Quality-of-Life Impairment in Neurofibromatosis Type 1
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Abstract:
Background
Neurofibromatosis type 1 affects quality of life (QoL) through association with severe complications, impact on cosmetic features, and uncertainty of the effects of the disorder.Objective
To evaluate the impact of the severity and visibility of neurofibromatosis type 1 on QoL.Design
Monocenter, cross-sectional study.Setting
One French academic dermatological and neurofibromatoses clinic.Patients
A total of 128 adult patients with neurofibromatosis type 1.Main Outcome Measures
Evaluation of severity and visibility using, respectively, the Riccardi and Ablon scales. Evaluation of skin disease–specific and general QoL using, respectively, Skindex-France and SF-36 (Short Form 36 health survey) profiles controlled for sex, age, severity, and visibility.Results
In a multiple regression model controlling for sex, age, and visibility, visibility remained independently associated with the alteration of 3 aspects of the skin disease–specific QoL (Skindex-France): emotions, physical symptoms, and functioning (P= .03,P= .009, andP= .002, respectively). Patients with more severe neurofibromatosis reported more effects on the following domains of their general health QoL (SF-36): physical function, bodily pain, general health perception, and vitality (P= .006,P= .03,P= .01, andP= .04, respectively).Conclusions
Neurofibromatosis type 1 has a significant impact on QoL through alteration of health and appearance. The consequences of visibility and severity from the viewpoint of patients can be evaluated using Skindex and the SF-36, respectively.Keywords:
Visibility
Vitality
Vitiligo
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Sir, Pigmentary disorders are cosmetically very disfiguring for patients and affect their quality of life. Acquired pigmentation in females can be caused by various dermatological conditions. Pigmented contact dermatitis (PCD), lichen planus pigmentosus (LPP), macular amyloidosis (MA), postinflammatory hyperpigmentation (PIH) prurigo pigmentosa, etc. can lead to diffuse as well as reticular pigmentation. Quality of life (QOL) indices assess the effects of disease on patients’ well-being. Originally, QOL instruments were created to address information about patient's biologic and psychosocial health, including ability to perform routine activities such as walking up and down stairs. The Dermatology Life Quality Index (DLQI) is a simple, practical, and self-administered questionnaire designed to assess limitations caused by the impact of skin disease.1 It is a health quality of life scale comprised of 10 items which focus on 6 dimensions: “symptoms,” “daily activities,” “leisure,” “work,” “personal relationships,” and “treatment.” The total score is calculated by adding the score of each question and ranges from a minimum of “0” to a maximum of “30,” with higher scores representing greater impairment of quality of life.1, 2 Interpretation of DLQI scores is done as: no effect at all on patient's life (0-1), small effect on patient's life (2-5), moderate effect on patient's life (6-10), very large effect on patient's life (11-20), and extremely large effect on patient's life (21-30). We assessed the quality of life in females presenting with acquired pigmentation involving exposed areas. Fifty-two adult females (19-48 years) who presented with such pigmentation on exposed sites, e.g face (n = 3), neck (n = 1), chest (n = 2), arms and forearms (n = 22), back (n = 18), and legs (n = 6) (with or without nonexposed sites), over a period of 1 year, were included in the study after getting a written informed consent. About 34.6% females had presented only because of cosmetic concerns, while rest had pruritus in addition. Diagnosis was made on the basis of clinical history, skin biopsy findings, direct immunofluorescence, and patch testing. Maximum number of females were diagnosed as PIH (n = 18), followed by MA (n = 15), PCD (n = 10), LPP (n = 7), confluent and reticulate papillomatosis (n = 1), and systemic sclerosis (n = 1). DLQI was measured by giving questionnaire-based proforma to the patients. Most of the patients (38.5%) showed moderate effects on their quality of life followed closely by very large effects on quality of life in 34.6% of patients. No effect on quality of life was seen in 3.8% of the patients (Figure 1). On analyzing individual conditions, in PIH moderate effect on quality of life was found in 50% patients. In MA, very large effect on patient's quality of life was found in 40% patients, which was closely followed by moderate effect in 33.3% of patients. In LPP and PCD very large and moderate effect on quality of life was found in of patients (40%) and (42.9%) (Figure 2). Overall, minimum score of quality of life was zero and maximum score was 25. Total mean score was 9.67 ± 5.313. Various studies regarding quality of life have been done in pigmentary disorders such as melasma, vitiligo, lentigo, postinflammatory hyperpigmentation, and idiopathic guttate hypomelanosis and have shown a significant negative impact on a person's health-related quality of life. DLQI had been studied in various dermatological disorders including hyperpigmentation disorders such as melasma, postinflammatory hyperpigmentaion, and vitiligo. In a study, of patients with diagnosed pigmentary disorders, using quality of life assessment questionnaire, majority of patients (53%) were not at all bothered by the skin's appearance. However, 47.3% of patients admitted, feeling self-conscious about their skin to some degree, 21.8% felt others focused on their skin, 32.7% felt unattractive because of their skin, 32.7% put effort into hiding pigmentary changes, and 23.6% felt their skin affected their activities. Majority of patients of postinflammatory hyperpigmentation did not show any significant effect on their quality of life in this study in contrast to our study which could be due to different inclusion criteria as we included female patients with hyperpigmentation involving exposed areas.3 In another study of health-related quality of Life in patients with primary cutaneous amyloidosis, cosmetic disfigurement and severe pruritus dramatically affected patient's quality of life in MA, which led to psychosocial stress and the impairment of emotional functioning.4 Patients with primary cutaneous amyloidosis experienced a significant impairment of life quality in terms of feelings, clothing, social, and leisure activities and daily routine. Strong impact of primary cutaneous amyloidosis was seen on patients’ quality of life both for dermatology-specific (using the DLQI) and for general health perceptions (using the SF-36). We suggest that quality of life measurements can be used in evaluation of patients presenting with pigmentation as this can be an important tool to assess the response to various therapeutic modalities.
Affect
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Chronic hand eczema (CHE) is a common disease that has a major impact on patients' health and on society.The purpose of this observational, open-label study was to assess the efficacy of treatment with 30 mg/d of oral alitretinoin on the quality of life (QoL) in a group of patients affected by CHE.This study included 15 patients, all suffering from severe CHE refractory to treatment with potent topical corticosteroids, who underwent treatment with 30 mg/d of alitretinoin for a period of 3 months. At the 1- and 3-month points, together with a clinical evaluation, the QoL of these patients were evaluated by the Dermatology Life Quality Index and visual analog scale (EQ5D-VAS).The oral administration of alitretinoin led to a notable QoL improvement among the patients, as shown by the statistically significant improvement in the Dermatology Life Quality Index and in the EQ5D-VAS after 1 and 3 months of therapy.
Refractory (planetary science)
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Hand dermatitis (HD) is inflammation of the skin of the hands. It ranges in severity and has a significant impact on quality of life (QoL). The physical and psychosocial burden of the disease is comparable with psoriasis in impairment of the QoL of the patient and depression. Quality of life correlates with disease severity.To evaluate the management outcome of HD with a disease severity tool by the physician and quality of life index.This was a cross-sectional study of a cohort of patients with HD over 24 weeks in the dermatology clinic of a tertiary hospital in Nigeria. Disease severity and quality of life were measured using the physician global assessment (PGA) and dermatology life quality index (DLQI), respectively. These were re-administered 6 weeks after treatment. Results were collated and analyzed.One hundred and seventy-seven (13.3%) of 1,329 patients had HD with F/M ratio of 1.2: 1; a mean age of 34.6 ± 7.4 years. Comparing PGA scores at baseline and 6 weeks, a statistically significant improvement was noted P < 0.001. The mean DLQI score at baseline was 10.38 and reduced to 3.02 post-treatment. The difference between the two means of the DLQI was ≥5 from baseline signifying improved quality of life. The domain of symptoms and feelings was the most affected at baseline and most improved afterward.HD has a significant impact on its sufferers and this correlates with disease severity. Appropriate management improves disease outcome and patients' quality of life.
Depression
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Omalizumab is a third-line treatment for chronic spontaneous urticaria (CSU). However, the real-life data on the impact of omalizumab on CSU-related quality of life (QoL) remain scarce.To investigate the impact of omalizumab on QoL and its predictors in CSU. A retrospective cohort study was done. The response to therapy was evaluated using urticaria activity score over 7 days (UAS7) and urticaria control test (UCT); the impairment in QoL was assessed using dermatology life quality index (DLQI) and chronic urticaria quality of life questionnaire (CU-Q2oL).Forty-two patients were included. All scores improved from baseline to first month and remained stable at the third month of treatment (p < .001). The gender, age, and angioedema had no significant effect on QoL, but the complete responders (UAS7:0-1) had better improvement rates in all scores compared to others. The baseline UAS7, DLQI, and CU-Q2oL scores were lower at the baseline in complete responders (p = .0001).A rapid and continual improvement in QoL was obtained with omalizumab treatment. A better UAS7, UCT, DLQI, and CU-Q2oL score at the baseline might be a predictor of a better response to omalizumab and more improvement in QoL.
Omalizumab
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アレルギー疾患への対応法は,これまでは医学的に病態を把握して治療を行うという視点で論じられてきた.生活者の視点で見る時に,アレルギー診療をスキルミクスで取り組むことや,Quality of Life(QOL)をものさしにするという発想がある.アトピー性皮膚炎でQOLに影響を及ぼす因子は,かゆみ,見た目,治療の負担,不安がある.アトピー性皮膚炎のQOL調査票は,成人ではSkindex-16,Dermatology Life Quality Indexがあり,日本語版がある.小児ではThe Children's Dermatology Life Quality Indexがあり,これにも日本語版がある.低年齢児では,養育者が主に治療を担うため,養育者の負担を評価するものが必要である.Quality of life in Primary Care givers of children with Atopic Dermatitisとその短縮版QP-CAD shortened to 9 questionsがある.患児と養育者双方のQOLを養育者が回答するthe Childhood Atopic Dermatitis Impact Scaleを日本の患者に合わせて修正したJapanese Culturally Modified Version of the Childhood Atopic Dermatitis Impact Scaleが利用できる.生活者の視点で医療を進めていく上で,オープンダイアローグという手法がありえる.
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Hidradenitis suppurativa is a recurrent, debilitating suppurative skin disease. The symptoms are much more than just physical, but studies of its impact on patients' psychological state and related quality of life are very limited. The current study was conducted with a group of 54 patients, aged 16-65 years, who had an active, but stable, course of hidradenitis suppurativa. The aim of the study was to determine the influence of hidradenitis suppurativa on a broad spectrum of psychophysical factors. Assessment was carried out using several questionnaires, which revealed the following mean scores: Dermatology Life Quality Index (DLQI) (12.67 +/- 7.7), Beck Depression Inventory-Short Form (BDI-SF) (5.87 +/- 4.68), Evers et al. "6-Item Scale" (3.87 +/- 3.65), EQ-5D (0.66 +/- 0.23 (health index) and 56.78 +/- 18.84 (VAS)), Functional Assessment of Chronic Illness Therapy - Fatigue scale (FACIT-F) (32.06 +/- 11.01) and Quality of Life Enjoyment and Satisfaction Questionnaire Short Form (Q-LES-Q-SF) (56.44 +/- 15.17%). The results highlight the important impact of hidradenitis suppurativa on a wide spectrum of psychophysical aspects and impairment of related quality of life among patients. Our findings indicate that advancement of the disease seems to be the most important factor negatively influencing patients' well-being (p < 0.01). It is noteworthy that an anogenital location appears to impair the hidradenitis suppurativa patients' quality of life most of all (p < 0.05), but the occurrence of lesions on uncovered skin plays a crucial role in the stigmatization level (p < 0.05).
Hidradenitis Suppurativa
Depression
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Abstract Background/Objectives The effect of pediatric psoriasis on quality of life has been demonstrated, but data regarding its influence on caregiver quality of life are scarce. The objective was to investigate how psoriasis affects quality of life of children and their caregivers. Methods This multicenter study included 129 children with psoriasis and their caregivers, who were family members accompanying patients to the clinic. Patient quality of life was measured using the Child Dermatology Life Quality Index. Caregiver quality of life was assessed using Dermatological Family Impact Scale, a 15‐item questionnaire validated for use in the Turkish language. Results Mean Child Dermatology Life Quality Index score was 7.6, indicating a moderate effect on patient quality of life. Symptoms and feelings were the most severely impaired domains of patient quality of life, and emotions was the most severely impaired domain of caregiver quality of life. Dermatological Family Impact Scale score was significantly correlated with Child Dermatology Life Quality Index (correlation coefficient [ r ] = .554, P < .001) and Psoriasis Area and Severity Index ( r = .350, P < .001). Caregivers of patients receiving systemic agents or phototherapy had relative impairment of multiple domains of quality of life compared to caregivers of patients receiving topical treatment only. Conclusion Psychosocial effect of pediatric psoriasis was shown to extend beyond the individual, highlighting the importance of addressing patient and caregiver quality of life concerns in an integrated approach.
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The impact of atopic dermatitis (AD) on the patient's quality of life is relatively well known. However, the influence on the patient's spouse has never been studied.To evaluate the impact of AD on the quality of life, sleeping and sexual life of patients and their partners.In this cross-sectional study, patients and their partners completed a number of questionnaires asking about their general health and their quality of life [Short Form 12, Epworth, Dermatology Life Quality Index (DLQI)] and completed an idiosyncratic measure asking about their sexual functioning. AD severity was clinician rated using Scoring atopic dermatitis (SCORAD).A total of 266 patients were included. The mean DLQI score was 8.8. The physical and mental composite 12 scores were 50.7 and 39.5, respectively. These 3 scores were significantly related to SCORAD. A decrease in sexual desire due to AD was noted in 57.5% of patients. The quality of life of partners did not appear to be particularly impaired, but 36.5% reported that the appearance of eczema had an impact on their sex life.The influence of AD on sex life is significant both for the patients and their partners.
SCORAD
Spouse
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