Marfan syndrome in combination with severe atopic dermatitis: importance of early diagnosis and treatment of patients with allergic dermatitis in childhood

2021 ◽  
Vol 16 (2) ◽  
pp. 68-73
Author(s):  
P.G. Svist ◽  
◽  
E.V. Orlova ◽  
L.M. Smirnova ◽  
S.S. Parshintseva ◽  
...  

We report a case of a rare combination of Marfan syndrome and severe atopic dermatitis (AD) in a 32-year-old male patient. He developed AD in early childhood and then had a relapse at the age of 18 years caused by stress. After this, AD became severe due to stress associated with aortic aneurysmectomy. The patient also had side effects caused by steroids, including multiple pustular eruptions, Kaposi's varicelliform eruption, edema and hyperemia of the periorbital area, pain and swelling of the small joints of the hands. These side effects, as well as specific cardiovascular symptoms typical of Marfan syndrome necessitated the search for alternative treatment strategies for severe AD. Eventually, we managed to find an effective treatment regimen that caused no side effects or adverse events, while the symptoms subsided. This case demonstrates the importance of early AD diagnosis in childhood in order to prevent its transition into severe chronic form that can hardly be managed in adulthood. Key words: atopic dermatitis, children, diagnostic criteria, clinical manifestations, hereditary diseases, Marfan syndrome

2019 ◽  
Vol 21 (10) ◽  
pp. 734-748 ◽  
Author(s):  
Baoling Guo ◽  
Qiuxiang Zheng

Aim and Objective: Lung cancer is a highly heterogeneous cancer, due to the significant differences in molecular levels, resulting in different clinical manifestations of lung cancer patients there is a big difference. Including disease characterization, drug response, the risk of recurrence, survival, etc. Method: Clinical patients with lung cancer do not have yet particularly effective treatment options, while patients with lung cancer resistance not only delayed the treatment cycle but also caused strong side effects. Therefore, if we can sum up the abnormalities of functional level from the molecular level, we can scientifically and effectively evaluate the patients' sensitivity to treatment and make the personalized treatment strategies to avoid the side effects caused by over-treatment and improve the prognosis. Result & Conclusion: According to the different sensitivities of lung cancer patients to drug response, this study screened out genes that were significantly associated with drug resistance. The bayes model was used to assess patient resistance.


2019 ◽  
Vol 32 (9) ◽  
pp. 606 ◽  
Author(s):  
Tiago Torres ◽  
Eduarda Osório Ferreira ◽  
Margarida Gonçalo ◽  
Pedro Mendes-Bastos ◽  
Manuela Selores ◽  
...  

With an increasing prevalence during the past decades, atopic dermatitis has become a global health issue. A literature search following a targeted approach was undertaken to perform this non-systematic review, which intends to provide an overview of the epidemiology, pathophysiology, clinical features, comorbidities, and current therapies for the treatment of atopic dermatitis. In sum, this is a heterogeneous skin disorder associated with variable morphology, distribution, and disease course. Although not completely understood, its pathogenesis is complex and seems to result from a combination of genetic and environmental factors that induce skin barrier dysfunction, cutaneous and systemic immune dysregulation, skin microbiota dysbiosis, and a strong genetic influence. Diagnosis is based on specific criteria that consider patient and family history and clinical manifestations. Overall disease severity must be determined by evaluating both objective signs and subjective symptoms. Therapeutic goals require a multistep approach, focusing on reducing pruritus and establishing disease control. Patients should be advised on basic skin care and avoidance of triggers. Topical anti-inflammatory agents should be considered in disease flares or chronic/recurrent lesions. In case of inadequate response, phototherapy, systemic immunosuppressants and, more recently, dupilumab, should be added. Nevertheless, the treatment of moderate-to-severe atopic dermatitis remains challenging and novel, efficacious, safe and targeted treatments are urgently needed. In conclusion, although the last few years have seen important improvement in the understanding of the disease, future research in atopic dermatitis will continue exploring gene-environment interactions and how it affects pathophysiology, disease severity, and treatment outcomes.


2018 ◽  
Vol 64 (1) ◽  
pp. 17-21
Author(s):  
Gyula Laszlo Fekete ◽  
László Fekete

AbstractObjectives: The aim of this clinical and therapy study was to evaluate the efficacy of NB-UVB phototherapy versus systemic therapy in moderate-to-severe atopic dermatitis of the adult.Material and methods: The subjects of the study were divided into two groups of 25 adult patients with moderate and severe atopic dermatitis according to the inclusion criteria. The first group of 25 patients were treated with systemic corticosteroids while the second group of 25 patients were treated with NB-UVB phototherapy. At the end of the study, after all the data were centralized, we performed a statistical analysis of the results, comparing the two groups as well as the efficacy of the different therapies.Results: In group I the clinical efficacy of the systemic corticosteroid treatment was achieved, on average, at 4 weeks in patients with moderate atopic dermatitis and at 6 weeks in patients with severe atopic dermatitis. In group II the clinical effecacy of NB-UVB phototherapy was achieved, on average, at 6 weeks for patients with moderate atopic dermatitis and at 8 weeks for those with the severe form. In both groups, the total IgE serum levels were elevated at the beginning, and they became normal throughout the clinical improvement. Remarkable therapy-related side effects were found in the first study group.Conclusion: We conclude that NB-UVB phototherapy had similar efficacy in treating moderate-to-severe atopic dermatitis with minimal side effects compared to systemic corticosteroid therapy.


2020 ◽  
pp. 27-35
Author(s):  
N. V. Kamut ◽  
M. M. Kiselova

The article is devoted to the study of features of clinical course of atopic dermatitis and vitamin D exchange in young children conducted by us by estimation of severity of atopic dermatitis depending on the value of SCORAD index, determination of 25(OH)D concentration in serum of children by immunochemical method with chemocentric analysis according to the classification approved by experts of the international endocrinological society. In the examination of 188 young children, two groups were formed on the basis of the Lviv City Children's Clinical Hospital. Children from 1 month to 1 year of age with manifestations of atopic dermatitis (n = 120), who were born full-term and had no birth defects, metabolic disorders, severe perinatal CNS lesions formed the main group. The comparison group consisted of 68 healthy young children without chronic diseases. All children had atopic dermatitis in the main group and were classified by severity (depending on the SCORAD index): children with mild severity – 31 (26 %), moderate 47 (39 %), and severe 42 (35 %). As a result of the study, we found a association between the severity of clinical manifestations of atopic dermatitis and the level of vitamin D in the serum. Comparative analysis of the severity of atopic dermatitis, depending on the level of vitamin D in the serum, showed that with severe atopic dermatitis in children of the main group, the level of 25(OH)D was significantly lower compared to its indicators in children with moderate to severe (P < 0.05). Serum vitamin D levels are significantly lower in all forms of atopic dermatitis severity and correlate significantly with the overall SCORAD severity score, erythema intensity, excoriation, itching, and sleep disturbances. The scientific data on the presence of feedback correlation (r = –0,48; P < 0,05) between the development of atopic dermatitis in children and their level of 25(OH)D in serum were added. Our findings suggest that there is a need for a differentiated approach to treating early-stage atopic dermatitis of varying severity, a personalized therapeutic approach to choosing a vitamin D supplementation, given the level of 25(OH)D in the serum.


2021 ◽  
Vol 97 (5) ◽  
pp. 26-37
Author(s):  
A. E. Karamova ◽  
V. V. Chikin ◽  
A. A. Kubanov ◽  
L. K. Davletbaeva

One of the methods of therapy for atopic dermatitis is long-wavelength ultraviolet therapy A (UVA-1- therapy). This review aims to provide the mechanisms of action of UVA-1-therapy an overview about the effectiveness of UVA-1-therapy in patients with atopic dermatitis taking into account factors that can affect the effectiveness of treatment radiation dose, skin phototype of patients, concomitant drug therapy. The available data on a decrease in the severity of atopic dermatitis as a result of the course of UVA-1-therapy and on a decrease in the severity of itching in patients are presented. The data on the rate of onset of the therapeutic effect of UVA-1-therapy and the duration of its maintenance are considered. The safety of UVA-1-therapy is discussed, and the most frequent undesirable effects a feeling of warmth, fever, itching, hyperpigmentation, are given. The possibility of developing side effects requiring discontinuation of treatment is assessed. The data obtained indicate the effectiveness and safety of the UFA-1-radiation in the treatment of patients with moderate-to-severe atopic dermatitis.


Asthma ◽  
2014 ◽  
pp. 115-138
Author(s):  
Neetu Talreja ◽  
Ronald Dahl

Primary ciliary dyskinesia, cystic fibrosis, and α‎1-antitrypsin deficiency are autosomal recessive hereditary diseases. These diseases should always be considered in cases of asthma. The diagnosis and management of these diseases, particularly with asthma, is an important challenge for clinicians. However, the diagnosis of these chronic diseases is evolving with better definition of phenotypic features and expansion of diagnostic tests. Optimizing and expanding access to the nongenetic tests is critical for ensuring a timely and accurate diagnosis. Early diagnostic strategies, better understanding of the complex interactions underlying the pathophysiology of lung disease, and emerging treatments show great promise for the future. The discovery of genetic and biomarker studies that will predict individuals at risk to develop the clinical manifestations of these diseases can lead to more personalized treatment strategies and a better prognosis.


1986 ◽  
Vol 14 (2) ◽  
pp. 85-90 ◽  
Author(s):  
G Rajka ◽  
H L Veijans

A randomized, double-blind, left-right study to compare the therapeutic efficacy and the cosmetic acceptability of the new hydrocortisone 17-butyrate (Locoid®) 0·1% fatty cream application form with desonide (Apolar®) 0·1% ointment was performed in thirty patients suffering from moderate to severe atopic dermatitis. The medications were applied to symmetrical, bilateral skin lesions twice daily for 4 weeks. Both treatments effected highly significant reductions of the score values for the severity of all clinical skin parameters assessed. Score reductions were, however, more pronounced on Locoid-treated sides than on Apolar-treated sides both after 2 and 4 weeks of therapy. It appeared further that clinical efficacy of treatment at completion of the study was also in favour of Locoid-treated sides, indicating that Locoid fatty cream is more effective than Apolar ointment. No serious side-effects were reported during the study. The expressed patient preferences with respect to cosmetic acceptability of treatments were significantly in favour of Locoid fatty cream, indicating that patients preferred the use of this new galenic formulation over an ointment formulation. It is concluded that the new application form of Locoid, a fatty cream, is a useful and beneficial addition to topical corticosteroid therapy, which will promote patient compliance in a wide range of corticosteroid-responsive skin diseases.


Author(s):  
Fernando Valenzuela ◽  
Javier Fernández ◽  
Marcela Aroca ◽  
Constanza Jiménez ◽  
Daniela Albers ◽  
...  

Atopic dermatitis (AD) is a protease-modulated chronic disorder with heterogenous clinical manifestations which may lead to an imprecise diagnosis. So far, there are no diagnostic protease tests for AD. We explored the gingival crevicular fluid (GCF) protease profile of periodontally-healthy individuals with moderate/severe AD compared to healthy controls. An exploratory case-control study was conducted. Matching AD patients (n=6) and controls (n=6) were enrolled at the International Center for Clinical Studies, Santiago, Chile. Complete dermatological and periodontal evaluations (involving the collection of GCF samples) were made. The levels of 35 proteases were analyzed using a human protease antibody array. The GCF levels of zinc-binding ADAM8, ADAM9, MMP8 and Neprilysin/CD10, aspartyl-binding Cathepsin E, and serin-binding Protein convertase9 and uPA/Urokinase proteases were lower in moderate/severe AD patients compared to controls (p&lt;0.05). No inter-group differences in the levels of the other 28 proteases were found. MMP8, Cathepsin E and ADAM9 were the biomarkers with the highest sensitivity and specificity regarding the detection of AD (p &lt; 0.05). The area under receiver operating characteristic (ROC) curve for MMP-8+ADAMP-9 was 0.90. In conclusion, differences in the protease profile between AD and control patients associated with MMP8, Cathepsin E and ADAM9. MMP8, ADAM9 and Cathepsin E may be useful as combined diagnostic and therapeutic biomarkers of moderate/severe AD.


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