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1.
Artigo em Inglês | MEDLINE | ID: mdl-34491662
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Artigo em Inglês | MEDLINE | ID: mdl-34491666

RESUMO

BACKGROUND: Quality of life (QoL) has not been evaluated in Indian patients having epidermolysis bullosa (EB). AIMS: The aims of the study were to measure health-related QoL in Indian patients having EB using the quality of life in epidermolysis bullosa (QoLEB) questionnaire, and to find its correlation with clinically measured disease severity. METHODS: In this observational cross-sectional study, the QoLEB questionnaire was translated from English to Hindi (QoLEB-Hin) and culturally adapted without a change in concept following standard guidelines. QoLEB-Hin and three clinical scores that have been independently validated in EB, that is, Birmingham Epidermolysis Bullosa severity score (BEBs), Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB) and Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI), were administered to EB patients/their parents in the presence of an expert. This was followed by validity and correlation studies. RESULTS: Fifty-four patients were recruited (19-females, 35-males; median age 5 years, range 0.025-36 years and 12 patients with an age >13 years). The parents answered the questions for 42 patients (age <13 years). Dystrophic epidermolysis bullosa was diagnosed in 32 (59.2%) patients (dominant dystrophic epidermolysis bullosa [DDEB]-19 [35.2%] and recessive dystrophic epidermolysis bullosa [RDEB]-13 [24.1%]). Junctional epidermolysis bullosa (JEB) and epidermolysis bullosa simplex (EBS) were each diagnosed in 11 (20.4%) patients. The mean ± standard deviation (SD) of QoLEB-Hin score of all epidermolysis bullosa patients was 11.3 ± 7.6 (range 0-28; median and interquartile range [IQR], 10, 10) and reflected an overall moderate degree of affliction on QoL of patients. Mean ± SD of QoLEB-Hin scores for EBS, JEB, DDEB and RDEB were 5.4 ± 3.7 (range, 1-13; median and IQR, 6, 6), 11 ± 6.2 (range, 1-22; median and IQR, 10, 6), 9 ± 5.7 (range, 0-19; median and IQR, 10, 10) and 20.1 ± 6.4 (range, 12-28; median and IQR, 19, 12.5), respectively (P < 0.001, Kruskal-Wallis analysis of variance). Cronbach's alpha coefficient of 0.946 was obtained for all items indicating excellent internal consistency and reliability. Mean sample adequacy was 0.91; absolute fit based off diagonal values was 0.99; indices root mean square error of approximation and root mean square residual were 0.04 and 0.05, respectively, and Tucker Lewis index was >1 indicating overfit. The mean time taken to complete the questionnaire was 6.1 min (range, 6-8 min). QoLEB-Hin correlated significantly (P < 0.001) with BEBs (ρ = 0.79), iscorEB (ρ= 0.63) and EBDASI (ρ = 0.77). Three multiple linear regression models were used to ascertain the strength of relationship between QoL-Hin, and BEBs, iSCOREB and EBDASI, respectively, after adjusting for age, gender and disease subtype. The EBDASI clinical score accounted for approximately 74% (R2 = 0.736, P < 0.001) of the variability in QOL-Hin, as compared to 73% and 55% by BEBs (R2 = 0.731, P < 0.001) and iscorEB (R2 = 0.545, P < 0.001), respectively. LIMITATIONS: Parents filled out the questionnaires for many patients and probably led to an overall moderate degree of affliction of QoL. Comparison with Dermatology Life Quality Index and other QoL scores were not done in this study. Furthermore, the scoring was done at one point in time, and test-retest measurements could not be performed. CONCLUSION: This study validated QoLEB-Hin in an Indian population finding an overall moderate reduction in QoL due to EB. Maximally affected QoL was seen in patients with RDEB. Furthermore, QoLEB-Hin had a variable positive correlation and association with all clinical severity assessment scores.

6.
Artigo em Inglês | MEDLINE | ID: mdl-34491667

RESUMO

BACKGROUND: The subtypes of chronic urticaria share a common clinical expression, but may show differences phenotypically. Meanwhile, two or more different subtypes of chronic urticaria can coexist in any given patient which may involve different phenotypes. AIMS: The study aims to compare the two phenotypes in terms of demographics, clinical profile and treatment response. METHODS: In this retrospective study, 2678 chronic urticaria patients were divided into the single subtype chronic urticaria group and mixed subtype chronic urticaria group as was appropriate.The differences in the clinical features, possible causes, urticaria activity score of seven days, dermatology life quality index score, laboratory investigations and response to treatments were evaluated among the two groups. RESULTS: An obvious female predominance was detected in chronic urticaria, especially in mixed subtype chronic urticaria patients. Of the 2678 chronic urticaria patients, there were 837(31.25%) mixed subtype chronic urticaria. Chronic spontaneous urticaria combined with symptomatic dermographism was the most common group in the mixed subtype chronic urticaria. Patients with mixed subtype chronic urticaria were more likely to have associated chest tightness/shortness of breath and showed greater urticaria activity. In patients with single subtype chronic urticaria, the positive rate of family history with allergic rhinitis, asthma or urticaria was lower. Based on evaluation of the treatment, control with second-generation antihistamines at licensed doses was achieved in only 38.83% of mixed subtype chronic urticaria patients, compared with 56.32% of patients with single subtype. LIMITATIONS: First, this study was a single-center design retrospective study. Second, omalizumab treatment was not included. Third, the differences between different subtypes of mixed subtype chronic urticaria were not discussed in detail. CONCLUSION: This study showed that mixed subtype chronic urticaria had some distinct features. Comprehensive knowledge about it may help us define effective therapeutic strategies and improve symptom control and the quality of life for chronic urticaria patients.

7.
Artigo em Inglês | MEDLINE | ID: mdl-34491668

RESUMO

BACKGROUND: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. AIM: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. METHOD: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. RESULTS: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. LIMITATIONS: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. CONCLUSION: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

8.
Artigo em Inglês | MEDLINE | ID: mdl-34491669

RESUMO

BACKGROUND: The indirect immunofluorescence test is useful in the serodiagnosis of pemphigus. As indirect immunofluorescence titers correlate with disease activity in pemphigus, it is often used as a monitoring tool. The sensitivity of indirect immunofluorescence depends on the substrate used, and the preferred substrates are monkey esophagus for pemphigus vulgaris and normal human skin for pemphigus foliaceus. AIMS: We evaluated oral mucosa as a substrate for indirect immunofluorescence in pemphigus. METHODS: Fifty patients with pemphigus (40 with pemphigus vulgaris and ten with pemphigus foliaceus) and 50 controls were enrolled for study. Demographic and clinical details were recorded and indirect immunofluorescence using two substrates (oral mucosa and normal human skin) was carried out in serial dilution. Desmoglein (Dsg) 1 and 3 enzyme-linked immunosorbent assay was also evaluated simultaneously. RESULTS: Indirect immunofluorescence was positive in 40 patients (80%) with oral mucosa substrate and 34 patients (68%) with normal human skin substrate. Circulating antibodies were detected with oral mucosa in 33 (82.5%) of the 40 pemphigus vulgaris patients and in 26 (65%) patients using normal human skin. Antibodies were detected in eight of the ten pemphigus foliaceus patients (80%) with normal human skin and in seven (70%) patients with oral mucosa. Dsg enzyme-linked immunosorbent assay was positive in 45 (90%) patients, and 37 of these were also indirect immunofluorescence positive with oral mucosa. In the five Dsg enzyme-linked immunosorbent assay-negative patients, indirect immunofluorescence with oral mucosa was positive in three. LIMITATIONS: A comparison of oral mucosa with monkey esophagus could not be performed. CONCLUSION: Oral mucosa is a suitable and sensitive substrate for indirect immunofluorescence in pemphigus. Further studies comparing the sensitivity of indirect immunofluorescence using oral mucosa with monkey esophagus are recommended.

16.
Artigo em Inglês | MEDLINE | ID: mdl-34491677

RESUMO

BACKGROUND: There are very few population-based studies on the prevalence of eczema among older persons Aims: To estimate the prevalence and types of eczema in those aged 65 years or more in the community and to evaluate the effectiveness of community-based interventions for case finding. METHODS: In the first stage of this cross-sectional survey, trained health workers of a non-governmental organization surveyed the eligible population and identified persons likely to have eczema. In the second stage, dermatologists examined such persons to ascertain the diagnosis. Statistical analysis was done using Epi Info software version 7. Prevalence of eczema was expressed in percentages. Chi-square test was used for comparing the difference in prevalence of eczema in various age groups and sex. RESULTS: Health workers identified 98 persons as possible cases of eczema after interviewing 385 older persons in the community. Among them 95 persons were examined by dermatologists and 44 were confirmed to have eczema (diagnostic accuracy of health workers = 46.3%).Point prevalence of eczema was 11.4% (44/385). Prevalence was similar in males and females. It was greater (18.2 %) among persons aged 81 years or more. Asteatotic eczema, gravitational eczema and lichen simplex chronicus were the more common types of eczema. LIMITATIONS: Possible underestimation of the prevalence rates due to limited medical knowledge of health workers; limited facilities for examination and investigations at the medical camps and home visits. CONCLUSION: There appears to be a considerable burden of eczema among older persons in the community. A community-based approach involving non-governmental organizations has the potential to identify cases and offer care close to their homes.

18.
Artigo em Inglês | MEDLINE | ID: mdl-34491679

RESUMO

BACKGROUND: Serration pattern analysis helps in the classification of subepidermal autoimmune blistering disorders; more precisely, it helps to differentiate epidermolysis bullosa acquisita from other subepidermal autoimmune blistering disorders. Most of the published reports of this tool have come from a single center. OBJECTIVES: The objectives of the study were to study the utility of serration pattern analysis in classifying subepidermal autoimmune blistering disorders. METHODS: Seventy five cases of subepidermal autoimmune blistering disorders were enrolled in this prospective study. A three millimeter punch biopsy was taken from the perilesional skin or mucosa for direct immunofluorescence; indirect immunofluorescence was carried out using salt-split skin. Subclassification of subepidermal autoimmune blistering disorders was done based on direct immunofluorescence, indirect immunofluorescence on salt-split skin, indirect immunofluorescence using knockout skin and serration pattern analysis findings. RESULTS: Indirect immunofluorescence was positive in 68 cases; 14 cases showed a dermal staining pattern while the rest showed either an epidermal or a combined pattern. All patients with epidermal or combined staining patterns showed "n" serrated pattern on direct immunofluorescence. Nine patients with dermal staining on indirect immunofluorescence also revealed an "n" serration pattern on direct immunofluorescence indicating the diagnosis of anti-p200 pemphigoid, and the rest showed a "u" serrated pattern. Three patients with negative indirect immunofluorescence showed "u" serration on direct immunofluorescence while the rest showed "n" serration. LIMITATIONS: ELISA and immunoblotting could not be performed due to resource constraints. CONCLUSION: Based on indirect immunofluorescence and serration pattern analysis, classification of the majority of patients with subepidermal autoimmune blistering disorders was possible in our study. Pattern recognition is a cost-effective tool and can be easily learnt. It is recommended to be practiced in all laboratories where facilities for advanced immunological diagnosis are unavailable.

19.
Artigo em Inglês | MEDLINE | ID: mdl-34491680

RESUMO

BACKGROUND: The tumor, nodes and metastasis (TNM) classification and stage grouping have been updated in the 8th edition of the American Joint Committee on Cancer (AJCC) melanoma staging manual. However, restaging all the previous cases are not recommended. AIMS: The aims of the study were to investigate the necessity of restaging Korean melanoma patients staged by the previous edition of the AJCC manual. METHODS: Differences in the staging criteria of the 7th and 8th editions of the AJCC manual were identified. The staging of 276 primary melanomas from January 2011 to December 2018 was classified by both 7th and 8th editions of the manual and their differences were compared. RESULTS: Staging by 7th and 8th edition of the AJCC manual differed in 64 cases (23.2%). The pathological prognostic staging changed in 35 (12.7%), and 29 (10.5%) had changes in only TNM classification but not the pathological staging. None of the patients needed additional sentinel lymph node biopsy or systemic treatment as a result of restaging. Additional counseling was needed for the patients, because melanoma-specific survival was increased in the 8th edition. LIMITATIONS: This is a retrospective study with relatively small number of patients at a single tertiary center in Korea. CONCLUSION: Assessment of the need for additional sentinel lymph node biopsy or systemic treatment is recommended because of the latest changes in the AJCC melanoma staging manual. Although the restaging of previously staged melanomas is not significantly needed in our patients, still the differences in TNM classification and/or pathological prognostic staging suggest the need to separately recognize the patients previously staged by 7th edition and recently staged by 8th edition. Careful counseling about melanoma-specific survival is needed for Asian patients.

20.
Artigo em Inglês | MEDLINE | ID: mdl-34491681

RESUMO

OBJECTIVES: The objectives of the study were to characterize the clinical profile of childhood leprosy presenting at tertiary leprosy care hospitals in the states of Bihar, West Bengal and Uttar Pradesh in India, and to determine the possible risk factors associated with disabilities at presentation. METHODS: Subjects were children with newly diagnosed leprosy registered for treatment at tertiary Leprosy Mission Hospitals in Muzaffarpur (Bihar), Purulia (West Bengal) and Faizabad (Uttar Pradesh), India, between June and December 2019. Demographic and leprosy characteristics were collected at the time of diagnosis. Parents/guardians were interviewed on reasons for delay in presenting at the hospital. Associations between various factors and delay in diagnosis were assessed. RESULTS: Among the 84 children, the mean (SD) age was 10 (3) years with a range of 4-14 years. There were more boys (58%) and most children were currently in school (93%), resident in rural areas (90%) and belonged to a lower socioeconomic status (68%). More children were diagnosed with multibacillary leprosy (69%), one-third of them being skin smear positive for Mycobacterium leprae. On presentation, 17% had deformity (5% grade 1 deformity and 12% grade 2), 29% had nerve involvement and skin lesions were spread across the body in half of the children. Mean (SD) duration of delay was 10.5 (9.8) months. Delayed presentation was more in boys (43% vs. 17%; P = 0.01), those without a history of migration for work compared to those who had a history of migration (40% vs. 9%; P = 0.008) and in those children who were from a poor economic status compared with those that came from a better economic status (44% vs. 7%; P = 0.001) Limitations: Because our study was conducted at tertiary care hospitals, the findings are not representative of the situation in the field. Furthermore, a comparison group of newly diagnosed adult leprosy patients with disability could have been included in the study. CONCLUSION: Childhood leprosy continues to occur in endemic pockets in India and a substantial number present with skin smear positivity and deformity. Guardians of these children cite many reasons for the delay in presentation.

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