Clinical experience and psychometric properties of the Children's Dermatology Life Quality Index (CDLQI), 1995-2012.

The Children's Dermatology Life Quality Index (CDLQI) is a widely used questionnaire to measure the quality of life of children aged from 4 to 16 years. The purpose of this review is to summarize all published data regarding the clinical experience of the CDLQI and its psychometric properties as a single reference source for potential users. A literature search was carried out to identify all articles describing the use of the CDLQI from 1995 to November 2012. One hundred and six articles were identified, with four excluded. The CDLQI has been used in 28 countries in 102 clinical studies and is available in 44 languages, including six cultural adaptations; a cartoon version is available in 10 languages. It has been used in 14 skin conditions and used in the assessment of 11 topical drugs, nine systemic drugs, 13 therapeutic interventions and two epidemiological and other studies. There is evidence of high internal consistency, test-retest reliability, responsiveness to change, and significant correlation with other subjective and objective measures. Rasch analysis has not been carried out and more information is needed concerning minimal clinically important difference; these are areas requiring further study.

Which children should we patch test?

BACKGROUND: Allergic contact dermatitis (ACD) in childhood was considered rare until recently. However, reports are increasing, which may reflect an increased incidence and/or more frequent patch testing of children. It is also likely that allergen exposure in children has changed with time. AIMS: To determine the most common contact allergens and the rate of positive patch-test reactions among children with suspected contact allergy. METHODS: We carried out a retrospective case study of 114 children (66 girls and 48 boys) aged from 3 to 15 years (median 11.5) patch tested over a 3-year period. Indications for patch testing included uncontrolled or deteriorating atopic dermatitis, localized dermatitis or a history of reacting to a specific allergen. RESULTS: Of 110 children for whom we had notes, 83 (75%) had a history of atopy. Positive reactions that were of current, past or possible relevance were seen in 61 children (54%); in 58 (52%) of 111 tested with the standard series (SS) and in 6 (10%) of 60 tested with the medicament series. None of the children patch tested to the corticosteroid (n = 47), shoe (n = 15), fragrance (n = 12), cosmetic (n = 10) or rubber (n = 5) series had a positive reaction. However, 11 (10%) reacted to rubber allergens within the SS and one of five to their own shoes. The lowest rate of relevant positive reactions was among those with deteriorating atopic dermatitis (22%) and facial (33%) or perioral dermatitis (40%), and the highest rate amongst those with eyelid (86%) or hand (71%) dermatitis. Nickel was the most common allergen (20%) in line with previous reports (82% female), followed by rubber chemicals (10%), fragrance (7.2%), cobalt (5.4%) and lanolin (wool alcohol) (4.5%). CONCLUSIONS: The reported incidence of ACD among children, in particular nickel and rubber allergy, appears to be increasing, which may relate to changing fashions and hobbies. Contact allergy should be considered in all children with dermatitis, particularly with eyelid or hand dermatitis, and patch testing carried out more frequently.

An audit of the impact of a consultation with a paediatric dermatology team on quality of life in infants with atopic eczema and their families: further validation of the Infants' Dermatitis Quality of Life Index and Dermatitis Family Impact score.

BACKGROUND: Atopic dermatitis (AD) accounts for 10-20% of referrals to secondary care dermatology, often requiring multiple visits and occupying much valuable time and resources. OBJECTIVES: We audited the usefulness (ease of use, reliability and sensitivity to change) of two simple and easy to use quality of life (QoL) measures, the Infants' Dermatitis Quality of Life Index (IDQOL) and Dermatitis Family Impact (DFI), for assessing the impact on QoL of AD in infants and their families in a routine clinical setting. We also examined the impact of an initial consultation with a dermatology team on AD severity and QoL impairment from the parent's perspective. METHODS: The parents of 203 infants (mean age 19.8 months) with AD attending paediatric dermatology clinics completed the DFI and IDQOL. The parents of 50 of these infants completed both questionnaires before first and second consultations. RESULTS: In the 203 children the mean of both the IDQOL and DFI scores was 8.47 (median 8 and 7 and SD 5.8 and 6.5, respectively). The IDQOL and DFI correlated well (r(s) = 0.776, P < 0.0001). The parent's assessment of the global severity of AD correlated well with the IDQOL score (r(s) = 0.636, P < 0.0001) but less well with the DFI (r(s) = 0.394, P < 0.001). The highest-scoring IDQOL items were itching and scratching, problems at bathtime and time taken to fall asleep. The highest-scoring DFI items were tiredness/exhaustion, sleep loss and emotional distress. In both measures these domains also correlated most strongly with eczema severity. After dermatology consultation the median global severity score, rated by 50 parents, fell from 2 (SD 0.83) to 1 (SD 0.8; 95% confidence interval, CI 0.5-1), the median IDQOL score fell from 8 (SD 5.92) to 5.5 (SD 5.92; 95% CI 2-5.5) and the median DFI score fell from 9 (SD 6.45) to 3 (SD 6.56; 95% CI 2-5.5). In 50 infants the median IDQOL scores for those infants with global AD severity scores of 1, 2 and 3 were 5 (SD 5.65), 8 (SD 4.27) and 14 (SD 5.67), respectively and improved by 10%, 38% and 64%, respectively while the median DFI scores improved by 54%, 56% and 79%, respectively. The most improved IDQOL items were the time taken to get to sleep and difficulty at mealtimes and the most improved DFI domains were tiredness/exhaustion and emotional distress in the parents. CONCLUSIONS: We have provided further important information on the effects of AD on infants and their families using the IDQOL and DFI QoL measures. We demonstrate the usefulness of these measures in routine clinical management of AD and show the beneficial effect for both infants and parents of the initial consultation by a dermatology team in a secondary care setting.

A comparative study of impairment of quality of life in children with skin disease and children with other chronic childhood diseases.

BACKGROUND: Chronic disease can have physical and psychological effects which affect social functioning. These effects can be better understood from the perspective of parent and child by the use of health-related quality of life (HRQL) measures. Various HRQL measures are now available, of which generic health measures have been the most widely used. These permit comparison between different diseases and also the normal population. OBJECTIVES: To cross-validate a new generic HRQL proxy measure for children, the Children's Life Quality Index (CLQI), with an established speciality-specific dermatological questionnaire, the Children's Dermatology Life Quality Index (CDLQI), in a group of children with chronic skin diseases. The impairment of HRQL in the same group of children with skin disease was then compared with that associated with other common chronic childhood diseases using the CLQI. METHODS: The CDLQI was completed by 379 children aged 5-16 years with skin disease of more than 6 months' duration. Their parents (n=379) and parents of 161 children aged 5-16 years with other chronic diseases were also asked to complete a proxy measure, the CLQI. RESULTS: Using linear regression analysis, the CLQI and the CDLQI scores showed a strong linear association (rs=0.72, P<0.001) and on a Bland-Altman plot, reasonably good agreement (expressing scores out of 100, the 95% limits of agreement were from -25.5/100 to 26.7/100). In the child's opinion psoriasis and atopic dermatitis (AD) caused the greatest impairment (CDLQI scores of 30.6% and 30.5%), followed by urticaria (20%) and acne (18%). Using the generic CLQI (scored 0-36), from the parental perspective the highest score was for AD (33%), followed by urticaria (28%), psoriasis (27%) and alopecia (19%). Comparing this with children with other chronic diseases, those with cerebral palsy had the highest score (38%), followed in descending order by those with generalized AD (33%), renal disease (33%), cystic fibrosis (32%), urticaria (28%), asthma (28%) and psoriasis (27%). Diseases such as epilepsy (24%) and enuresis (24%) scored higher than diabetes (19%), localized eczema (19%), alopecia (19%) and acne (16%). CONCLUSIONS: Using the CLQI we have shown that HRQL impairment in children with chronic skin disease is at least equal to that experienced by children with many other chronic diseases of childhood, with AD and psoriasis having the greatest impact on HRQL among chronic skin disorders and only cerebral palsy scoring higher than AD. Cross-validation of the CLQI with the CDLQI in the group of children with skin disease demonstrates a strong linear association and good agreement between the two.

Quality of life and acne in Scottish adolescent schoolchildren: use of the Children's Dermatology Life Quality Index (CDLQI) and the Cardiff Acne Disability Index (CADI).

BACKGROUND: Acne vulgaris is known to adversely affect all aspects of quality of life. However, although acne is thought to occur in the majority of adolescents, there are few data currently available on the impact of acne in this age group. OBJECTIVES: Measurement of the impairment of health-related quality of life (HRQoL) in teenage Scottish schoolchildren in a comparative study using two HRQoL questionnaires. A secondary objective was to collect data on the use and perceived efficacy of medical and over-the-counter (OTC) preparations. STUDY DESIGN: An anonymous cross-sectional survey of 200 adolescent (15-18 years) Dundee schoolchildren was conducted by means of two self-reported questionnaires: the Children's Dermatology Life Quality Index (CDLQI) and the Cardiff Acne Disability Index (CADI). Data on demographics and therapeutic modalities and their perceived efficacy were also collected. ANALYSIS: Statistical analysis was performed using the package Stata 7.0. RESULTS: Self-reported acne was present in 83% of teenagers (147/178), with similar sex distribution (54% male, 46% female). The overall mean CDLQI score (max. 30) was low 1.7 {6% impairment} (CI -1 to 0), range 0-19. Nine pupils scored between 5 and 9 {17-30% impairment} suggesting moderate HRQoL impairment and three scored > 10 {> 33% impairment} indicating severe impairment. The overall mean CADI score (max. 15) of 1.9 {13% impairment}, CI 0 to 1 (range 0-15) was also low, but 12 pupils scored between 5 and 9 {33-60% impairment}, one scoring 10 + {> 67% impairment} and one scoring the maximum, 15 {100% impairment}. There was no significant difference in mean scores between the sexes in either questionnaire (P = 0.5). There was good correlation between the results from the two questionnaires (Spearman's rho = 0.62). Three-quarters (75%) had used OTC products, of which only a third (33%) felt they helped 'a lot'. Fifteen per cent were receiving prescribed treatment from their doctors of which 66% found it helpful. CONCLUSIONS: Self-reported acne occurred in 83% (147/178) of the Scottish teenagers involved in this study, which confirms previous reports of a high prevalence of acne in teenagers. Cross-validation of the CLDQI and CADI demonstrated good correlation and both scales were easy to administer and identified 11% (16/147) of teenagers who perceive their lives to be significantly affected by their acne (8% moderately to severely, 3% severely). It is important to identify and treat such teenagers early to reduce the future socio-economic burden of their acne.

A pilot study on the use of wet wraps in infants with moderate atopic eczema.

Wet wrap therapy (WWT) is a well-established treatment for severe atopic dermatitis (AD). However little evidence exists to justify widespread use in the community for less severe eczema. We compared the efficacy of WWT with a standard regime of hydrocortisone, to control moderate AD in children. We carried out a single-observer, randomized, controlled pilot study in 19 children under 5 years of age, with AD of 30% or more body surface area, using only 1% hydrocortisone (HC) prior to the study. Group one applied HC once in the morning for 2 weeks, with wet wraps twice daily for week 1, but only at night for week 2. Group two applied HC twice daily without wet wraps. Both applied emollient twice daily and as necessary. The primary outcome measure was the Six Area, Six Sign Atopic Dermatitis (SASSAD) severity score, and the secondary outcome measures were the Infants Dermatology Quality of Life Index (IDQOL), the Dermatitis Family Impact (DFI) score and the weight of topical steroids and emollients used. Over the 2-week active therapy period the mean fall in SASSAD was 8 [95% confidence interval (CI), -18 to +2; P = 0.11] more in the non-WWT group, the median change in the IDQOL was 2 for Group one and 7 for Group two (95% CI for difference, -10 to +3; P = 0.24) and the median change in DFI score was 2 for Group one and 5 for Group two (95% CI for difference, -14 to +2; P = 0.42). This small study has shown that conventional therapy with HC and emollients alone is as effective as WWT for infants with moderately severe, widespread AD, and provides weak evidence to suggest that it may be more effective. We would not advocate routine use of WWT for moderate eczema without further evaluation.

Parental knowledge of topical therapies in the treatment of childhood atopic dermatitis.

Poor adherence with therapy is a major cause of treatment failure in atopic dermatitis. Reasons given are multifactorial, and include fear of real or imaginary side-effects, under-prescribing, failure to renew prescriptions on time, lack of time, and child refusal of therapy. Most important, however, is lack of knowledge about treatment, in particular the use of topical corticosteroid (TCS) therapy. We conducted a questionnaire-based study to determine the level of use and knowledge of commonly prescribed TCS preparations amongst parents or carers of 100 children attending paediatric outpatient clinics. Weakly potent TCSs were the most commonly used (86%), but poorly understood. Only 35 (41%) who had used hydrocortisone were aware that it was weakly potent, and 44% graded it as moderately potent. Of 65 who had used the moderately potent TCS clobetasone butyrate 0.05% (Eumovate); Glaxo Wellcome, Uxbridge, UK), 19 (29%) graded it as potent and eight (12%) as weak. Of 50 who had used betamethasone valerate 0.1% (Betnovate); Glaxo Wellcome, Uxbridge, UK), 42% did not grade it as potent. Understanding of TCS/antimicrobial combinations was generally worse. The hydrocortisone 1%/fusidic acid 2% combination (Fucidin H(R); Leo, Risborough, Bucks, UK) was graded as moderate or strong by 88% of the 74 who had used it. Over half (53%) of the 34 using the combination of clobetasone butyrate 0.05%/nystatin 100000 i.u./g tetracycline 3% (Trimovate); Glaxo Wellcome, Uxbridge, UK) assumed that it was a potent TCS. Forty-nine had used Fucibet (betamethasone valerate 0.1%, fusidic acid 2%; Leo, Risborough, Bucks, UK) but 34.5% did not grade it as potent. There was poor knowledge of the strengths of some of the most commonly used TCSs, and all steroid/antimicrobial combinations were perceived as being of greater potency than the constituent steroid alone. Fusidic acid was thought to be a steroid by almost half (46.9%) of the respondents. The packaging of the different products by some pharmaceutical companies is remarkably similar and labelling contains information on the compound and percentage rather than potency of the TCS. This may be a source of confusion. We recommend that manufacturers clearly label TCS products by potency as mild, moderate, potent or very potent and that packaging is sufficiently different for each strength of TCS or emollient to avoid confusion. In order to achieve optimal topical treatment for atopic dermatitis, patients and their carers must receive adequate information and training in how and when to use topical therapies in conjunction with written care plans.

Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency.

Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.

The Children's Dermatology Life Quality Index: validation of the cartoon version.

BACKGROUND: In 1995 the Children's Dermatology Life Quality Index (CDLQI) was developed as a tool to allow quality of life assessment of children with skin conditions. This initial questionnaire was in a written format. OBJECTIVES: Using the same validated questions, a full-colour cartoon version has been developed. The aim of this study was to validate this against the initial written questionnaire in a three-part study. METHODS: The first part of the study piloted the use of both versions in an outpatient setting. One hundred and one children completed both versions of the CDLQI in a random order. A further 66 children completed the cartoon CDLQI in outpatients, and subsequently completed the cartoon version on the same day at home, which was returned by post. The scores were compared. In the second part, in more controlled conditions to eliminate parental and investigator bias, 107 children with current dermatological problems were administered both versions of the CDLQI in a random order. The scores were analysed, and time to complete each version, and the child and parental preferences, were recorded. The third part assessed compliance by asking 546 children recently reviewed in dermatology clinics to return a single completed postal CDLQI. Half of the children were given the text, and half the cartoon version. RESULTS: The median age of participating children was 11 years. There was no significant difference in scores between the two versions in both parts 1 and 2, but the cartoon version was completed faster (median 90 s) than the written version (median 120 s) (P < 0.0001). Both children and their parents significantly preferred the cartoon version and found it easier to use. Forty-six per cent of the postal CDLQI questionnaires were returned; there was no difference in compliance between the two versions. CONCLUSIONS: The cartoon CDLQI is equivalent to the previously validated written CDLQI version, but is faster and easier for children to use, and is preferred by both children and parents.

The Infants' Dermatitis Quality of Life Index.

BACKGROUND: The impact on quality of life (QOL) caused by atopic dermatitis (AD) has been quantified in children and adults using established QOL measures. However, these are not suitable for use in infants under the age of 4 years, when AD usually develops. OBJECTIVES: To validate a new parent-generated QOL questionnaire, the Infants' Dermatitis QOL Index (IDQOL), which measures the impact of AD on the infant, and to provide further validation of the Family Dermatitis Index (FDI), which measures the impact of a child's dermatitis on the family. METHODS: Parents of 102 predominately caucasian infants under 4 years with AD (34 postal and 68 outpatients) were asked to complete the IDQOL and the FDI on two separate occasions to test for repeat validity. The Infants' Behavioural Check List (BCL) was also given to the study group and to parents of 22 normal control infants. Post-treatment IDQOL and FDI questionnaires were obtained from 25 of the study group. RESULTS: The return rate for initial questionnaires was 87.3% (61 boys, 28 girls) and for retest 70.6%. The mean score for IDQOL was 7.89 and for FDI 8.87. Spearman rank correlation between the IDQOL and FDI was high (r = 0.87). Correlations of IDQOL and FDI with clinical severity assessment by parents were lower (r = 0.58 for IDQOL and r = 0.5 for FDI). Test-retest data for IDQOL and FDI confirmed repeatability, there being negligible differences between the pairs using the method of Bland and Altman. The three highest scoring questions for IDQOL referred to itching and scratching, mood change and sleep disturbance. For the FDI they were parental sleep disturbance, tiredness and exhaustion, and emotional distress. Post-treatment questionnaires from 25 patients indicated sensitivity to clinical change with both IDQOL and FDI. Parameters of behaviour measured using the BCL in 82 study infants and 22 controls showed greater problems with frequent night-time wakening (43% vs. 4.5%) and miserable mood changes (24.4% vs. 9%) in the study infants. CONCLUSIONS: Initial validation of the IDQOL and further validation of the FDI show good test-retest repeatability and apparent sensitivity to change with treatment. The effect on health-related QOL as measured by these methods is poorly correlated with clinical severity, confirming that QOL measures should be used in conjunction with clinical measures for global assessments of disease impact. This work requires further validation but suggests that QOL measures may be useful as outcome measures in clinical practice and research. Their simple construction allows quick and easy use, which is particularly valuable in large-scale and postal studies.

The family impact of childhood atopic dermatitis: the Dermatitis Family Impact Questionnaire.

Little information is available about the effect of childhood atopic dermatitis (AD) on family function. The aim of this study was to identify the areas of family life most affected and their perceived importance. Intensive qualitative interviews with 34 families were conducted and 11 basic problem areas were identified. A detailed questionnaire was prepared, part of which addressed the perceived importance of particular issues using the framework of multi-attribute utility theory. The results from using this questionnaire in 41 families were analysed and a shorter 10-question one-page Dermatitis Family Impact (DFI) questionnaire designed (maximum score = 30). In affected families the mean DFI score was 9.6 +/- 7.0 (range 0-27, n = 56) and in unaffected families the mean score was 0.4 +/- 0.9 (range 0-3, n = 26, P < 0.0001). The DFI could potentially be used as an extra measure in clinical studies, or to help guide appropriate management of AD.

Long-term efficacy and safety of cyclosporin in severe adult atopic dermatitis.

A prospective, open, multicentre study was performed to investigate the efficacy and safety of long-term treatment with cyclosporin in adults with severe atopic dermatitis. Subjects were treated for a maximum of 48 weeks. For the first 8 weeks, cyclosporin was administered at 2.5 mg/kg per day. The dose was then adjusted according to response. Disease activity was monitored using the six-area, six-sign score and the proportion of skin involved. Pruritus and sleep disturbance were assessed using four-point scales. Response was further evaluated on a five-point scale. Adverse events, blood pressure and serum biochemistry were monitored. Tolerability was assessed on a five-point scale. One hundred subjects were enrolled and 65 completed 48 weeks of treatment. Withdrawals occurred due to remission (three), inadequate response (seven), protocol violations (11) and adverse events (14, of which seven were probably treatment related). Cyclosporin produced rapid and highly significant improvements in all indices of disease activity. Sixty-five subjects considered that they had shown a considerable improvement or complete clearance of disease. Most patients relapsed after cessation of treatment, but neither signs nor symptoms had returned to baseline severity 8 weeks later. Blood pressure and serum creatinine levels increased slightly, and in one subject renal impairment was a major factor contributing to withdrawal of the drug. Overall, 85 subjects rated the tolerability of cyclosporin as good or very good. The results indicate that cyclosporin has a place in the long-term treatment of severe atopic dermatitis provided that appropriate patients are selected and careful monitoring is performed.

Localized unilateral hyperhidrosis secondary to an eccrine naevus.

We describe a patient with intermittent unilateral hyperhidrosis localized to the left hand only. Histology confirmed the presence of an eccrine naevus.

The Children's Dermatology Life Quality Index (CDLQI): initial validation and practical use.

Skin disease can cause severe disability and handicap in children. Measurement of the impact of skin disease on the quality of life is required to aid clinical decision-making, for clinical research, for audit of paediatric dermatology services, and for political reasons, to aid arguments for more resources for the care of children with skin disease. Adult measures are inappropriate, as the lives of children differ markedly from those of adults. The purpose of this study was to create and initially validate a simple practical questionnaire for use in children. One hundred and sixty-nine children, aged 3-16 years, attending a paediatric dermatology clinic, wrote down, with the help of their parents, all the ways in which their skin disease affected their lives. One hundred and eleven different aspects were identified; 10 questions were composed to cover these aspects, using a structure similar to the Adult Dermatology Life Quality Index. This draft questionnaire was piloted on two series, totalling 40 children, and minor alterations were made to improve clarity. The Children's Dermatology Life Quality Index (CDLQI) questionnaire (maximum score 30) was then given to a further 233 dermatology paediatric out-patients (CDLQI mean = 5.1, SD = 4.9), 47 normal controls (mean 0.4, 0.7) and 55 control patients attending a general paediatric clinic (mean 0.7, 2.5). The CDLQI scores for eczema (mean = 7.7, 5.6, n = 47), psoriasis (5.4, 5.0, n = 25) and acne (5.7, 4.4, n = 40), were all highly significantly greater than for moles and naevi (2.3, 2.9, n = 29). The highest mean score was that for scabies (mean = 9.5, 10.5, n = 6).(ABSTRACT TRUNCATED AT 250 WORDS)

Dapsone-induced motor peripheral neuropathy in pemphigus foliaceus.

We describe a case of pemphigus foliaceus complicated by dapsone-induced motor peripheral neuropathy. This idiosyncratic dapsone side-effect, which is rare other than in leprosy patients, was noteworthy for both its rapid onset, occurring within a month of commencing treatment, and its profound and prolonged nature.