Dermatitis herpetiforme como carta de presentación de la enfermedad celíaca

RESUMEN: La Dermatitis Herpetiforme (DH) es una enfermedad ampollar autoinmune (EAI) que corresponde a la manifestación cutánea de la Enfermedad Celíaca (EC), más precisamente de la intolerancia al gluten. Clínicamente se manifiesta como una erupción papulo-vesicular pruriginosa, topografiada fundamentalmente en superficies de extensión de extremidades. El diagnóstico se realiza mediante el estudio histopatológico de piel lesional e inmunofluorescencia directa (IFD) de piel perilesional, la cual muestra hallazgos característicos. En su patogenia intervienen factores genéticos, inmunológicos, y ambientales. El tratamiento de elección es la dieta libre de gluten (DLG) y la Dapsona. Se ha reportado una asociación cercana al 15% entre la afectación cutánea e intestinal, no existe hasta el momento ningún estudio prospectivo que muestre la frecuencia real de EC en pacientes con DH

Parents’ self-efficacy, outcome expectations, and self-reported task performance when managing atopic dermatitis in children: Instrument reliability and validity

BACKGROUND: Support and education for parents faced with managing a child with atopic dermatitis is crucial to the success of current treatments. Interventions aiming to improve parent management of this condition are promising. Unfortunately, evaluation is hampered by lack of precise research tools to measure change. OBJECTIVES: To develop a suite of valid and reliable research instruments to appraise parents' self-efficacy for performing atopic dermatitis management tasks; outcome expectations of performing management tasks; and self-reported task performance in a community sample of parents of children with atopic dermatitis. METHODS: The Parents' Eczema Management Scale (PEMS) and the Parents' Outcome Expectations of Eczema Management Scale (POEEMS) were developed from an existing self-efficacy scale, the Parental Self-Efficacy with Eczema Care Index (PASECI). Each scale was presented in a single self-administered questionnaire, to measure self-efficacy, outcome expectations, and self-reported task performance related to managing child atopic dermatitis. Each was tested with a community sample of parents of children with atopic dermatitis, and psychometric evaluation of the scales' reliability and validity was conducted. SETTING AND PARTICIPANTS: A community-based convenience sample of 120 parents of children with atopic dermatitis completed the self-administered questionnaire. Participants were recruited through schools across Australia. RESULTS: Satisfactory internal consistency and test-retest reliability was demonstrated for all three scales. Construct validity was satisfactory, with positive relationships between self-efficacy for managing atopic dermatitis and general perceived self-efficacy; self-efficacy for managing atopic dermatitis and self-reported task performance; and self-efficacy for managing atopic dermatitis and outcome expectations. Factor analyses revealed two-factor structures for PEMS and PASECI alike, with both scales containing factors related to performing routine management tasks, and managing the child's symptoms and behaviour. Factor analysis was also applied to POEEMS resulting in a three-factor structure. Factors relating to independent management of atopic dermatitis by the parent, involving healthcare professionals in management, and involving the child in the management of atopic dermatitis were found. Parents' self-efficacy and outcome expectations had a significant influence on self-reported task performance. CONCLUSIONS: Findings suggest that PEMS and POEEMS are valid and reliable instruments worthy of further psychometric evaluation. Likewise, validity and reliability of PASECI was confirmed.

The relationship between parents’ self-efficacy beliefs, outcome expectations, child behaviour, and management of atopic dermatitis

Atopic dermatitis (AD) is a chronic inflammatory skin condition, characterized by intense pruritis, with a complex aetiology comprising multiple genetic and environmental factors. It is a common chronic health problem among children, and along with other allergic conditions, is increasing in prevalence within Australia and in many countries worldwide. Successful management of childhood AD poses a significant and ongoing challenge to parents of affected children. Episodic and unpredictable, AD can have profound effects on children’s physical and psychosocial wellbeing and quality of life, and that of their caregivers and families. Where concurrent child behavioural problems and parenting difficulties exist, parents may have particular difficulty achieving adequate and consistent performance of the routine management tasks that promote the child’s health and wellbeing. Despite frequent reports of behaviour problems in children with AD, past research has neglected the importance of child behaviour to parenting confidence and competence with treatment. Parents of children with AD are also at risk of experiencing depression, anxiety, parenting stress, and parenting difficulties. Although these factors have been associated with difficulty in managing other childhood chronic health conditions, the nature of these relationships in the context of child AD management has not been reported. This study therefore examined relationships between child, parent, and family variables, and parents’ management of child AD and difficult child behaviour, using social cognitive and self-efficacy theory as a guiding framework. The study was conducted in three phases. It employed a quantitative, cross-sectional study design, accessing a community sample of 120 parents of children with AD, and a sample of 64 child-parent dyads recruited from a metropolitan paediatric tertiary referral centre. In Phase One, instruments designed to measure parents’ self-reported performance of AD management tasks (Parents’ Eczema Management Scale – PEMS) and parents’ outcome expectations of task performance (Parents’ Outcome Expectations of Eczema Management Scale – POEEMS) were adapted from the Parental Self-Efficacy with Eczema Care Index (PASECI). In Phase Two, these instruments were used to examine relationships between child, parent, and family variables, and parents’ self-efficacy, outcome expectations, and self-reported performance of AD management tasks. Relationships between child, parent, and family variables, parents’ self-efficacy for managing problem behaviours, and reported parenting practices, were also examined. This latter focus was explored further in Phase Three, in which relationships between observed child and parent behaviour, and parent-reported self-efficacy for managing both child AD and problem behaviours, were explored. Phase One demonstrated the reliability of both PEMS and POEEMS, and confirmed that PASECI was reliable and valid with modification as detailed. Factor analyses revealed two-factor structures for PEMS and PASECI alike, with both scales containing factors related to performing routine management tasks, and managing the child’s symptoms and behaviour. Factor analysis was also applied to POEEMS resulting in a three-factor structure. Factors relating to independent management of AD by the parent, involving healthcare professionals in management, and involving the child in management of AD were found. Parents’ self-efficacy and outcome expectations had a significant influence on self-reported task performance. In Phase Two, relationships emerged between parents’ self-efficacy and self-reported performance of AD management tasks, and AD severity, child behaviour difficulties, parent depression and stress, conflict over parenting issues, and parents’ relationship satisfaction. Using multiple linear regressions, significant proportions of variation in parents’ self-efficacy and self-reported performance of AD management tasks were explained by child behaviour difficulties and parents’ formal education, and self-efficacy emerged as a likely mediator for the relationships between both child behaviour and parents’ education, and performance of AD management tasks. Relationships were also found between parents’ self-efficacy for managing difficult child behaviour and use of dysfunctional parenting strategies, and child behaviour difficulties, parents’ depression and stress, conflict over parenting issues, and relationship satisfaction. While significant proportions of variation in self-efficacy for managing child behaviour were explained by both child behaviour and family income, family income was the only variable to explain a significant proportion of variation in parent-reported use of dysfunctional parenting strategies. Greater use of dysfunctional parenting strategies (both lax and authoritarian parenting) was associated with more severe AD. Parents reporting lower self-efficacy for managing AD also reported lower self-efficacy for managing difficult child behaviour; likewise, less successful self-reported performance of AD management tasks was associated with greater use of dysfunctional parenting strategies. When child and parent behaviour was directly observed in Phase Three, more aversive child behaviour was associated with lower self-efficacy, less positive outcome expectations, and poorer self-reported performance of AD management tasks by parents. Importantly, there were strong positive relationships between these variables (self-efficacy, outcome expectations, and self-reported task performance) and parents’ observed competence when providing treatment to their child. Less competent performance was also associated with greater parent-reported child behaviour difficulties, parent depression and stress, parenting conflict, and relationship dissatisfaction. Overall, this study revealed the importance of child behaviour to parents’ confidence and practices in the contexts of child AD and child behaviour management. Parents of children with concurrent AD and behavioural problems are at particular risk of having low self-efficacy for managing their child’s AD and difficult behaviour. Children with more severe AD are also at higher risk of behaviour problems, and thus represent a high-risk group of children whose parents may struggle to manage the disease successfully. As one of the first studies to examine the role and correlates of parents’ self-efficacy in child AD management, this study identified a number of potentially modifiable factors that can be targeted to enhance parents’ self-efficacy, and improve parent management of child AD. In particular, interventions should focus on child behaviour and parenting issues to support parents caring for children with AD and improve child health outcomes. In future, findings from this research will assist healthcare teams to identify parents most in need of support and intervention, and inform the development and testing of targeted multidisciplinary strategies to support parents caring for children with AD.

Re-examining the evidence in radiation dermatitis management literature : an overview and a critical appraisal of systematic reviews

Purpose: To provide an overview and a critical appraisal of systematic reviews (SRs) of published interventions for the prevention/management of radiation dermatitis. Methods and Materials: We searched Medline, CINAHL, Embase, and the Cochrane Library. We also manually searched through individual reference lists of potentially eligible articles and a number of key journals in the topic area. Two authors screened all potential articles and included eligible SRs. Two authors critically appraised and extracted key findings from the included reviews using AMSTAR (the measurement tool for “assessment of multiple systematic reviews”). Results: Of 1837 potential titles, 6 SRs were included. A number of interventions have been reported to be potentially beneficial for managing radiation dermatitis. Interventions evaluated in these reviews included skin care advice, steroidal/nonsteroidal topical agents, systemic therapies, modes of radiation delivery, and dressings. However, all the included SRs reported that there is insufficient evidence supporting any single effective intervention. The methodological quality of the included studies varied, and methodological shortfalls in these reviews might create biases to the overall results or recommendations for clinical practice. Conclusions: An up-to-date high-quality SR in the prevention/management of radiation dermatitis is needed to guide practice and direction for future research. We recommend that clinicians or guideline developers critically evaluate the information of SRs in their decision making.

An overview of level I evidence for the management of radiation dermatitis literature

Background: Despite the technologic advances, radiation dermatitis is still a prevalent and distressing symptom in patients with cancer undergoing radiotherapy. Systematic reviews (SRs) are regarded as level I evidence providing direction for clinical practice and guidelines. This overview aims to provide a critical appraisal of SRs published on interventions for the prevention/management of radiation dermatitis. Methodology: We searched the following electronic databases: MEDLINE, CINAHL, EMBASE, and the Cochrane Library (up to Feb 2012). We also hand-searched reference lists of potentially eligible articles and a number of key journals in the area. Two authors screened all potential articles and included eligible SRs. Two authors critically appraised and extracted key findings from the included reviews using the “A Measurement Tool to Assess Systematic Reviews” (AMSTAR). Results: Of 1837 potential titles, six SRs were included. A number of interventions have been reported to be potentially beneficial for managing radiation dermatitis. Interventions evaluated in these reviews included skin care advice, steroidal/non-steroidal topical agents, systematic therapies, modes of radiation delivery, and dressings. However, all the included SRs reported that there is insufficient evidence supporting any single effective intervention. The methodological quality of the included studies varied, and methodological shortfalls in these reviews may create biases to the overall results or recommendations for clinical practice. Conclusions and implications: An up-to-date high quality SR in preventing/managing radiation dermatitis is needed to guide practice and direction for future research. Clinicians or guideline developers are recommended to critically evaluate the information of SRs in their decision making.

Incontinence-associated dermatitis: A cross-sectional prevalence study in the Australian acute care hospital setting

The purpose of this cross-sectional study was to identify the prevalence of incontinence and incontinence-associated dermatitis (IAD) in Australian acute care patients and to describe the products worn to manage incontinence, and those provided at the bedside for perineal skin care. Data on 376 inpatients were collected over 2 days at a major Australian teaching hospital. The mean age of the sample group was 62 years and 52% of the patients were male. The prevalence rate of incontinence was 24% (91/376). Urinary incontinence was significantly more prevalent in females (10%) than males (6%) (χ2  = 4·458, df = 1, P = 0·035). IAD occurred in 10% (38/376) of the sample group, with 42% (38/91) of incontinent patients having IAD. Semi-formed and liquid stool were associated with IAD (χ2  = 5·520, df = 1, P = 0·027). Clinical indication of fungal infection was present in 32% (12/38) of patients with IAD. Absorbent disposable briefs were the most common incontinence aids used (80%, 70/91), with soap/water and disposable washcloths being the clean-up products most commonly available (60%, 55/91) at the bedside. Further data are needed to validate this high prevalence. Studies that address prevention of IAD and the effectiveness of management strategies are also needed.

Management of atopic dermatitis in children : evaluation of parents' self-efficacy, outcome expectations, and self-reported task performance using the Child Eczema Management Questionnaire

Child behaviour management is crucial to successful treatment of atopic dermatitis. This study tested relationships between parents’ self-efficacy, outcome expectations, and self-reported task performance when caring for a child with atopic dermatitis. Using a cross-sectional study design, a community-based convenience sample of 120 parents participated in pilot-testing of the Child Eczema Management Questionnaire - a self-administered questionnaire which appraises parents’ self-efficacy, outcome expectations, and self-reported task performance when managing atopic dermatitis. Overall, parents’ self-reported confidence and success with performing routine management tasks was greater than that for managing their child’s symptoms and behaviour. Therewas a positive relationship between time since diagnosis and self-reported performance of routine management tasks; however, success with managing the child’s symptoms and behaviour did not improve with illness duration. Longer time since diagnosis was also associated with more positive outcome expectations of performing tasks that involved others in the child’s care (i.e. healthcare professionals, or the child themselves). This study provides the foundation for further research examining relationships between child, parent, and family psychosocial variables, parent management of atopic dermatitis, and child health outcomes. Improved understanding of these relationships will assist healthcare providers to better support parents and families caring for children with atopic dermatitis. KEYWORDS

Childhood atopic dermatitis : a cross-sectional study of relationships between child and parent factors, atopic dermatitis management, and disease severity

Background: Successful management of atopic dermatitis poses a significant and ongoing challenge to parents of affected children. Despite frequent reports of child behaviour problems and parenting difficulties, there is a paucity of literature examining relationships between child behaviour and parents' confidence and competence with treatment. Objectives: To examine relationships between child, parent, and family variables, parents' self-efficacy for managing atopic dermatitis, self-reported performance of management tasks, observed competence with providing treatment, and atopic dermatitis severity. Design: Cross-sectional study design. Participants A sample of 64 parent-child dyads was recruited from the dermatology clinic of a paediatric tertiary referral hospital in Brisbane, Australia. Methods: Parents completed self-report questionnaires examining child behaviour, parents' adjustment, parenting conflict, parents' relationship satisfaction, and parents' self-efficacy and self-reported performance of key management tasks. Severity of atopic dermatitis was assessed using the Scoring Atopic Dermatitis index. A routine home treatment session was observed, and parents' competence in carrying out the child's treatment assessed. Results: Pearson's and Spearman's correlations identified significant relationships (p< .05) between parents' self-efficacy and disease severity, child behaviour difficulties, parent depression and stress, parenting conflict, and relationship satisfaction. There were also significant relationships between each of these variables and parents' self-reported performance of management tasks. More profound child behaviour difficulties were associated with more severe atopic dermatitis and greater parent stress. Using multiple linear regressions, significant proportions of variation in parents' self-efficacy and self-reported task performance were explained by child behaviour difficulties and parents' formal education. Self-efficacy emerged as a likely mediator for relationships between both child behaviour and parents' education, and self-reported task performance. Direct observation of treatment sessions revealed strong relationships between parents' treatment competence and parents' self-efficacy, outcome expectations, and self-reported task performance. Less competent task performance was also associated with greater parent-reported child behaviour difficulties, parent depression and stress, parenting conflict, and relationship dissatisfaction. Conclusion: This study revealed the importance of child behaviour to parents' confidence and practices in the context of atopic dermatitis management. Children with more severe atopic dermatitis are at risk of presenting with challenging behaviour problems and their parents struggle to manage the condition successfully.

Transforming growth factor -beta signaling through Smad3 in allergy : Studies in the mechanisms of asthma, atopic dermatitis and allergic contact reactions

Transforming growth factor β signalling through Smad3 in allergy Allergic diseases, such as atopic dermatitis, asthma, and contact dermatitis are complex diseases influenced by both genetic and environmental factors. It is still unclear why allergy and subsequent allergic disease occur in some individuals but not in others. Transforming growth factor (TGF)-β is an important immunomodulatory and fibrogenic factor that regulates cellular processes in injured and inflamed skin. TGF-β has a significant role in the regulation of the allergen-induced immune response participating in the development of allergic and asthmatic inflammation. TGF-β is known to be an immunomodulatory factor in the progression of delayed type hypersensitivity reactions and allergic contact dermatitis. TGF-β is crucial in regulating the cellular responses involved in allergy, such as differentiation, proliferation and migration. TGF-β signals are delivered from the cytoplasm to the nucleus by TGF-β signal transducers called Smads. Smad3 is a major signal transducer in TGF-β -signalling that controls the expression of target genes in the nucleus in a cell-type specific manner. The role of TGF-β-Smad3 -signalling in the immunoregulation and pathophysiology of allergic disorders is still poorly understood. In this thesis, the role of TGF-β-Smad -signalling pathway using Smad3 -deficient knock out mice in the murine models of allergic diseases; atopic dermatitis, asthma and allergic contact reactions, was examined. Smad3-pathway regulates allergen induced skin inflammation and systemic IgE antibody production in a murine model atopic dermatitis. The defect in Smad3 -signalling decreased Th2 cytokine (IL-13 and IL-5) mRNA expression in the lung, modulated allergen induced specific IgG1 response, and affected mucus production in the lung in a murine model of asthma. TGF-β / Smad3 -signalling contributed to inflammatory hypersensitivity reactions and disease progression via modulation of chemokine and cytokine expression and inflammatory cell recruitment, cell proliferation and regulation of the specific antibody response in a murine model of contact hypersensitivity. TGF-β modulates inflammatory responses - at least partly through the Smad3 pathway - but also through other compensatory, non-Smad-dependent pathways. Understanding the effects of the TGF-β signalling pathway in the immune system and in disease models can help in elucidating the multilevel effects of TGF-β. Unravelling the mechanisms of Smad3 may open new possibilities for treating and preventing allergic responses, which may lead to severe illness and loss of work ability. In the future the Smad3 signalling pathway might be a potential target in the therapy of allergic diseases.

Tacrolimus ointment for long-term treatment of atopic dermatitis

Atopic dermatitis (AD) or atopic eczema is characterised by a superficial skin inflammation with an overall Th2 cell dominance and impaired function of the epidermal barrier. Patients also are at an increased risk for asthma and allergic rhinitis. Treatment with tacrolimus ointment inhibits T cell activation and blocks the production of several inflammatory cytokines in the skin, without suppressing collagen synthesis. The aims of this thesis were to determine: (1) long-term efficacy, safety, and effects on cell-mediated immunity and serum IgE levels in patients with moderate-to-severe AD treated for 1 year with tacrolimus ointment or a corticosteroid regimen, (2) the 10-year outcome of eczema, respiratory symptoms, and serum IgE levels in AD patients initially treated long-term with tacrolimus ointment, and (3) pharmacokinetics and long-term safety and efficacy of 0.03% tacrolimus ointment in infants under age 2 with AD. Cell-mediated immunity, reflecting Th1 cell reactivity, was measured by recall antigens and was at baseline lower in patients with AD compared to healthy controls. Treatment with either 0.1% tacrolimus ointment or a corticosteroid regimen for one year enhanced recall antigen reactivity. Transepidermal water loss (TEWL), an indicator of skin barrier function, decreased at months 6 and 12 in both tacrolimus- and corticosteroid-treated patients; TEWL for the head and neck was significantly lower in tacrolimus-treated patients. Patients in the 10-year open follow-up study showed a decrease in affected body surface area from a baseline 19.0% to a 10-year 1.6% and those with bronchial hyper-responsiveness at baseline showed an increase in the provocative dose of inhaled histamine producing a 15% decrease in FEV1, indicating less hyper-responsiveness. Respiratory symptoms (asthma and rhinitis) reported by the patient decreased in those with active symptoms at baseline. A good treatment response after one year of tacrolimus treatment predicted a good treatment response throughout the 10-year follow-up and a decrease in total serum IgE levels at the 10-year follow-up visit. The 2-week pharmacokinetic and the long-term study with 0.03% tacrolimus ointment showed good and continuous improvement of AD in the infants. Tacrolimus blood levels were throughout the study low and treatment well tolerated. This thesis underlines the importance of effective long-term topical treatment of AD. When the active skin inflammation decreases, cell-mediated immunity of the skin improves and a secondary marker for Th2 cell reactivity, total serum IgE, decreases. Respiratory symptoms seem to improve when the eczema area decreases. All these effects can be attributed to improvement of skin barrier function. One potential method to prevent a progression from AD to asthma and allergic rhinitis may be avoidance of early sensitisation through the skin, so early treatment of AD in infants is crucial. Long-term treatment with 0.03% tacrolimus ointment was effective and safe in infants over age 3 months.

Topical tacrolimus in atopic dermatitis : Effects of long-term treatment on skin and respiratory symptoms

Objective: Patients with atopic dermatitis often have a poor long-term response to conventional topical or systemic treatments. Staphylococcal superinfections, skin atrophy due to corticosteroid use, and asthma and allergic rhinitis are common. Only a few, usually short-term, studies have addressed the effects of different treatments on these problems. Tacrolimus ointment is the first topical compound suitable for long-term treatment. The aim of this thesis was to evaluate the effects of long-term topical tacrolimus treatment on cutaneous staphylococcal colonization, collagen synthesis, and symptoms and signs of asthma and allergic rhinitis. Methods: Patients with moderate-to-severe atopic dermatitis were treated with intermittent 0.1% tacrolimus ointment in prospective, open studies lasting for 6 to 48 months. In Study I, cutaneous staphylococcal colonization was followed for 6 to 12 months. In Study II, skin thickness and collagen synthesis were followed by skin ultrasound and procollagen I and III propeptide concentrations of suction blister fluid samples for 12 to 24 months and compared with a group of corticosteroid-treated atopic dermatitis patients and with a group of healthy subjects. Study III was a cross-sectional study of the occurrence of respiratory symptoms, bronchial hyper-responsiveness, and sputum eosinophilia in atopic dermatitis patients and healthy controls. In Study V, the same parameters as in Study III were assessed in atopic dermatitis patients before and after 12 to 48 months of topical tacrolimus treatment. Study IV was a retrospective follow-up of the effect of tacrolimus 0.03% ointment on severe atopic blepharoconjunctivitis and conjunctival cytology. Results: The clinical response to topical tacrolimus was very good in all studies (p≤0.008). Staphylococcal colonization decreased significantly, and the effect was sustained throughout the study (p=0.01). Skin thickness (p<0.001) and markers of collagen synthesis (p<0.001) increased in the tacrolimus-treated patients significantly, whereas they decreased or remained unchanged in the corticosteroid-treated controls. Symptoms of asthma and allergic rhinitis (p<0.0001), bronchial hyper-responsiveness (p<0.0001), and sputum eosinophilia (p<0.0001) were significantly more common in patients with atopic dermatitis than in healthy controls, especially in subjects with positive skin prick tests or elevated serum immunoglobulin E. During topical tacrolimus treatment the asthma and rhinitis (p=0.005 and p=0.002) symptoms and bronchial hyper-responsiveness (p=0.02) decreased significantly, and serum immunoglobulin E and sputum eosinophils showed a decreasing trend in patients with the best treatment response. Treatment of atopic blepharoconjunctivitis resulted in a marked clinical response and a significant decrease in eosinophils, lymphocytes, and neutrophils in the conjunctival cytology samples. No significant adverse effects or increase in skin infections occurred in any study. Conclusions: The studies included in this thesis, except the study showing an increase in skin collagen synthesis in tacrolimus-treated patients, were uncontrolled, warranting certain reservations. The results suggest, however, that tacrolimus ointment has several beneficial effects in the long-term intermittent treatment of atopic dermatitis. Tacrolimus ointment efficiently suppresses the T cell-induced inflammation of atopic dermatitis. It has a normalizing effect on the function of the skin measured by the decrease in staphylococcal colonization. It does not cause skin atrophy as do corticosteroids but restores the skin collagen synthesis in patients who have used corticosteroids. Tacrolimus ointment has no marked systemic effect, as the absorption of the drug is minimal and decreases along with skin improvement. The effects on the airway: decrease in bronchial hyper-responsiveness and respiratory symptoms, can be speculated to be caused by the decrease in T cell trafficking from the skin to the respiratory tissues as the skin inflammation resolves, as well as inhibition of epicutaneous invasion of various antigens causing systemic sensitization when the skin barrier is disrupted as in atopic dermatitis. Patients with moderate-to-severe atopic dermatitis seem to benefit from efficient long-term treatment with topical tacrolimus.