensibilização Imunológica/efeitos adversos , Dessensibilização Imunológica/métodos , Feminino , Humanos , Masculino , Mastocitose Sistêmica/diagnósticoRESUMO
Hymenoptera venom allergy (HVA) is the leading cause of anaphylactic reactions in adults and the second most common cause in children. Venom immunotherapy (VIT) is used to elicit an immune tolerance against hymenoptera venom in allergic patients and is based on the administration of purified venom extracts regularly for defined periods. The protocols of administration include 2 phases: an up-dosing phase that incrementally reaches the final dose resulting in a protective effect, and a maintenance phase in order to obtain the sustained effect. The goal of this review is to detail the efficacy and the safety of the up-dosing phase also named rush. Pathophysiological mechanisms, indications of VIT and technical aspects of up-dosing protocol are also covered.
RESUMO
OBJECTIVES: Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Secondary objectives were to evaluate the ability of bone marrow tryptase (BMT) level to identify patients with FF, and to describe bone involvement in SM. METHODS: We analyzed retrospectively all consecutive patients seen in our expert center, with a diagnosis of SM according to the 2001 WHO criteria, and with complete bone assessment. We collected data about lifetime fractures, types of cutaneous manifestations, degranulation symptoms, blood and BMT levels, bone mineral density assessed by densitometry and KIT mutation. We performed a univariate analysis investigating the factors associated with FF and then a logistic multivariable regression analysis. We assessed the ability of bone marrow tryptase to identify patients with FF. RESULTS: Eighty-nine patients with SM were included. Thirty-six patients (40.4%) suffered from osteoporosis and twenty-five (28.1%) experienced lifetime FF. Univariate analysis identified age at diagnosis and disease onset, presence of telangiectasia macularis eruptiva perstans, digestive symptoms, mast cells activation symptoms, elevated BMT, low femoral and lumbar BMD, as associated with FF. Multivariate analysis identified elevated BMT, low femoral T score and older age at diagnosis as independently associated with FF. CONCLUSIONS: Low femoral T-score, BMT level, and older age at diagnosis are markers associated with FF in SM. BMT may represent an important biomarker to predict FF in SM patients.
Assuntos
Fraturas Ósseas/complicações , Fraturas Ósseas/epidemiologia , Mastocitose Sistêmica/complicações , Mastocitose Sistêmica/epidemiologia , Adulto , Medula Óssea/enzimologia , Demografia , Feminino , Fraturas Ósseas/diagnóstico , Fraturas Ósseas/enzimologia , Humanos , Masculino , Mastocitose Sistêmica/enzimologia , Pessoa de Meia-Idade , Análise Multivariada , Prevalência , Curva ROC , Fatores de Risco , Triptases/metabolismoRESUMO
BACKGROUND: There is considerable debate about the prevalence and relevance of food allergy (FA) in atopic dermatitis (AD). The aim of this study was to investigate the prevalence of and risk factors for FA in a cohort of children with AD attending a multidisciplinary paediatric allergy clinic. METHODS: The analysis was performed on 386 children (50.8% boys, median age 4 years) consecutively evaluated for AD. A diagnosis of FA was established on positive skin tests and/or a specific IgE value or a positive oral food challenge. RESULTS: Point prevalence of FA was 17.8%. Egg, peanuts, milk, tree nut and mustard accounted for 93% of cases. 37.7% of children had ≥2 positive food reactions. Risk factors associated with FA were young age (OR=7.9 when ≤2 years compared with ≥5 years), moderate to severe AD (OR=7.8 for severe and 2.4 for moderate AD) and onset of AD before 3 months of age (OR=5.7). CONCLUSION: Point prevalence of FA in children with AD is lower than initially reported in patients recruited in a paediatric allergology setting. Children≤2 years of age with early-onset or severe AD are at higher risk of FA and may be candidates for FA evaluation.
Assuntos
Dermatite Atópica/complicações , Hipersensibilidade Alimentar/complicações , Hipersensibilidade Alimentar/epidemiologia , Adolescente , Criança , Pré-Escolar , Árvores de Decisões , Feminino , Hipersensibilidade Alimentar/diagnóstico , Humanos , Lactente , Masculino , Prevalência , Fatores de RiscoRESUMO
Since its description by noon in 1911, desensitization, or allergen specific immunotherapy (SIT), has been largely used in respiratory allergic diseases treatment. It remains the only etiologic treatment for allergic diseases. The development of the sublingual route and new forms of medication, as an alternative to subcutaneous injection, has led to large scale clinical trials. Many of them had been performed with allergen tablets, particularly in the field of pollen allergy. These studies have confirmed that SIT is efficient in reducing all respiratory allergic symptoms. Data on long-term benefits and sustained efficacy after stopping treatment have also been published. These show an impact on natural history of allergic disease, in particular, a reduction in the risk of asthma in desensitized rhinitic subjects and in the acquisition of new sensitivities. The basic mechanisms of immunotherapy are becoming better understood and allow us to envisage improvements in this therapeutic method in the future. The sublingual route appears to be safer with a better safety profile. This may lead to an extension of allergen specific immunotherapy indications in patients with respiratory allergic diseases.