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1.
Br J Dermatol ; 2024 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-38863109

RESUMEN

BACKGROUND: Therapeutic patient education (TPE) is recommended for children with atopic dermatitis (AD), but no consensus has been reached on the optimal tailoring of delivery. While repeated multidisciplinary group education sessions have shown effectiveness, the benefits of one-on-one educational interventions led by nurses for children with AD have not yet been assessed. OBJECTIVES: To assess the benefits of additional, well-structured, 1-h nurse-led individual TPE interventions in children with AD and their families compared with standard care alone. METHODS: Children with moderate-to-severe AD and their parents were randomized to receive a 1-h nurse-led education session in addition to standard care vs. standard care alone. The primary outcome was the area under the curve (AUC) of the SCORing of Atopic Dermatitis index (SCORAD) from baseline to week 24 (lower AUC values represent better long-term control of the disease). RESULTS: In our study, 176 patients were randomized across 11 centres, and 153 were included in the full analysis set. The mean (SD) age was 4.47 (4.57) years. By week 24, there were no significant differences in the AUCs of the SCORAD between the two groups (P = 0.3). Secondary outcomes including patient-reported severity and quality of life [AUCs of the patient-oriented SCORAD (PO-SCORAD) and Infants' Dermatitis Quality of Life Index (IDLQI), Children's Dermatitis Quality of Life Index (CDLQI) and Family Dermatitis Quality of Life Index (FDLQI)] were not significantly different between the two groups. The only significant change observed in the intervention group, when compared with the one receiving standard care, was a decrease in topical steroid phobia, as assessed by the topical corticosteroid phobia (TOPICOP) score. Prespecified subgroup analyses showed that disease severity in the intervention group was significantly lower throughout the study, compared with the standard-care group when participants had moderate AD at baseline (n = 47); while participants with severe AD at baseline (n = 106) did not show benefit from the intervention. Participants showed no additional benefit from the intervention regardless of age group. CONCLUSIONS: This study did not show any additional effectiveness, in long-term severity control, of a 1-h nurse-led TPE intervention in children with AD treated with standard care, compared with those treated with standard care alone. However, it should be noted that the intervention reduced the fear of using topical steroids and may be beneficial for patients in the subgroup with moderate AD.

2.
Eur J Pediatr ; 182(9): 4133-4141, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37432503

RESUMEN

The purpose of the study is to highlight clinical signs that are either suggestive of or against the diagnosis of AHEI to improve diagnosis and management. The medical records of children under 3 years old diagnosed with AHEI were retrospectively reviewed. Clinical data and photographs were reviewed by three independent experts, and the cases were classified as probable, doubtful, or unclear AHEI. Of the 69 cases of children diagnosed with AHEI included in 22 centers, 40 were classified as probable, 22 as doubtful, and 7 as unclear. The median age of patients with probable AHEI was 11 months [IQR 9-15], and they were in overall good condition (n = 33/40, 82.5%). The morphology of the purpura was targetoid in 75% of cases (n = 30/40) and ecchymotic in 70% of cases (n = 28/40) and affected mostly the legs (n = 39/40, 97%), the arms (n = 34/40, 85%), and the face (n = 33/40, 82.5%). Edema was observed in 95% of cases and affected mostly the hands (n = 36/38, 95%) and feet (n = 28/38, 74%). Pruritus was absent in all patients with probable AHEI and described for 6/21 with doubtful AHEI (29%). AHEI was the original diagnosis in only 24 patients (n = 24/40, 60%). The major differential diagnoses were purpura fulminans and urticaria multiforme.  Conclusion: AHEI, which the diagnosis is made on clinical findings, is often misdiagnosed. Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in a young child with a good overall condition are highly suggestive of AHEI. What is Known: •Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis affecting children under 3 years old. •Appropriate diagnosis is important to distinguish this benign disease from more serious diseases to avoid investigations and treatments, iatrogenic harm and unnecessary follow-up. What is New: •AHEI is an uncommon disorder often misdiagnosed by pediatricians and dermatologists. •Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in an infant with a good overall condition are highly suggestive of AHEI.

3.
Pediatr Dermatol ; 40(2): 349-351, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36305593

RESUMEN

Neonatal toxic shock syndrome-like exanthematous disease (NTED) was first described in Japan in the 1990s. It results from the secretion of superantigenic toxins by Staphylococcus aureus. Diagnostic criteria include generalized macular erythema and at least one of the following three features: fever (>38°C), thrombocytopenia (<150,000/mm3 ), low positive C reactive protein (10-50 mg/L) in the absence of another known disease process. We herein describe four cases from France, involving both MSSA and "Geraldine" MRSA. This report aims to bring this underdiagnosed disease to the attention of pediatricians and infectious disease specialists, to improve the management of affected newborns.


Asunto(s)
Toxinas Bacterianas , Exantema , Choque Séptico , Recién Nacido , Humanos , Enterotoxinas , Choque Séptico/diagnóstico , Superantígenos , Staphylococcus aureus , Exantema/diagnóstico
4.
Dermatol Ther ; 35(11): e15828, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-36107157

RESUMEN

Combined therapies involve the use of multiple drugs to increase efficacy and reduce the toxicity of individual treatments. We evaluated the use of combinations of conventional systemic therapies and biologics in children with psoriasis in daily practice. This two-part study used data from the 170 children in the Franco-Italian BiPe cohorts to evaluate the use, efficacy, and safety of combined conventional systemic-biologic therapies, and from a survey carried out among French and Italian dermatologists to better understand the reasons for using or avoiding these combinations. In total, 33 children (19.4%) from 13 dermatology centers received 48 combined conventional systemic-biologic therapies (cumulative duration: 43.6 years), including three triple combination therapies (acitretin-methotrexate, with a TNF-alpha inhibitor). A total of 14 different combinations were used, most frequently etanercept-acitretin (n = 10), adalimumab-acitretin (n = 7), adalimumab-methotrexate (n = 5), and ustekinumab-methotrexate (n = 5). The combined therapies were started at biologic initiation in 41 cases (85.4%), and after a period of biologic monotherapy in the remaining 7 cases. Mean PGA and PASI scores decreased between baseline and M3 with all the combinations used. Four serious adverse events were reported, all with favorable outcomes. The survey was completed by 61 dermatologists: 39 (63.9%) had previously used or planned to use the combined therapies, most commonly TNF-alpha inhibitors with acitretin or methotrexate. The main reason for using these treatments was to improve the outcome of biologic therapies in cases of partial efficacy or loss of efficacy. Combined therapies have been used frequently in the treatment of childhood psoriasis, in a range of clinical situations and in variable drug combinations, without significant toxicity. Although the use of these combined therapies needs to be clarified in future management guidelines, these combined therapies should be considered for the treatment of children with severe psoriasis, psoriatic arthritis, and recalcitrant disease.


Asunto(s)
Productos Biológicos , Fármacos Dermatológicos , Psoriasis , Niño , Humanos , Acitretina/efectos adversos , Acitretina/uso terapéutico , Adalimumab/efectos adversos , Adalimumab/uso terapéutico , Productos Biológicos/efectos adversos , Productos Biológicos/uso terapéutico , Fármacos Dermatológicos/efectos adversos , Fármacos Dermatológicos/uso terapéutico , Dermatólogos , Etanercept/efectos adversos , Etanercept/uso terapéutico , Metotrexato/efectos adversos , Metotrexato/uso terapéutico , Psoriasis/tratamiento farmacológico , Resultado del Tratamiento , Inhibidores del Factor de Necrosis Tumoral/efectos adversos , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico
5.
Eur J Pediatr ; 181(3): 1167-1174, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-34782946

RESUMEN

To evaluate the risk factors for crusted scabies in children in France. The retrospective multicenter study, conducted in France, of children (aged < 18 years) with profuse and/or crusted scabies confirmed by dermoscopy and/or microscopy. Data were obtained using a standardized questionnaire. We included 20 children. The mean age was 4.5 years, and 70% of the patients were girls. Their medical history revealed a neurological pathology (agenesis of the corpus callosum; n = 1, 5.0%), prematurity (n = 1, 5.0%), Down syndrome (n = 1, 5.0%), atopic dermatitis (n = 2, 10%), and asthma (n = 2, 10.0%). Fifteen (75.0%) children were treated with steroids before being diagnosed with scabies: 12 (60.0%) with topical steroids, one (5.0%) with a systemic steroid, and two (10.0%) with inhaled steroids. One child (5.0%) lived in a precarious environment. The mean duration of pruritus was 3.4 months, and that of the skin lesions was 3.1 months. The most commonly affected areas for crusted scabies were the palms/hands (66.7%) and the armpits (33.3%). Thirteen children (65.0%) were hospitalized, 14 (70.0%) were treated with ivermectin and all received topical treatments; 85.7% were cured within an average of 38 days, but one child had a relapse 3 months later in the form of common scabies.Conclusion: The main risk factor for developing crusted scabies in France was the misdiagnosis and the use of corticosteroids, especially topical forms typically used in "healthy" children. Management of the children was effective and similar to that used in adults. What is Known: • Crusted scabies is an extremely contagious disease which is rarely reported in infancy, especially in healthy children. • The main risk factors include immunosuppression, physical debilitation, and intellectual disability. What is New: • The main risk factor of severe scabies in this study was delayed diagnosis associated with the use of topical or systemic corticosteroids. • The treatment was successful in 85.7% of cases, and 65% of children needed to be hospitalized.


Asunto(s)
Escabiosis , Administración Tópica , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Terapia de Inmunosupresión , Ivermectina/uso terapéutico , Estudios Retrospectivos , Escabiosis/diagnóstico , Escabiosis/tratamiento farmacológico , Escabiosis/epidemiología
6.
Pediatr Blood Cancer ; 68(9): e29140, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-34109735

RESUMEN

BACKGROUND: The somatic BRAFV600E mutation occurs in 38-64% of pediatric cases of Langerhans cell histiocytosis (LCH). Vemurafenib (VMF), a BRAF inhibitor, was approved for refractory BRAFV600E mutated LCH. In adults, VMF causes frequent cutaneous adverse events (CAE) including skin tumors (squamous cell carcinomas, melanomas), but little is known in children. The objective of this study was to evaluate the frequency, clinical spectrum, and severity of CAEs in children treated with VMF for LCH. In addition, a correlation between CAE occurrence and VMF dose, residual plasma levels (RPLs), and efficacy was searched for. PROCEDURE: Multicentric retrospective observational study including patients <18 years treated with VMF alone for refractory BRAFV600E mutated LCH in 13 countries between October 1, 2013 and December 31, 2018. RESULTS: Fifty-seven patients: 56% female, median age 2.1 years (0.2-14.6), median treatment duration 4.1 months (1.4-29.7). Forty-one patients (72%) had at least one CAE: photosensitivity (40%), keratosis pilaris (32%), rash (26%), xerosis (21%), and neutrophilic panniculitis (16%). No skin tumor was observed. Five percent of CAEs were grade 3. None were grade 4 or led to permanent VMF discontinuation. Dose reduction was necessary for 12% of patients, temporary treatment discontinuation for 16%, none leading to loss of efficacy. VMF dose, median RPL, and efficacy were not correlated with CAE occurrence. CONCLUSIONS: At doses used for pediatric LCH, CAEs are frequent but rarely severe and have little impact on the continuation of treatment when managed appropriately. Regular dermatological follow-up is essential to manage CAEs and screen for possible induced skin tumors.


Asunto(s)
Histiocitosis de Células de Langerhans , Enfermedades de la Piel/inducido químicamente , Vemurafenib , Adolescente , Niño , Preescolar , Femenino , Histiocitosis de Células de Langerhans/tratamiento farmacológico , Humanos , Lactante , Masculino , Mutación , Inhibidores de Proteínas Quinasas , Proteínas Proto-Oncogénicas B-raf/antagonistas & inhibidores , Proteínas Proto-Oncogénicas B-raf/genética , Neoplasias Cutáneas/diagnóstico , Vemurafenib/efectos adversos
7.
Clin Exp Allergy ; 49(4): 526-536, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30672059

RESUMEN

BACKGROUND: Peach is a common elicitor of food allergic reactions. Peach-induced immediate reactions may occur as benign pollen-food syndromes, usually due to birch pollen-related PR-10 cross-reactivity in temperate climates, and as potentially severe primary food allergies, predominantly related to nsLTP Pru p 3 in Mediterranean regions. The newly described peach allergen Pru p 7 has gained recent attention as a potential peach allergy severity marker. Sensitization to Pru p 7 and its allergenic homologues of the gibberellin-regulated protein family occurs in areas with high Cupressaceae tree pollen exposure. OBJECTIVE: We sought to investigate the distribution, clinical characteristics and molecular associations of Pru p 7 sensitization among subjects with suspected peach allergy in different regions of France. METHODS: Subjects with suspected peach allergy (n = 316) were included. Diagnostic work-up was performed according to current guidelines, including open food challenge when required. IgE antibody measurements and competition experiments were performed using the ImmunoCAP assay platform. RESULTS: Sensitization to Pru p 7 was present in 171 (54%) of all subjects in the study and in 123 of 198 (62%) diagnosed as peach allergic, more than half of whom were sensitized to no other peach allergen. Frequency and magnitude of Pru p 7 sensitization were associated with the presence of peach allergy, the clinical severity of peach-induced allergic reactions and the level of cypress pollen exposure. Cypress pollen extract completely outcompeted IgE binding to Pru p 7. Pru p 7 was extremely potent in basophil activation tests. CONCLUSION AND CLINICAL RELEVANCE: A subtype of Cupressaceae pollinosis, characterized by Pru p 7 sensitization, can be an underlying cause of severe peach allergy.


Asunto(s)
Antígenos de Plantas/inmunología , Reacciones Cruzadas/inmunología , Cupressus/inmunología , Hipersensibilidad a los Alimentos/diagnóstico , Hipersensibilidad a los Alimentos/inmunología , Polen/inmunología , Prunus persica/efectos adversos , Adolescente , Adulto , Anciano , Alérgenos/inmunología , Basófilos/inmunología , Basófilos/metabolismo , Niño , Preescolar , Susceptibilidad a Enfermedades , Femenino , Hipersensibilidad a los Alimentos/epidemiología , Humanos , Inmunización , Inmunoglobulina E/inmunología , Lactante , Masculino , Persona de Mediana Edad , Prevalencia , Factores de Riesgo , Índice de Severidad de la Enfermedad , Adulto Joven
8.
Acta Derm Venereol ; 99(6): 539-543, 2019 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-30810215

RESUMEN

Methotrexate has demonstrated its efficiency for the treatment of juvenile localized scleroderma but some patients may be resistant. The aim of our study was to define the profile of such patients. We performed an observational retrospective multicenter study between 2007 and 2016 and included all children seen in the French Paediatric Dermatology and Rheumatology departments with active localized scleroderma treated by methotrexate for a minimum of 4 months. Metho-trexate efficacy was assessed clinically and/or by imaging between the fourth to twelfth months of treatment. A total of 57 patients were included. Metho-trexate dosage ranged from 7 to 15 mg/m2/week. Only 4 patients were resistant. No common features could be identified between these 4 patients. Children with localized scleroderma are rarely resistant to metho-trexate and we did not identify a clinical profile for those resistant patients.


Asunto(s)
Fármacos Dermatológicos/uso terapéutico , Resistencia a Medicamentos , Metotrexato/uso terapéutico , Esclerodermia Localizada/tratamiento farmacológico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos
9.
N Engl J Med ; 372(8): 735-46, 2015 Feb 19.
Artículo en Inglés | MEDLINE | ID: mdl-25693013

RESUMEN

BACKGROUND: Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS: We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS: Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS: This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).


Asunto(s)
Antagonistas Adrenérgicos beta/administración & dosificación , Hemangioma/tratamiento farmacológico , Propranolol/administración & dosificación , Administración Oral , Antagonistas Adrenérgicos beta/efectos adversos , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Esquema de Medicación , Femenino , Humanos , Hipotensión/inducido químicamente , Lactante , Masculino , Propranolol/efectos adversos , Resultado del Tratamiento
10.
J Hum Genet ; 63(3): 257-261, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29263414

RESUMEN

The focal facial dermal dysplasias (FFDDs) are a group of rare inherited developmental disorders characterized by congenital scar-like atrophic lesions in the bitemporal (FFDD1, 2, and 3) or preauricular (FFDD4) areas. FFDD4 is an autosomal-recessive trait characterized by preauricular skin defects without additional dysmorphic findings. Previously, only two CYP26C1 mutations in four unrelated patients with FFDD4 were reported. Here, we report two additional unrelated FFDD4 patients with four CYP26C1 mutations including three novel lesions: a missense mutation, c.230G>C (p.Arg77Pro), and two splice-site mutations, c.1191+1G>T (IVS5(+1)G>T) and c.1191+2insT (IVS5(+2)insT). In silico analyses predicted all three mutations as pathogenic. Compound heterozygosity was validated through parental studies. These results provide further evidence that CYP26C1 mutations are the molecular genetic basis of FFDD4. Identification of additional cases by dermatologists, pediatricians, and medical geneticists will lead to further understanding of the clinical spectrum of FFDD4 and define its molecular genetic heterogeneity.


Asunto(s)
Familia 26 del Citocromo P450/genética , Displasia Ectodérmica/diagnóstico , Displasia Ectodérmica/genética , Estudios de Asociación Genética , Mutación , Fenotipo , Alelos , Sustitución de Aminoácidos , Análisis Mutacional de ADN , Displasias Dérmicas Faciales Focales , Heterocigoto , Humanos , Lactante , Masculino
14.
J Am Acad Dermatol ; 76(3): 478-487, 2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-27742172

RESUMEN

BACKGROUND: Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports. OBJECTIVE: We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations. METHODS: A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed. RESULTS: Of the 78 patients included in the study, 56 underwent cranial and brain imaging. Twenty-three of the 56 patients (41%) had abnormal findings, including the following: (1) cranial/bone defect (30.4%), with direct communication with the central nervous system in 28.6%; (2) venous malformations (25%); or (3) central nervous system abnormalities (12.5%). Meningeal heterotopia in 34.6% (9/26) was the most common neuroectodermal association. Sinus pericranii, paraganglioma, and combined nevus were also identified. LIMITATIONS: The partial retrospective design and predominant recruitment from the dermatology department are limitations of this study. CONCLUSIONS: Infants with HCS or HTS are at high risk for underlying neurovascular anomalies. Magnetic resonance imaging scans should be performed in order to refer the infant to the appropriate specialist for management.


Asunto(s)
Anomalías Múltiples/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Coristoma/diagnóstico por imagen , Cabello/anomalías , Meninges , Cráneo/diagnóstico por imagen , Venas/diagnóstico por imagen , Encéfalo/anomalías , Reacciones Falso Negativas , Reacciones Falso Positivas , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Imagen Multimodal , Placa Neural , Neuroimagen , Estudios Prospectivos , Estudios Retrospectivos , Cuero Cabelludo/patología , Cráneo/anomalías , Tomografía Computarizada por Rayos X , Ultrasonografía Doppler en Color , Venas/anomalías
15.
Pediatr Dermatol ; 34(3): 282-289, 2017 May.
Artículo en Inglés | MEDLINE | ID: mdl-28271540

RESUMEN

BACKGROUND/OBJECTIVES: Emollients are part of the standard treatment for atopic dermatitis (AD), although there is limited evidence that regular use of emollients as management therapy reduces the frequency of flares and corticosteroid consumption. The objective of this study was to evaluate the benefit of emollient use in the management of mild to moderate AD in children by assessing the ability of two different emollients (particularly V0034CR) to prevent flares and to reduce the use of corticosteroids. METHODS: In this randomized, open-label study, patients with a current flare were treated with a potent topical corticosteroid. After flare resolution, patients were centrally randomized to V0034CR emollient, reference emollient, or no emollient (1:1:1 ratio) for 12 weeks. New flares were medically assessed before being treated with a moderately potent corticosteroid. RESULTS: A total of 335 children 2 to 6 years of age were randomized. At 12 weeks, the percentage of patients with one or more flares was statistically significantly lower with V0034CR (35.1%) than without emollient (67.6%; p < 0.001). Fewer patients treated with V0034CR required any corticosteroids or immunosuppressants (23.6%) than patients with no emollient (43.3%) at 12 weeks. The difference was significant at all time points (p = 0.002). Patients treated with emollients had a longer time to first flare, fewer flares, higher complete remission rates, less corticosteroid consumption, lower Investigator Global Assessment scores, and lower Scoring Atopic Dermatitis scores than those who were not. V0034CR was well tolerated, with no specific safety concerns. CONCLUSION: Regular emollient use in children with mild to moderate AD reduces flares and corticosteroid consumption.


Asunto(s)
Dermatitis Atópica/tratamiento farmacológico , Emolientes/administración & dosificación , Glicerol/administración & dosificación , Parafina/administración & dosificación , Niño , Preescolar , Emolientes/efectos adversos , Femenino , Glucocorticoides/administración & dosificación , Glicerol/efectos adversos , Humanos , Masculino , Parafina/efectos adversos , Brote de los Síntomas , Resultado del Tratamiento
16.
Pediatr Dermatol ; 34(1): 58-63, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-27873356

RESUMEN

BACKGROUND/OBJECTIVES: Little information is available on the prevalence and clinical aspects of nail involvement in children with psoriasis. The objective of this study was to evaluate the prevalence and clinical aspects of and the risk factors for nail involvement in French children with psoriasis. METHODS: We performed a multicenter, cross-sectional study in 23 French dermatology centers. All children seen during the 1-year study were systematically included. Clinical features of the nails were collected. Association with clinical aspects of the disease and comorbidities were evaluated. RESULTS: Of 313 children with psoriasis (mean age 9.1 ± 4.2 yrs; 149 boys, 164 girls), 31.1% had familial psoriasis and 30% had severe psoriasis. The mean age at onset was 6.1 ± 3.7 years. Nails were involved in 32.3% of children. The main clinical aspects were pitting (69.1%) for fingernails and onycholysis (40.0%) and pachyonychia (27.5%) for toenails. All of the fingers were involved at similar frequencies, whereas the big toe was involved twice as often as the others (p < 0.005). Nail involvement was associated with male sex (p < 0.001), palmoplantar psoriatic (p < 0.001), severity of disease (p = 0.003), and psoriatic arthritis (p = 0.03). CONCLUSION: The prevalence of nail involvement was 32.3% in children with psoriasis. Clinical aspects in children are reported, as well as clinical associations. As in adults, nail psoriasis is closely associated with psoriatic arthritis.


Asunto(s)
Enfermedades de la Uña/epidemiología , Uñas/patología , Psoriasis/epidemiología , Adolescente , Niño , Preescolar , Estudios Transversales , Femenino , Francia/epidemiología , Humanos , Masculino , Prevalencia , Factores de Riesgo
17.
JAMA ; 318(18): 1798-1809, 2017 11 14.
Artículo en Inglés | MEDLINE | ID: mdl-29136445

RESUMEN

Importance: Epicutaneous immunotherapy may have potential for treating peanut allergy but has been assessed only in preclinical and early human trials. Objective: To determine the optimal dose, adverse events (AEs), and efficacy of a peanut patch for peanut allergy treatment. Design, Setting, and Participants: Phase 2b double-blind, placebo-controlled, dose-ranging trial of a peanut patch in peanut-allergic patients (6-55 years) from 22 centers, with a 2-year, open-label extension (July 31, 2012-July 31, 2014; extension completed September 29, 2016). Patients (n = 221) had peanut sensitivity and positive double-blind, placebo-controlled food challenges to an eliciting dose of 300 mg or less of peanut protein. Interventions: Randomly assigned patients (1:1:1:1) received an epicutaneous peanut patch containing 50 µg (n = 53), 100 µg (n = 56), or 250 µg (n = 56) of peanut protein or a placebo patch (n = 56). Following daily patch application for 12 months, patients underwent a double-blind, placebo-controlled food challenge to establish changes in eliciting dose. Main Outcomes and Measures: The primary efficacy end point was percentage of treatment responders (eliciting dose: ≥10-times increase and/or reaching ≥1000 mg of peanut protein) in each group vs placebo patch after 12 months. Secondary end points included percentage of responders by age strata and treatment-emergent adverse events (TEAEs). Results: Of 221 patients randomized (median age, 11 years [quartile 1, quartile 3: 8, 16]; 37.6% female), 93.7% completed the trial. A significant absolute difference in response rates was observed at month 12 between the 250-µg (n = 28; 50.0%) and placebo (n = 14; 25.0%) patches (difference, 25.0%; 95% CI, 7.7%-42.3%; P = .01). No significant difference was seen between the placebo patch vs the 100-µg patch. Because of statistical testing hierarchical rules, the 50-µg patch was not compared with placebo. Interaction by age group was only significant for the 250-µg patch (P = .04). In the 6- to 11-year stratum, the response rate difference between the 250-µg (n = 15; 53.6%) and placebo (n = 6; 19.4%) patches was 34.2% (95% CI, 11.1%-57.3%; P = .008); adolescents/adults showed no difference between the 250-µg (n = 13; 46.4%) and placebo (n = 8; 32.0%) patches: 14.4% (95% CI, -11.6% to 40.4%; P = .40). No dose-related serious AEs were observed. The percentage of patients with 1 or more TEAEs (largely local skin reactions) was similar across all groups in year 1: 50-µg patch = 100%, 100-µg patch = 98.2%, 250-µg patch = 100%, and placebo patch = 92.9%. The overall median adherence was 97.6% after 1 year; the dropout rate for treatment-related AEs was 0.9%. Conclusions and Relevance: In this dose-ranging trial of peanut-allergic patients, the 250-µg peanut patch resulted in significant treatment response vs placebo patch following 12 months of therapy. These findings warrant a phase 3 trial. Trial Registration: clinicaltrials.gov Identifier: NCT01675882.


Asunto(s)
Alérgenos/administración & dosificación , Arachis/inmunología , Desensibilización Inmunológica/métodos , Hipersensibilidad al Cacahuete/terapia , Administración Cutánea , Adolescente , Adulto , Niño , Relación Dosis-Respuesta Inmunológica , Método Doble Ciego , Femenino , Humanos , Masculino , Persona de Mediana Edad
18.
J Pediatr ; 174: 211-217.e2, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-27157448

RESUMEN

OBJECTIVE: To describe the features of Frey syndrome (auriculotemporal nerve dysfunction with gustatory flushing) in childhood. STUDY DESIGN: A multicenter, retrospective, descriptive observational national case series study was conducted with the help of French academic societies. Diagnostic criteria were based on clinical history, and sometimes also on photographs or provocation tests. RESULTS: Forty-eight cases were identified, with 2 subtypes: 35 unilateral and 13 bilateral. Associated sweating was reported in only 10% of cases. Diagnosis was made in only 20% of children at the first consultation and inappropriate dietary restriction was prescribed for 21%. Instrumented vaginal delivery was significantly associated with unilateral forms (OR [unilateral vs bilateral] = 29; 95% CI 3.99-311.58; P < .001). The outcome was favorable overall with 57% regression, 20% recovery, and only 23% persistence of initial symptoms. Regression was more frequent in unilateral forms (OR = 6.60; 95% CI 1.23-44.04; P = .016), observed in 69% of unilateral forms at a median age of 27 (24-48) months. Recovery predominated in bilateral forms (OR = 0.05; 95% CI 0-0.38; P = .001), observed in 58% of bilateral cases at a median age of 8 (7-9) months. CONCLUSIONS: Frey syndrome in childhood is a rare but benign condition with mild symptoms and a favorable outcome in most cases. Unilateral forms are mostly associated with instrumented delivery. Pediatricians should be familiar with this disorder in order to avoid misdiagnosis, mainly as food allergy, and unnecessary referrals and tests.


Asunto(s)
Sudoración Gustativa/diagnóstico , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Hipersensibilidad a los Alimentos/diagnóstico , Humanos , Lactante , Recién Nacido , Masculino , Evaluación de Resultado en la Atención de Salud , Derivación y Consulta , Estudios Retrospectivos , Sudoración Gustativa/complicaciones , Sudoración Gustativa/terapia
19.
Acta Derm Venereol ; 96(6): 802-6, 2016 Aug 23.
Artículo en Inglés | MEDLINE | ID: mdl-26925822

RESUMEN

Data regarding systemic therapies in the management of atopic dermatitis are limited. The aim of this study was to provide evidence for the efficacy and tolerance of systemic immunosuppressive treatments for moderate-to-severe adult atopic dermatitis. A single-centre retrospective study was conducted. A total of 54 patients were prescribed systemic treatments between 2000 and 2014. Of these, 28 received methotrexate and 55.6% were considered as responders based on Physician's Global Assessment, 17 received azathioprine (37.5% responders), 43 received cyclosporin A (65.9% responders) and 7 received a combination therapy with methotrexate and azathioprine (57.1% responders). These treatments were well-tolerated overall and few adverse events required discontinuation of treatment. Combination therapy associating methotrexate and azathioprine appears to be a promising treatment for patients who fail to respond to conventional monotherapies.


Asunto(s)
Dermatitis Atópica/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Adolescente , Adulto , Anciano , Azatioprina/uso terapéutico , Ciclosporina/uso terapéutico , Quimioterapia Combinada , Femenino , Humanos , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento
20.
Pediatr Blood Cancer ; 62(5): 798-806, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25683226

RESUMEN

BACKGROUND: The dermatologic adverse events (AEs) of various molecularly targeted therapies are well-described in adult cancer patients. Little has been reported on the incidence and clinical presentation of such AEs in pediatric patients with cancer. To address this gap, we analyzed the dermatologic AEs reported across clinical trials of targeted anticancer therapies in pediatric patients. PROCEDURES: We conducted an electronic literature search (PubMed, American Society of Clinical Oncology annual meetings' abstracts, ClinicalTrials.gov, NCI's Pediatric Oncology Branch webpage) to identify clinical trials involving targeted anticancer therapies that reported dermatologic AEs in their safety data. Studies were limited to the pediatric population, monotherapy trials (oncology), and English language publications. RESULTS: Pooled data from 19 clinical studies investigating 11 targeted anticancer agents (alemtuzumab, rituximab, imatinib, dasatinib, erlotinib, vandetanib, sorafenib, cabozantinib, pazopanib, everolimus, and temsirolimus) were analyzed. The most frequently encountered dermatologic AEs were rash (127/660; 19%), xerosis (18/100; 18%), mucositis (68/402; 17%), and pruritus (12/169; 7%). Other AEs included pigmentary abnormalities of the skin/hair (13%), hair disorders (trichomegaly, hypertrichosis, alopecia, and madarosis; 14%), urticaria (7%), palmoplantar erythrodysesthesia (7%), erythema, acne, purpura, skin fissures, other 'unknown skin changes', exanthem, infection, flushing, telangiectasia, and photosensitivity. CONCLUSION: This study describes the dermatologic manifestations of targeted anticancer therapy-related AEs in the pediatric population. Since these AEs are often associated with significant morbidity, it is imperative that pediatric oncologists be familiar with their recognition and management, to avoid unnecessary dose modifications and/or termination, and to prevent impairments in patients' quality of life.


Asunto(s)
Antineoplásicos/efectos adversos , Terapia Molecular Dirigida/efectos adversos , Neoplasias/tratamiento farmacológico , Enfermedades de la Piel/inducido químicamente , Adulto , Niño , Ensayos Clínicos como Asunto , Humanos , Metaanálisis como Asunto , Neoplasias/patología , Pronóstico , Enfermedades de la Piel/patología
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