Perception of xeroderma pigmentosum support group members of xeroderma pigmentosum lifestyle impact

Xeroderma pigmentosum (XP) is a genetic condition, which can cause an extreme sensitivity to sunlight and an increased risk of skin cancer due to errors in DNA repair. An online survey was administered to a convenience sample of participants who were members of an online support group for XP patients and their families to determine common symptoms and quality of life. The Dermatologic Life Quality Index (DLQI) or the Children's Dermatologic Life Quality Index (CDLQI) was used depending on patient age. A total of four patients and two parents of young patients completed our survey. Quality of life as measured through the DLQI and CDLQI was moderately affected.     

The impact of psoriasis on quality of life

Psoriasis is a chronic inflammatory skin disease in which the signs vary from one patient to another and over time. Traditionally, physicians have used various parameters to assess the severity of the disease: percentage of body surface area covered, erythema, plaque thickness, degree of scaling and systemic symptoms such as arthritis. However, these clinical assessments alone do not accurately reflect the overall effect of the disease on patients' daily activities. Apart from the clinical severity of affected areas, psoriasis can also have a profound psychosocial impact on the patient's quality of life. This concept is multidimensional, encompassing the physical, social and psychological wellbeing of the person and is based on the patient's view of their condition.     

Erythropoietic protoporphyria in the U.K.: clinical features and effect on quality of life

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare inherited photodermatosis that causes lifelong painful photosensitivity. Neither its full clinical spectrum nor its impact on quality of life (QoL) has been investigated in a large cohort of patients. OBJECTIVES: To document the clinical features of EPP and its impact on QoL in a high proportion of all patients with EPP resident in the U.K. METHODS: Patients with EPP were identified from U.K. clinical databases and assessed by the same clinical investigator over a 7-month period using a standardized proforma and validated adult (Dermatology Life Quality Index, DLQI) and children's (Children's Dermatology Life Quality Index, CDLQI) QoL questionnaires. RESULTS: Three hundred and eighty-nine living patients with EPP were identified, of whom 223 [114 females, 109 males; median age 34 years (range: 5-87), from 193 families] were investigated. Total erythrocyte porphyrin (TEP) was higher in males (median: 25.3 micromol L1) than females (median: 19.3 micromol L1). The median ages at onset and diagnosis were 1 and 12 years, respectively. Median times for onset of symptoms after sun exposure, onset of signs (oedema, erythema) and resolution of symptoms were 20 min, 6 h and 3 days, respectively. Most patients reported absence of protection by glass (92%), priming (85%), exacerbation by wind (68%), no family history of photosensitivity (56%), no symptoms during winter (56%) and had chronic skin lesions (79%). Symptoms changed little with age but improved during pregnancy in 47% of gravid women. Most patients used protective clothing and a sunscreen; 28% were taking beta-carotene and a further 56% had taken it; 29% were not under regular medical care. Two patients (1%) had liver failure and 8% reported gallstone disease. QoL was markedly impaired, with scores similar to those in severe dermatological disease (mean DLQI score 14.0, n = 176; mean CDLQI score 12.8, n = 44), indicating a large effect on patients' lives. DLQI scores correlated weakly with TEP (rs = 0.228; P = 0.002) and time to onset of symptoms (rs = -0.233; P = 0.002) but not with age at onset. CONCLUSIONS: EPP is a persistent, severely painful, socially disabling disease with a marked impact on QoL. Its diagnosis is often overlooked. None of TEP, age at onset nor time to onset of symptoms is a useful predictor of impaired QoL in individual patients.     

The Family Dermatology Life Quality Index: measuring the secondary impact of skin disease

BACKGROUND: Skin diseases are known to have a major impact on the lives of patients and their families. Many instruments are available to measure the health-related quality of life (HRQoL) of patients but no measure has been developed so far to quantify the secondary impact on family members of the patients. OBJECTIVES: To develop and validate a dermatology-specific instrument to measure the adverse impact on the HRQoL of family members of patients with skin disease. METHODS: Detailed semi-structured interviews were conducted with family members of patients to identify different aspects of HRQoL affected. An initial draft version of the questionnaire based on the main topic areas was pilot tested to assess the face and content validity. A 10-item questionnaire, the Family Dermatology Life Quality Index (FDLQI), was finalized after modifications to the draft questionnaire based on feedback from families and dermatology professionals and on item reduction. Psychometric evaluation was conducted on a new cohort of family members (n = 132) who completed the FDLQI and the patients (n = 109) who completed the Dermatology Life Quality Index (DLQI). RESULTS: Fifty-nine different aspects of family members' HRQoL were identified from the analysis of the interviews, which were categorized into main topic areas. Factor analysis of 10 items of the final questionnaire revealed two factors and together these explained 60% of the common variance. The FDLQI demonstrated high internal consistency (Cronbach's alpha = 0.88) and test-retest (intraclass correlation coefficient = 0.94) reliabilities. The responsiveness of the instrument to change was shown by significant change in the family members' FDLQI scores in cases where patients' clinical condition either improved or worsened. Construct validity was assessed by testing a number of a priori hypotheses. A strong correlation was seen between the family members' FDLQI scores and patients' DLQI scores (r = 0.69), a significantly higher FDLQI score was seen for inflammatory skin diseases compared with noninflammatory diseases/isolated lesions (P < 0.0001), and there was a positive relationship between the family members' FDLQI scores and patients' disease severity (r = 0.49). CONCLUSIONS: The FDLQI is simple and practical and seems to have the potential to be used as an additional outcome measure in clinical practice and evaluation research.     

Use of topical therapies for pediatric psoriasis: A systematic review

Psoriasis is one of the most common chronic skin diseases, affecting 1%‐3% of the general population. It can have a significant negative impact on a patient's quality of life, and in approximately 30% of patients first symptoms can be traced back to childhood. We have performed a comprehensive literature search using the MEDLINE database in order to ascertain the efficacy and adverse reactions of topical treatments in pediatric psoriasis. A total of 13 relevant articles were identified on the following topical agents: corticosteroids, calcineurin inhibitors, vitamin D analogs, and dithranol. Corticosteroids achieved clearance in 72.7% of patients. Calcitriol lead to a 57.2%‐100% mean improvement in severity, and calcipotriol to 52%‐64%. Combination of calcipotriol and corticosteroids achieved an improvement in mean severity ranging between 32.1% and 80%. Treatment with tacrolimus lead to an >50% improvement. Finally, short contact dithranol lead to a variable response in clearance between different studies, ranging between 3.7% and 81%. No serious adverse reactions were documented, the most common local reaction being irritation. Pediatric psoriasis is a common and challenging condition with no easy and definitive solution. Topical agents are safe, easy to use, readily available and cheap. However, they need to be applied repeatedly, may cause skin irritation, and can be messy. Based on the results presented above, we recommend utilizing all the available topical options before escalating to systemic treatments.     

The impact of changes in clinical severity on psychiatric morbidity in patients with psoriasis: a follow-up study

BACKGROUND: Psoriasis has a strong impact on quality of life and is correlated to psychopathological states. It is important to investigate the effect of clinical changes on psychological status. OBJECTIVES: To analyse the extent of clinical change and its effect on the presence of psychiatric morbidity in a group of patients with psoriasis. METHODS: All eligible adults hospitalized with psoriasis in a dermatological hospital (February 2000-February 2002) were given the self-administered Psoriasis Area and Severity Index (SAPASI) to assess clinical severity, the 12-item General Health Questionnaire (GHQ-12) to detect patients with psychological problems (defined as 'cases') and the Skindex-29 to evaluate symptoms. The same questionnaires were completed by the patients a month after hospital discharge. RESULTS: In our population of 414 patients, the incidence of GHQ cases becoming noncases was correlated with the SAPASI percentage improvement, ranging from 17.6% in patients with SAPASI worsened or unchanged at follow-up, to 68.2% in patients with clearance of psoriasis. Also, the proportion of patients who became GHQ noncases was much higher in patients with improvement of >/= 50% in symptoms, compared with patients with no improvement or worsening (70% vs. 32%, respectively). In a multivariate model the possible determinants of the passage from GHQ case to noncase were: SAPASI improvement, symptom improvement, no localization on the face, and gender (i.e. women were less likely to improve psychologically). CONCLUSIONS: The improvement in clinical severity and symptoms was associated with a decreased frequency of psychiatric disturbance. However, dermatologists should be aware that even in the presence of vast clinical improvement patients may still substantially suffer psychologically.     

The psychosocial and occupational impact of chronic skin disease

ABSTRACT:  Chronic skin diseases, particularly psoriasis and atopic dermatitis, have a negative impact on patients' quality of life. Patients often experience significant psychological and social distress such as increased levels of depression and fear of stigma. Skin diseases can also impact patients' occupational lives by causing them to miss work or be less productive. Quality of life instruments provide important information for healthcare professionals, the general public, and those involved in distribution of healthcare resources, which helps prevent chronic skin disease from being overlooked amidst other medical conditions.     

Evaluating the severity of dermatologic disorders

Assessment of a patient's disease severity is an essential component of formulating therapeutic strategies. However, disorders of the skin are often not amenable to strict classification criteria, and the dermatologist relies upon personal thresholds of severity when assessing the patient's overall condition. A number of grading systems have arisen, primarily from the need for standardized end points in clinical trials; in some circumstances, these severity assessments may assist the clinician in the evaluation and treatment of dermatologic disease. In this review, we will summarize the results of available severity scores of frequently encountered dermatologic disorders and discuss their utility in the management of disease in a clinician's office.     

The impact of topical photoprotectants intended for daily use on lifetime ultraviolet exposure

Background Exposure to solar ultraviolet (UV) radiation is believed to be an important contributor to facial photoaging. Daily application of topical photoprotectants is thought to mitigate this process. Objectives To examine the importance of a number of independent factors in reducing the lifetime UV exposure of facial skin achieved by daily use of photoprotective products. Methods A behavioral model of solar UV exposure to the face is incorporated with the spectral profile of two different candidate topical products, the age at which regular photoprotection begins, the SPF of the products, and whether the product is applied year‐round or just in the summer months to examine the reduction in lifetime UV exposures achieved by daily use of photoprotective products. Results The results show that regular use of topical photoprotective agents reduces significantly lifetime UV exposure to the face compared with nonuse. Analysis of variance shows that the most important factor is to begin regular daily use early in life. The SPF and spectral profile of the product is of lesser importance, as is whether daily use is confined to the summer months rather than year‐round. Conclusions While it remains unproven and speculative, there is good reason to suppose that regular use of daily facial topical products containing UV filters, particularly if started early in adult life, will be translated into fewer visible signs of aging later in life.     

Quality of life and psychosocial aspects in Greek patients with psoriasis: a cross-sectional study

BACKGROUND

Psoriasis is a common, long-term skin disease associated with high levels of psychological distress and a considerable adverse impact on life. The effects of psoriasis, beyond skin affliction, are seldom recognized and often undertreated.

OBJECTIVE

The aim of the study is to evaluate the quality of life, anxiety and depression, self-esteem and loneliness in patients with psoriasis.

METHODS

Eighty-four patients with psoriasis were enrolled in the study. The quality of life, depression and anxiety, loneliness and self-esteem of the patient were assessed using the Dermatology Life Quality Index, Hospital Anxiety and Depression Scale, the UCLA loneliness Scale (UCLA-Version 3) and Rosenberg''s Self-esteem Scale, respectively.

RESULTS

The Dermatology Quality of Life Index score among psoriasis patients was 12.61 ± 4.88. They had statistically significantly higher scores according to the Hospital Anxiety and Depression Scale -anxiety subscale (p=0.032)-compared with healthy volunteers. Moreover, a statistically significant difference was found between the two groups concerning the UCLA-scale (p=0.033) and RSES-scale (p<0.0001). Female patients presented with lower self-esteem than male patients.

CONCLUSION

Psoriasis is a distressing, recurrent disorder that significantly impairs quality of life. Therefore, the recognition and future management of psoriasis may require the involvement of multi-disciplinary teams to manage the physical, psychological and social aspects of the condition, as is the case for systemic, long-term conditions.     

Experiences of stigmatization play a role in mediating the impact of disease severity on quality of life in psoriasis patients

BACKGROUND: Psoriasis may have a severe impact on patients' quality of life (QOL) in several ways, an impact mediated by the mental or physical difficulties they have to deal with during the course of the disease and the various treatment regimens. In addition, psoriatic patients often suffer from experiences of stigmatization (EOS) related to the disease. OBJECTIVES: This study was designed to test the hypotheses that psoriasis patients report higher levels of stigmatization than a comparison group, and that their EOS play a role in mediating the impact of the severity of psoriasis on their QOL. METHODS: One hundred patients with psoriasis (study group) and 100 patients with mixed skin problems (comparison group) were matched according to age, sex and education. All subjects answered questionnaires on EOS and QOL. A dermatologist diagnosed the diseases and measured severity scores. The Psoriasis Area and Severity Index score was used for psoriasis and a linear severity score for the comparison patients. The mediating effect of EOS was analysed using structural equation modelling (SEM). SEM is a multivariate statistical method used to examine the consistency of a theory relating one group of variables (termed a 'latent construct') to another: in the present study, the relationship between EOS and QOL. RESULTS: Psoriatic patients were found to report significantly higher levels of EOS related to the disease, compared with the comparison group. No significant differences were found regarding QOL or severity of disease. Clinical severity of psoriasis was found to correlate negatively with QOL in psoriasis patients. EOS were found to have a complete mediating effect for the severity of disease on the QOL in patients with psoriasis. This result was not found among the comparison group patients. CONCLUSIONS: The results of this study indicate that psoriasis patients experience higher levels of stigmatization than do other dermatological patients, and that these EOS mediate the association between disease severity and patients' reported low levels of QOL. Treatment of psoriatic patients should consider these results and should include tools for psychosocial intervention.     

手部湿疹对生活质量的影响及派瑞松对其改善的研究

目的：研究手部湿疹对患者生活质量的影响以及派派瑞松对其的改善情况。方法：采用DLQI问卷，120例手部湿疹(HE)患者填写问卷，并外出派瑞松4周，分别在用药1周、2周、4周后再次填写问卷，然后进行分析。结果：DLQI分数在性别、年龄、病程、教育程度等没有差异。但是男性的问题主要集中在工作、关系和治疗上，而女性则是主要影响症状，家务和休闲，局部外用派瑞松1周可以改善患者的生活质量，4周则完全改善。结论：本研究证实HE可以严重影响患者的生活质量，而派瑞松可以有效地改善。     

The effect of pulsed dye laser on cutaneous leishmaniasis and its impact on the Dermatology Life Quality Index

Background and objective: Cutaneous leishmaniasis is a prevalent skin condition especially in the Middle Eastern region. The use of pulsed dye laser (PDL) for the treatment of cutaneous leishmaniasis (CL) has not been thoroughly evaluated. We assessed primarily the efficacy of 595-nm PDL in the treatment of CL lesions and interpreted its impact on the quality of life (QOL) of affected patients as measured by the Dermatology Life Quality Index (DLQI). Patients and methods: Twenty-five lesions from 12 patients were treated with a single pass of PDL over the whole lesions to develop a purpuric end point. Parameters used for this treatment were 7 J/cm² fluence, 10-mm spot size, and 0.45-ms pulse duration. The laser settings were maintained in all subsequent treatments. Results: Excellent response was noted in 13 of the 25 lesions after 3 sessions, while 12 of the remaining 25 lesions required 4 sessions toward complete recovery. The mean DLQI scores pre- and post-laser treatments were 12.67 and 4.25, respectively. All patients experienced a statistically significant improvement in their QOL (p < 0.05, paired t-test). Conclusion: The PDL is a new safe modality for treating cases of CL with further larger-scale studies could better determine its role.     

Occupational contact dermatitis has an appreciable impact on quality of life

In this study, we examined the impact of occupational contact dermatitis on quality of life. 181 patients, diagnosed with occupational contact dermatitis over a period of 3 years (1996–1999 inclusive), were sent a questionnaire based on the Dermatology Life Quality Index (DLQI) and the Short Form‐36 (SF‐36). 60 (32%) patients were in industrial occupations and 27 (14%) in health care. An overall response rate of 39% (n=70) was obtained. The median DLQI score was 5, with a mean score 6.6 (SD 6.4), which is similar to that seen in Behçet’s syndrome and urticaria. There was no statistically‐significant difference between male and female median scores (p=0.98) and no significant correlation between age and DLQI score nor between DLQI score and time from diagnosis. The most problematic quality of life areas were symptoms and feelings. Males scored highest in problems associated with work, relationships and treatment, whereas females scored highest in problems associated with symptoms and feelings, daily activities and leisure. The SF‐36 scores showed an association between physical problems and emotional problems affecting work. From this study, it can be seen that occupational contact dermatitis has an appreciable impact on quality of life.