RESUMO
BACKGROUND: Toe-web (TW) intertrigo is a common disease of fungal or bacterial origin. Gram-negative bacterial (GNB) TW intertrigo consists of weeping, erosive, painful lesions that may be recurrent, leading to functional disability. Eczema is often associated with this condition. The management of intertrigo is poorly codified. OBJECTIVE: To evaluate the efficacy and safety of a standardized treatment plan using topical steroids in relation to the course and the frequency of recurrence of GNB-TW intertrigo. METHODS: We conducted a prospective open interventional multicentre study from June 2020 to June 2021. Standardised treatment using TCS together with follow-up via phone calls were performed over a 6-month period. In addition, a retrospective historical monocentric study was performed for patients with suspected TW-GNB intertrigo treated without standardized management. The primary endpoint was disease duration. We performed a Wilcoxon test to compare the median duration of GNB-TW intertrigo in both series. RESULTS: We included 13 patients in the prospective cohort and 14 in the retrospective cohort. In both cohorts, most patients were male with a median age of 59â¯years. The most frequent signs were fissures and exudates. Eczema was often associated (51.8%). Identified risk factors were psoriasis, local humidity, fungal intertrigo, vascular disease (arterial or venous insufficiency), and a history of multiple local treatments prior to diagnosis. Pseudomonas aeruginosa was the predominant pathogen (48.1%). Median durations of TW-GNB intertrigo were 56â¯days and 61â¯days. There was no significant difference in the median duration of the disease between the prospective and the retrospective cohorts (respectively61â¯days and 56â¯days; pâ¯>â¯0.58). Relapses were more frequent in the retrospective cohort (respectively 7.7% and 21.4%). CONCLUSION: GNB-TW intertrigo is a difficult-to-treat disease often associated with eczema. While topical corticosteroids (TCS) seem to be an effective and well-tolerated treatment they do not appear to reduce disease duration compared to other treatments.
RESUMO
BACKGROUND: There is limited data about the clinical meaningfulness of the Scoring of Atopic Dermatitis (SCORAD) and Patient-Oriented SCORAD (PO-SCORAD), particularly in children with mild-to-moderate AD. Regular use of patient-reported outcomes, may deliver more accurate information about the overall health status of AD patients than routine but sparse physician assessments. OBJECTIVE: To confirm the correlation between SCORAD, PO-SCORAD, Patient-Oriented Eczema Measure (POEM) and Investigator's Global Assessment (IGA). To evaluate the interpretability and clinical usefulness of the SCORAD and PO-SCORAD scores in children. METHODS: Data were drawn from a 12-week randomized controlled trial in 335 children, aged 2-6 years, with mainly mild-to-moderate AD. Investigators captured SCORAD and IGA at each study visit. Parents used PO-SCORAD twice-weekly, and POEM once-weekly. RESULTS: There were strong correlations between PO-SCORAD and SCORAD (r = 0.874), PO-SCORAD and POEM (0.734) and PO-SCORAD and IGA (0.613). The best fit ('k' statistic: 0.68) between SCORAD and IGA classes was noted for the following SCORAD categories: <12 (clear/almost clear); 12-25 (mild); and ≥25 (moderate/severe). PO-SCORAD area under the curve over 8 weeks was significantly greater than that of SCORAD (p = 0.0002), giving a better estimate of disease severity between visits. Patients with a flare within the next 7 days had significantly higher PO-SCORAD scores 7 days before the flare (p < 0.0001). Moderate erythema was the most significant flare predictor (p < 0.0001). CONCLUSION: PO-SCORAD is robust and reliable and appears to warrant far greater utility in routine clinical practice than other scores. PO-SCORAD, used twice-weekly, may improve the management of patients with AD.
Assuntos
Dermatite Atópica , Criança , Humanos , Pré-Escolar , Dermatite Atópica/diagnóstico , Índice de Gravidade de Doença , Gravidade do Paciente , Medidas de Resultados Relatados pelo Paciente , Imunoglobulina A , Qualidade de VidaRESUMO
BACKGROUND: Phototherapy is used to treat atopic dermatitis (AD). Evidence for its efficacy, impact on quality of life, cost-effectiveness and short- and long-term safety with real-life usage is weak. OBJECTIVES: We established a taskforce to examine how phototherapy is currently being used as a treatment for AD across the United Kingdom and Europe to inform our understanding and guide future research into management of patients with AD using UV-based phototherapies. METHODS: An anonymous electronic multiple-response survey exploring phototherapy prescribing practices and experience of phototherapy modalities was developed by the study authors and sent to members of phototherapy networks from the United Kingdom and Europe. Responses were received between February and July 2021. RESULTS: About 144 respondents from 27 European countries completed the survey. NBUVB was the most widely used [n = 138 (96%)]. Home-based NBUVB was available in 8/27 countries (25/144 respondents, 17%). Oral psoralen-UVA (PUVA) was more widely available than bath PUVA (n = 106, 74% vs. n = 60, 42%) and used mainly in adult patients. 49/144 (34%) of respondents had access to UVA1. Phototherapy would be considered instead of systemic treatment in 96% of adults and 82% of children for NBUVB, versus 40% of adults and 3% of children for PUVA. Starting doses, standard dosing increments, length of treatment courses, lifetime limits for treatments and thresholds for performing annual skin assessments varied between responders. CONCLUSIONS: NBUVB was the most widely used phototherapy for AD in adult and paediatric patients, while PUVA and UVA1 were less used. Prescribing practices varied considerably, highlighting the lack of consensus practice in many different aspects of phototherapy for the treatment of AD in children and adults. This indicates that further studies are required to determine optimal phototherapeutic regimens for AD and informs our understanding of parameters that should be included in future high-quality randomized controlled trials (RCT) of phototherapy.
Assuntos
Dermatite Atópica , Terapia Ultravioleta , Adulto , Humanos , Criança , Dermatite Atópica/terapia , Fototerapia , Europa (Continente) , Reino UnidoRESUMO
BACKGROUND: Atopic dermatitis (AD) is a heterogeneous inflammatory skin disease with different clinical phenotypes based on factors such as age, race, comorbidities, and clinical signs and symptoms. The effect of these factors on therapeutic responses in AD has only been scarcely studied and not for upadacitinib. Currently, there is no biomarker predicting response to upadacitinib. OBJECTIVES: Evaluate the efficacy of the oral Janus kinase inhibitor upadacitinib across patient subgroups (baseline demographics, disease characteristics and prior treatment) in patients with moderate-to-severe AD. METHODS: Data from phase 3 studies (Measure Up 1, Measure Up 2 and AD Up) were utilized for this post hoc analysis. Adults and adolescents with moderate-to-severe AD were randomized to receive once daily oral upadacitinib 15 mg, upadacitinib 30 mg or placebo; patients enrolled in the AD Up study received concomitant topical corticosteroids. Data from the Measure Up 1 and Measure Up 2 studies were integrated. RESULTS: A total of 2584 patients were randomized. A consistently greater proportion of patients achieved at least 75% improvement in the Eczema Area and Severity Index, a 0 or 1 on the validated Investigator Global Assessment for Atopic Dermatitis, and improvement in itch (including an achievement of a reduction of ≥4; and score of 0/1 in Worst Pruritus Numerical Rating Scale) with upadacitinib compared with placebo at Week 16, regardless of age, sex, race, body mass index, AD severity, body surface area involvement, history of atopic comorbidities or asthma, or previous exposure to systemic therapy or cyclosporin. CONCLUSIONS: Upadacitinib had consistently high skin clearance rates and itch efficacy across subgroups of patients with moderate-to-severe AD through Week 16. These results support upadacitinib as a suitable treatment option in a variety of patients. TRIAL REGISTRATION: ClinicalTrials.gov Identifiers: NCT03569293 (Measure Up 1), NCT03607422 (Measure Up 2) and NCT03568318 (AD Up).
Assuntos
Dermatite Atópica , Humanos , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/diagnóstico , Resultado do Tratamento , Compostos Heterocíclicos com 3 Anéis/uso terapêutico , Prurido/tratamento farmacológico , Índice de Gravidade de Doença , Método Duplo-CegoRESUMO
BACKGROUND: The factors associated with early relapse of infantile haemangioma (IH) after a first course of treatment with oral propranolol for at least six months (initiated after the marketing authorization had been granted) have not previously been investigated. OBJECTIVES: To identify factors associated with the risk of early relapse in children with IH treated with oral propranolol according to the current prescribing guidelines. METHODS: We performed a multicentre, retrospective, case-control study, using the Ouest Data Hub database. All children treated for at least 6â¯months with oral propranolol for IH between 31 June 2014 and 31 December 2021, and with a follow-up visit at least three months after treatment discontinuation were included. A case was defined as relapse of IH within three months of treatment discontinuation; each case was matched for age at treatment initiation and for centre, with four (relapse-free) controls. The association between relapse and treatment or IH characteristics was expressed as an odds ratio (OR) from univariate and multivariate conditional logistic regressions. RESULTS: A total of 225 children were included. Of these, 36 (16%) relapsed early. In a multivariate analysis, a deep IH component was a risk factor for early relapse [ORâ¯=â¯8.93; 95%CI: 1.0-78.9, pâ¯=â¯0.05]. A propranolol dosage level of less than 3â¯mg/kg/day protected against early relapse [ORâ¯=â¯0.11; 95%CI: 0.02-0.7, pâ¯=â¯0.02]. Tapering before propranolol discontinuation was not associated with a lower risk of early relapse. CONCLUSION: The risk factors for late and early relapse are probably different. Investigation of the risk factors for early vs. late IH relapse is now warranted.
Assuntos
Hemangioma Capilar , Neoplasias Cutâneas , Criança , Humanos , Lactente , Estudos de Casos e Controles , Estudos Retrospectivos , Propranolol/uso terapêutico , Doença Crônica , Resultado do Tratamento , Administração Oral , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This second part of the guideline includes recommendations and detailed information on basic therapy with emollients and moisturizers, topical anti-inflammatory treatment, antimicrobial and antipruritic treatment and UV phototherapy. Furthermore, this part of the guideline covers techniques for avoiding provocation factors, as well as dietary interventions, immunotherapy, complementary medicine and educational interventions for patients with atopic eczema and deals with occupational and psychodermatological aspects of the disease. It also contains guidance on treatment for paediatric and adolescent patients and pregnant or breastfeeding women, as well as considerations for patients who want to have a child. A chapter on the patient perspective is also provided. The first part of the guideline, published separately, contains recommendations and guidance on systemic treatment with conventional immunosuppressive drugs, biologics and janus kinase (JAK) inhibitors, as well as information on the scope and purpose of the guideline, and a section on guideline methodology.
Assuntos
Anti-Infecciosos , Produtos Biológicos , Dermatite Atópica , Fármacos Dermatológicos , Eczema , Adolescente , Anti-Infecciosos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Antipruriginosos/uso terapêutico , Produtos Biológicos/uso terapêutico , Criança , Dermatite Atópica/tratamento farmacológico , Fármacos Dermatológicos/uso terapêutico , Eczema/tratamento farmacológico , Emolientes/uso terapêutico , Feminino , Humanos , Janus QuinasesRESUMO
The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This first part of the guideline includes general information on its scope and purpose, the health questions covered, target users and a methods section. It also provides guidance on which patients should be treated with systemic therapies, as well as recommendations and detailed information on each systemic drug. The systemic treatment options discussed in the guideline comprise conventional immunosuppressive drugs (azathioprine, ciclosporin, glucocorticosteroids, methotrexate and mycophenolate mofetil), biologics (dupilumab, lebrikizumab, nemolizumab, omalizumab and tralokinumab) and janus kinase inhibitors (abrocitinib, baricitinib and upadacitinib). Part two of the guideline will address avoidance of provocation factors, dietary interventions, immunotherapy, complementary medicine, educational interventions, occupational and psychodermatological aspects, patient perspective and considerations for paediatric, adolescent, pregnant and breastfeeding patients.
Assuntos
Dermatite Atópica , Eczema , Adolescente , Azatioprina/uso terapêutico , Criança , Ciclosporina/uso terapêutico , Dermatite Atópica/tratamento farmacológico , Eczema/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Ácido Micofenólico/uso terapêuticoRESUMO
BACKGROUND: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD). OBJECTIVE: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice. METHODS: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial. RESULTS: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial. LIMITATIONS: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information. CONCLUSION: These real-life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group.
Assuntos
Conjuntivite , Dermatite Atópica , Criança , Humanos , Dermatite Atópica/tratamento farmacológico , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Conjuntivite/induzido quimicamente , Estudos de Coortes , Imunoglobulina ARESUMO
INTRODUCTION: Lamellar ichthyosis is a rare congenital disorder that can be encountered by plastic surgeon in a daily practice. Its clinical diagnosis makes it an significant pathology to identify and to know how to treat. MATERIAL AND METHODS: We report the case of a patient suffering from lamellar ichthyosis complicated by erosive pseudo pustulosis of the scalp. Our treatment protocol with two intra-lesional delayed-corticoids (Kenacort ®) injections three months apart showed significant clinical improvement of the lesions. DISCUSSION: Congenital lamellar ichthyosis regroups various clinical presentations. Most of the therapeutic strategies described in the literature involve local and systemic treatments, weighing on patients and leading to modest results. Surgical treatment or hyaluronic injections have also been reported but they raise problematics regarding morbidity and efficiency. CONCLUSION: Our therapeutic strategy by two Kenacort ® injections three months apart is simple, reproductible and has shown efficiency in the treatment of our patient suffering from congenital lamellar ichthyosis complicated with erosive pseudo pustulosis of the scalp.
Assuntos
Ictiose Lamelar , Humanos , Ictiose Lamelar/complicações , Ictiose Lamelar/diagnóstico , Ictiose Lamelar/patologia , Couro Cabeludo/patologiaAssuntos
Contratura , Doenças Musculares , Fibrose Pulmonar , Anormalidades da Pele , Biópsia , Proteínas de Ciclo Celular/genética , Contratura/genética , Fibrose , Humanos , Doenças Musculares/diagnóstico , Doenças Musculares/genética , Mutação , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/genética , Anormalidades da Pele/diagnóstico , Anormalidades da Pele/genética , Anormalidades da Pele/patologia , Tendões/patologiaRESUMO
Dupilumab is a recombinant monoclonal IgG4 type antibody which inhibits IL4 and IL13 signaling. It is indicated in moderate to severe atopic dermatitis (AD) in adults and adolescents over 12 years of age. Its side effects include conjunctivitis and blepharoconjunctivitis, affecting between 4.7% and 28% of patients depending on the study. The incidence of conjunctivitis in patients treated with dupilumab for AD appears to be higher than placebo in clinical studies. This increase was not observed in patients treated with dupilumab for asthma or sinonasal polyposis. The risk factors for conjunctivitis in patients with AD are disease severity, pre-existence of conjunctivitis and low concentrations of dupilumab, but the pathophysiology of this disease is poorly understood. A literature search carried out in April and May 2020 showed an increase in the number of publications on the subject, but there are currently no official joint dermatologist-ophthalmologist recommendations for prevention and management. The objective of this article is to provide an overview of the status of this subject, to address the main questions raised by this type of conjunctivitis and to suggest a course of action for starting and continuing treatment with dupilumab in patients with AD, according to the recommendations of the French Ophthalmologist/Dermatologist group CEDRE.
Assuntos
Conjuntivite , Dermatite Atópica , Eczema , Adolescente , Adulto , Anticorpos Monoclonais Humanizados , Conjuntivite/induzido quimicamente , Conjuntivite/tratamento farmacológico , Conjuntivite/epidemiologia , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/epidemiologia , Eczema/tratamento farmacológico , Humanos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
BACKGROUND: Neurofibromatosis 1 (NF1) is one of the most common inherited disorders characterized by mutations in the tumour suppressor gene NF1. Its clinical manifestations are highly variable and unpredictable. A specific NF1 mutation does not predict the severity or complications of the disease. OBJECTIVE: The objective of this study was to build an empirical classification scheme without any a priori hypotheses to identify the underlying NF1 subtypes that best explain the observed heterogeneity. METHODS: We performed latent class analysis (LCA) of 1351 consecutive NF1 patients aged >17 years seen between 2002 and 2014. Data and phenotypic features were collected prospectively on a standardized form. RESULTS: The median age was 36.8 (17-81) years. A three-class model showed the best fit: 706 (52%) belonged to the LC1 'Cutaneous neurofibromas' class having preferentially cutaneous neurofibromas (99%), plexiform neurofibromas (63%) and blue-red macules (29%); 593 (44%) belonged to the LC2 'Subcutaneous neurofibromas' class characterized by the presence of at least 10 subcutaneous neurofibromas (21%) and a familial form (77%) and 52 (4%) belonged to the LC3 'Dysmorphic phenotype' class characterized by dysmorphic features (78%) and learning difficulties (87%). Patients in LC1 had a higher likelihood of developing scoliosis (RR = 1.7, 95% confidence interval (CI) [1.2-2.4]). Patients in LC2 were more likely to be men (RR = 1.4, 95% CI [1.1-1.7]). Patients in LC3 were at higher risk of having an optic pathway glioma (RR = 4.8, 95% CI [1.9-11.8]) and epilepsy (RR = 4.5, 95% CI [1.8-11.6]). CONCLUSION: Our findings invite the performance of a larger cohort study to test whether the various latent classes reflect different underlying genetic modifiers of these phenotypic traits.