Sun Avoidance Research Articles

Vitamin D level and its association with adiposity among multi-ethnic adults in Kuala Lumpur, Malaysia: a cross sectional study.

BackgroundVitamin D deficiency is highly prevalent in both temperate as well as tropical countries. Obesity is one of the factors contributing to vitamin D deficiency. As our country has a high prevalence of overweight and obesity, we aimed to study serum 25-hydroxyvitamin D (25(OH)D) level and its association with adiposity using various adiposity indicators; and to study other risk factors that affect serum 25(OH)D level among multi-ethnic adults in Kuala Lumpur, Malaysia.MethodsThis was a cross sectional study conducted with a multistage sampling. All permanent teachers working in government secondary schools in Kuala Lumpur were invited for the study. The data collection included serum 25(OH)D, Parathyroid Hormone (PTH), body fat percentage, waist circumference, body mass index (BMI) and blood pressure. Demographic characteristics, sun avoidance, sun exposure and physical activity were enquired from the participants using a self-administered questionnaire. The data was analyzed using a complex sample analysis.ResultsA total of 858 participants were recruited. Majority of them were Malays, females and had tertiary education. The overall prevalence of vitamin D deficiency (<20 ng/ml) was 67.4 %. Indian participants (80.9 %) had the highest proportion of vitamin D deficiency, followed by Malays (75.6 %), others (44.9 %) and Chinese (25.1 %). There was a significant negative association between serum 25(OH)D level with BMI (β = −0.23) and body fat percentage (β = −0.14). In the multivariate linear regression analysis, Malays, Indians and females (p < 0.001); higher BMI and larger waist circumference (p < 0.05) were significantly associated with lower serum 25(OH)D level. The full model explained 32.8 % of the variation between participants in the serum 25(OH)D level. The two most influential factors affecting serum 25(OH)D level were ethnicity and gender.ConclusionsThe prevalence of vitamin D deficiency among our participants was high. Adiposity was associated with serum 25(OH)D level. Skin pigmentation and gender based behaviours were more dominant in contributing to serum 25(OH)D level. Health education should be targeted in weight management, gender based behaviours on sun exposure, as skin pigmentation is non-modifiable.

Malignant and Pre-Malignant Manifestations of Xeroderma Pigmentosum in Ghanaians

Introduction: Xeroderma pigmentosum is an autosomal recessive disease with sun sensitivity, photophobia, early onset of freckling, and subsequent neoplastic changes on sun-exposed surfaces. There is cellular hypersensitivity to UV radiation and to certain chemicals in association with abnormal DNA repair. Patients with defective DNA nucleotide excision repair (NER) have defects in one of seven NER genes; xeroderma pigmentosum variants have normal NER and a defect in a polymerase gene. Study design: This is a case presentation of five patients with the features of xeroderma pigmentosum, aged 48, 26, 15, 14 and 8 years. The first and last patients were males. Each of the first four patients presented with areas of hyper- and hypo-pigmentation over sun exposed body surfaces. Each of them had a minimum of two cutaneous malignancies, distributed on the upper chest, face or scalp. The fifth patient had skin atrophy, with mottled hyperpigmentation and hypopigmentation but had no malignant lesions. Result: The first, second and fourth patients had their lesions surgically excised and the defects were skin grafted. The third patient was treated with radiotherapy. All the lesions were confirmed histologically as squamous cell carcinoma. No recurrence has been observed. Conclusion: Xeroderma pigmentosum in Ghanaians presents with squamous cell carcinoma involving the head, neck and upper trunk. A minimum period of exposure to UV radiation, not precisely known, is required for the development of the lesions. Education on sun avoidance and protective clothing is necessary to prevent morbidity and mortality.

NUTRITIONAL RICKETS – REVISITED

Nutritional rickets is a bone disease in early childhood resulting in bone pain, delayed motor development, and bending of the bones, caused by vitamin D deficiency and/or inadequate dietary calcium intake. The consequences of nutritional rickets include stunted growth, developmental delay, lifelong bone deformities, seizures, cardiomyopathy, and even death. Dark skin pigmentation, sun avoidance, covering the skin, prolonged breast feeding without vitamin D supplementation, insufficient calcium supply, are important risk factors for nutritional rickets. The disease is most commonly seen in children from the Middle East, Africa, and South Asia in high-income countries. European Vitamin D Association (EVIDAS) recommends screening all children from the risk groups (dark skin ethnic groups) for nutritional rickets. Nutritional rickets is entirely preventable when regular vitamin D supplementation and adequate dietary calcium intake are implemented. Vitamin D supplementation of all neonates and infants with a use of 400 IU/d during the first year of life and with at least 600 IU/d of vitamin D and 500mg/d of calcium thereafter will effectively prevent nutritional rickets. EVIDAS call on national health authorities to implement vitamin D deficiency prevention programs as addition to vaccination programs.

Melasma and Vitiligo: Novel and Experimental Therapies

Several factors are implicated in the pathogenesis of melasma, including genetic predisposition, ultraviolet radiation, thyroid disease, pregnancy, oral contraceptives, and drugs. The current approach to management incorporates a prevention strategy that includes sun avoidance, a broad spectrum sunscreen, discontinuation of oral contraceptives, avoidance of contact allergens and sensitizers, and minimization of heat and friction in the skin [1]. An intrauterine levonorgestrel-releasing device may be safer than oral contraceptives when melasma is concerned [2].

Vitamin D levels in patients with albinism compared with those in normally pigmented Black patients attending dermatology clinics in the Free State province, South Africa.

Associations between vitamin D deficiency and a broad variety of independent diseases, including several bone diseases, various types of cancer, autoimmune diseases, hypertension, and cardiovascular disease, have been suggested. It is therefore important to detect and treat vitamin D deficiency in high-risk groups. Because patients with albinism (PWA) practice a policy of strict sun avoidance, they may be at risk for low levels of vitamin D. This study was conducted in patients attending dermatology clinics in the Free State, South Africa, to determine sun avoidance behavior in the patient population and to compare 25-hydroxyvitamin D (25[OH]D) levels in PWA with those in normally pigmented Black patients attending the same clinics. Serum 25(OH)D levels were assessed in 50 PWA and 50 normally pigmented Black control subjects. Questionnaires on sun exposure avoidance behaviors were administered to all participants. The present study showed no statistically significant difference in median 25(OH)D levels between PWA and controls with normally pigmented Black skin. Rather, the study found a tendency for controls to have lower 25(OH)D levels. A total of 53% (n = 53) of all study participants (PWA and controls) had a 25(OH)D level of <20 ng/ml; thus most participants were deficient in vitamin D. Most of the PWA who participated in the study did not adhere to strict sun avoidance behavior. People with albinism attending dermatology clinics in the Free State, South Africa, need not be viewed as specifically at risk for low vitamin D levels.

Self-reported sunscreen use and urinary benzophenone-3 concentrations in the United States: NHANES 2003–2006 and 2009–2012

BackgroundSunscreens protect against skin cancer and other harmful effects of solar ultraviolet radiation (UVR). Epidemiologic and public health surveys often rely on self-reported sunscreen use to estimate sun exposure and avoidance, but questions remain about the validity of self-reports. Benzophenone-3 (BP-3), a common sunscreen ingredient, can be detected in the urine. Prior studies suggest that BP-3 concentrations increase after application of sunscreen. ObjectivesThe goal of this study was to assess the validity of self-reported frequency of sunscreen use in relation to urinary BP-3 concentrations in a representative sample of the general US population, including in sub-groups defined by age, sex and race/ethnicity. MethodsTo assess the relationship between categorical self-reported sunscreen use and creatinine-corrected urinary BP-3 concentrations, we conducted a linear regression adjusted for age, sex, race/ethnicity, six-month time period, body mass index, education, and sun avoidance behaviors. We tested for effect modification by age, sex, ethnicity and time period of measurement using multiplicative interaction terms and a F test. ResultsBP-3 was positively associated with self-reported frequency of sunscreen use across all ages, sexes, race/ethnicities, and time periods. Crude and multivariate adjusted models were all statistically significant. R-square was relatively low for all models, ranging from 0.15 to 0.43. ConclusionsUrinary BP-3 is positively associated with self-reported frequency of sunscreen use in the general US population, even in groups with overall low sunscreen use. These results suggest that self-report is a valid, although weak, way of assessing relative frequencies of sunscreen usage in a population-based study.

PORFIRIAS CUTÂNEAS EM IDADE PEDIÁTRICA

As porfirias são um conjunto de doenças metabólicas raras que resultam, maioritariamente, de um defeito geneticamente programado das enzimas da biossíntese do grupo heme. As manifestações clínicas são muito variáveis, assim como a sua gravidade e prognóstico. A fotossensibilidade é característica das porfirias cutâneas. O diagnóstico diferencial pode ser difícil devido à inespecificidade dos sintomas e à sobreposição dos achados laboratoriais. O tratamento ainda é controverso, embora a evicção da exposição solar seja essencial. Neste artigo pretende-se realizar uma revisão teórica sobre as porfirias com manifestações cutâneas em idade pediátrica, enfatizando o diagnóstico e tratamento de cada subtipo.

Validity and Reliability of Sun Protection Behavior Scale among Turkish Adolescent Population

The aim of this research was to adapt the Sun Protection Behavior Scale (SPBS) to Turkish and to perform validity and reliability analyses. The scale was administered to a total of 900 adolescents, the retest to 91 adolescents. The construct validity of the scale was evaluated using exploratory (EFA) and confirmatory factor analysis (CFA). The EFA and CFA were applied to sample groups of 449 and 451 people, respectively. The Cronbach alpha coefficients for the Turkish form of the SPBS (α=.74) and its sunscreen (α=.88) and hat use (α=.70) subscales were found to be ≥.70 while the sun avoidance subscale was calculated to be .67. The item-total score correlation between the scale and its subscales was ≥.26 and the test-retest correlations were found to be ≥.51. The CFA results verified the 8-item, 3-factor Turkish version of the SPBS. The confirmatory factor loadings for the scale were .45-.80 for sun avoidance, .72-.93 for sunscreen use, and .66-.83 for hat use. In particular, SPBS and sunscreen use (p<.001) exhibited significantly high mean scores among girls and economically better backgrounds (p=.007, p<.001, respectively). In addition, SPBS (p=.004) and hat use (p<.001) revealed that the mean scores were significantly high in younger adolescents. The SPBS was found to be valid and reliable and its psychometric characteristics acceptable. The scale can be used to measure the behavior of Turkish adolescent populations with respect to sun protection.

Actinic Keratosis

Actinic keratosis is a cutaneous lesion characterized by proliferation of atypical epidermal keratinocytes due to prolonged exposure to exogenous factors such as ultraviolet radiation. AKs are in-situ-squamous cell carcinomas (PEC) of the skin. AK typically presents as erythematous, scaly patch or papule (classic AK), occasionally as thick, adherent scale on an erythematous base. Mostly fair-skinned adults are affected. AKs typically occur in areas of frequent sun exposure (balding scalp, face, "H-region", lateral neck, décolleté, dorsum of the hand and lower extremities). Actinic Cheilitis is the term used for AKs appearing on the lips. The diagnosis of AK is based on clinical examination including inspection and palpation. The typical palpable rough surface of AK often precedes a visible lesion. Dermoscopy may provide additional information. If diagnosis is uncertain and invasion suspected, biopsy and histopathologic evaluation should be performed. The potential for progression to invasive PECs mandates therapeutic intervention. Treatment options include topical and systemic therapies. Topical therapies are classified into physical, medical and combined physical-chemical approaches and a sequential combination of treatment modalities is possible. Topical-physical cryotherapy is the treatment of choice for isolated, non-hypertrophic AK. Topical-medical treatment, e. g. 5-fluoruracil (5FU) cream or Imiquomod or Ingenolmebutat application is used for multiple, non-hypertrophic AKs. For hypertrophic AKs, a dehorning pretreatment with salicinated vaseline is recommended. Isolated hypertrophic AKs often need cryotherapy with prolonged freezing time or several consecutive applications. Sequentially combined approaches are recommended for multiple, hypertrophic AKs. Photodynamic therapy (PDT) as example for a combined physical-chemical approach is an established treatment for multiple, non-hypertrophic and hypertrophic AKs. Prevention includes avoidance of sun and application of sunscreen with high sun protection factor. Regular clinical checkups aid in early recognition of AKs.

Mediation analysis of decisional balance, sun avoidance and sunscreen use in the precontemplation and preparation stages for sun protection

Objective: Mediation analyses of sun protection were conducted testing structural equation models using longitudinal data with three waves. An effect was said to be mediated if the standardised path between processes of change, decisional balance and sun protection outcomes was significant.Design: Longitudinal models of sun protection using data from individuals in the precontemplation (N = 964) and preparation (N = 463) stages who participated of an expert system intervention.Main outcome measures: Nine processes of change for sun protection, decisional balance constructs of sun protection (pros and cons), sun avoidance behaviour and sunscreen use.Results: With the exception of two processes in the preparation stage, processes of change predicted the pros (r = .126–.614), and the pros predicted the outcomes (r = .181–.272). Three models with the cons as mediator in the preparation stage, and none in the precontemplation stage, showed a mediated relationship between processes and outcomes.Conclusion: In general, mediation analyses found both the process of change-to-pros and pros-to-behaviour paths significant for both precontemplation and preparation stages, and for both sun avoidance and sunscreen use outcomes. Findings provide support for the importance of assessing the role of underlying risk cognitions in improving sun protection adherence.

Treating and preventing tissue shift and wrinkles caused by compression

Both the literature and the authors' findings support the idea that the ageing process nor genetic disposition are reason enough to cause facial wrinkles. The tissue damage caused by ultraviolet B (UVB) exposure and an action or force, such as repeated facial expression, gravitational shift and tissue shift caused by compression, work together to create wrinkles. This suggests that stringent preventative measures beyond the recommended use of adequate sun protection factor and sun avoidance, where possible, will affect the formation of wrinkles on the face and neck. The purpose of this paper is to educate aesthetic nurses on the significance of these preventative measures. When considering facial tissue protection aesthetic nurses must not only consider UVB irradiation, but also the lesser-known damaging effects of tissue shift due to the compression exhibited in habitual, side and/or stomach sleeping positions.

Coverage of skin cancer and recreational tanning in North American magazines before and after the landmark 2006 International Agency for Research on Cancer report.

BackgroundSkin cancer is an increasingly important global public health problem. Mass media is a key source of skin cancer information. We examined how media coverage of skin cancer has changed over time as a consequence of the release of a key public health report from the International Agency for Research on Cancer (IARC) in 2006, which linked ultraviolet (UV) radiation from indoor tanning and skin cancer.MethodsA directed content analysis of skin cancer and tanning coverage in 29 popular North American magazines (2001–2012) examined reporting of skin cancer risk factors, UV behaviors, and early detection in article text (n = 761) and images (n = 1267). Chi-square and correlational analyses were used determine whether coverage changed in relation to the 2006 IARC report.ResultsThe total volume of articles about skin cancer and tanning increased modestly after the IARC report (χ2 = 4.57, df = 1, p < .05); however, key IARC report messages (e.g., avoid indoor tanning, UV as a risk factor) were no more likely to be reported after compared to before the report. There were virtually no changes in the percentage of coverage for both risk factors and early detection information over time. There were some changes in the percentage of coverage about UV behaviors after the IARC report, but these variables were not directly related to the report. Magazines were more likely to encourage sunscreen use (χ2 = 11.55, df = 1, p < .01) and less likely to promote the tanned look as attractive (χ2 = 9.72, df = 1, p < .01) after the IARC report. It also became less common for magazines to promote sun avoidance (χ2 = 6.82, df = 1, p < .01) and use of sunless tanners (χ2 = 7.46, df = 1, p < .01) after the report.ConclusionsDespite a modest increase in volume of coverage post-IARC report, key messages from the report were not taken up by the media. While there have been some improvements in magazine reporting, there is a need for more effective dissemination of public health messages about skin cancer and tanning.

Effects of tailored risk communications for skin cancer prevention and detection: the PennSCAPE randomized trial.

Prevention and early detection measures for melanoma, such as sun avoidance and skin examinations, are important, but are practiced inconsistently. In this replication of the Project SCAPE trial, we sought to determine whether tailored print materials were more effective at improving adherence than generic print materials for patients at increased risk of skin cancer. Participants were randomized to receive personalized mailed communications about their skin cancer risk and recommended sun protection, or generic mailings. Participants were Caucasian adults, at moderate or high risk for skin cancer, recruited in outpatient primary care. The main outcomes were overall sun protection behaviors and specific protective behaviors including use of sunscreen, shirt, hat, sunglasses, shade, and sun avoidance; recent sunburns; and skin self-examination and provider skin examination. One hundred ninety-two (93.2%) subjects completed the study. Six outcome variables showed significant intervention condition effects in mixed effects models: overall sun protection behavior (P = 0.025); sunscreen use (P = 0.026); use of sunglasses (P = 0.011); sunburns in the past three months (P = 0.033); recency of last skin self-exam (P = 0.017); and frequency of skin exams by health care provider (P = 0.016). Relative to generic communications, tailored risk communications resulted in improved adherence to six skin cancer protective behaviors, including a composite sun protection behavior measure, sunburns, and health care provider skin examinations. Tailored interventions can be more effective in improving patient prevention behaviors than nontailored, generic information for patients at moderate to high risk of skin cancer.

Skin Tone Dissatisfaction, Sun Exposure, and Sun Protection in Australian Adolescents.

This study aimed to assess the adoption of sun protection and sun exposure behaviors, the extent to which these behaviors group together, and the relationship between skin tone dissatisfaction and sun-related behaviors in South Australian adolescents (aged 12-17). A total of 2,875 secondary school students (1,461 male and 1,414 female) completed a questionnaire including questions about sun protection and sun exposure behaviors and skin tone dissatisfaction. Regular adoption of sun protection behaviors was low and ranged from 20% (wearing protective clothing) to 44% (sunscreen use). A principal components analysis identified four subgroups of sun-related behaviors: sun protection, appearance enhancement, sun avoidance, and sun exposure. Females had significantly higher skin tone dissatisfaction than males. Skin tone dissatisfaction was associated with decreased sun protection and avoidance and increased appearance enhancement and sun exposure in both males and females. Skin tone dissatisfaction plays an important role in Australian adolescents' sun-related behavior. Appearance-based interventions may be effective in reducing skin cancer risk through reduced sun exposure.

Decisional balance and self-efficacy for sun protection: measurement among Turkish adolescents.

Sun protection is important for skin cancer prevention, but many adolescents do not protect themselves from the sun. Instrumentation derived from the transtheoretical model (TTM) can be used to study the process of change in health behaviors like sun protection. The purpose of this study was to translate and adapt TTM-based decisional balance and self-efficacy for sun protection scales from English to Turkish and assess psychometric properties of scores when the scales are used among Turkish adolescents. The Decisional Balance Scale (DBS) and the Self-Efficacy Scale (SES) for sun protection were adapted to Turkish culture using translation and back-translation. The scales were administered to a total of 900 adolescents in two Turkish schools. Confirmatory factor analysis was used to assess dimensionality. External validity was evaluated by comparing subscale scores across reported stages of change for sun protection. Reliability estimates for scores on the DBS Pros and DBS Cons and the SES Sunscreen Use scales were high and SES Hat Use and Sun Avoidance were moderate. The two-factor correlated model for the DBS and the three-factor correlated model for the SES reported in other studies were confirmed. Means increased across the stages of change for sun protection and sunscreen use for the DBS Pros and the SES subscales as predicted by the TTM, but the pattern of DBS Cons means did not. Scores from the Turkish version of the DBS and SES for sun protection were valid, reliable, and appropriate for Turkish culture. The pattern of means for the SES and DBS Pros across the stages of change supported propositions of the TTM. Theoretical inconsistencies in the pattern of DBS Cons scores across the stages of change suggest that greater attention to conceptualization and measurement of the DBS Cons for sun protection and sunscreen use is needed.

Effects of a short messaging service-based skin cancer prevention campaign in adolescents.

Skin cancer prevention emphasizes early adoption and practice of sun protection behaviors. Adolescence represents a high-risk period for ultraviolet radiation exposure, presenting an opportunity for intervention. The ubiquity of mobile phones among teens offers an engaging medium through which to communicate prevention messages. To evaluate a skin cancer prevention intervention using short messaging service (SMS, or text messages) to impact sun-related knowledge, beliefs, and behaviors among adolescents. The intervention was conducted in middle school youth (N=113) recruited in April or October 2012. Participants were English speakers, 11-14 years old, routinely carried a mobile phone, and completed a 55-minute sun safety education program. Participants were sent three sun safety-themed SMS messages each week for 12 weeks. Skin and sun protective knowledge, beliefs, behaviors, and post-intervention program satisfaction were collected and analyzed at baseline and end of intervention (April/June 2012; October 2012/January 2013). Paired responses were tested for equality using Wilcoxon signed-rank tests. Ninety-six students (85%) completed the study. At 12 weeks, significant positive changes were reported for sun avoidance during peak ultraviolet radiation, sunscreen application, wearing hats and sunglasses, and knowledge about skin cancer risk. Participants expressed moderately high satisfaction with the program, and 15% shared messages with family or friends. A brief, SMS-based intervention affected youth skin cancer prevention behaviors and knowledge. Future research will determine whether program effects were sustained at 24 weeks and explore how sun safety parenting practices inform these effects.

Is there a golden mean for sun exposure?

Is there a golden mean for sun exposure?

Phototoxic dermatoses in pediatric BMT patients receiving voriconazole

We investigated the incidence of phototoxic skin reactions in pediatric BMT recipients treated with voriconazole. Nine out of 40 patients (22.5%), all Caucasian, developed skin lesions in sun-exposed distributions. Dermatologic findings included sunburn-like erythema, pseudo-porphyria, linear papulovesicular lesions, severe erosive cheilitis, dermatoheliosis and lentigines. Patients were treated with sun avoidance, high-potency sunscreens, and topical steroids with significant improvement in all cases. Prolonged voriconazole use requires close monitoring for chronic skin toxicities. Long-term risks including the risk of skin cancer need to be investigated.

A133: Initial Benchmarking of the Quality of Medical Care of Children and Adolescents with Lupus

Background/Purpose:Recently, 26 quality indicators (QI) categorized into 9 domains for childhood‐onset systemic lupus erythematous (cSLE) have been developed based on international consensus and scientific evidence. QI are defined as minimum standards of medical care in support of optimal disease outcomes. The current level at which these QI are followed has not been well documented. Hence, the objective of this study is to assess the current quality of medical care received by patients with cSLE at tertiary pediatric rheumatology centers.Methods:Cross‐sectional data pertaining to the QI were acquired via chart review and analyzed collectively in 290 cSLE patients followed at four tertiary pediatric rheumatology centers—two in the United States and two in Brazil.Results:Adherence to the QI differed by QI domain, ranging from 41 to 100%. The QI domain with the highest adherence 100% was Laboratory Testing at the time of diagnosis, and the lowest domain 41% was Medication Management. The recommended kidney biopsies for newly diagnosed lupus nephritis were done in only 67% of the patients and 85% of the patients received at least one of the recommended vaccinations (Table ). Cardiovascular risks factors were discussed in 86% of cases when patient was 13 years or older. Adherence to the QI differences between countries and insurance status will be presented, supporting that the set of current QI are suitable for international use. Adherence to QI by Domain Quality Indicators by Domain Results Lab testing at diagnosis &amp; screening Obtained diagnostic/confirmatory labs within first two visits 100% Obtained lab surveillance of CBC, renal, LFT every 12 months 85% General prevention Prescribed influenza and/or encapsulated organisms vaccination, unless contraindicated 85% Discussed and documented education on sun avoidance at least once in the medical record (e.g., wearing protective clothing, applying sunscreens whenever outdoors, and avoiding sunbathing) 89% Discussed transition plan to appropriate adult healthcare providers with patient age ≥14 years. 78% Lupus nephritis (LN) and hypertension management Kidney biopsy discussed/ordered/performed if developed proteinuria &gt;500 mg/day, or worsening GFR, or urinary sediment 60% Evaluated by a nephrologist in the last year of care for lupus nephritis (LN) and of hypertension 65% Evaluated by rheumatologist every 3 months in last year if a patient has known LN regardless of disease activity. 96% Received kidney biopsy when diagnosed with LN 67% Class III/IV, treated with immunosuppressive + glucocorticoids within 1 month 92% Angiotensin receptor blocker (ARB) or, Angiotensin–converting enzyme (ACE) inhibitor were prescribed if ongoing proteinuria &gt;500 mg/day or worsening GFR in last year of care. 96% Check assessments (Kidney function, urine sediment, and proteinuria) every three months. 99% Medication Management If started new meds, discussed risk vs. benefit of therapy 97% Currently prescribed any antimalarial therapy 92% Attempted to taper a dose of steroids not acceptable for chronic use. 89% Attempted to taper and unable to decrease steroid; added/increased steroid sparing agent. 41% Surveillance for medication safety done at regular intervals. 99% Bone Health Received at least one bone mineral density testing DEXA scan 72% Repeat bone mineral density testing if baseline testing outside normal limits (Z score ≤2) 62% Prescribed calcium/vitamin D if a patient is on any steroid therapy. 78% Ophthalmological surveillance Receives eye exams annually while on anti‐malarial therapy 82% Receives eye exams annually while on glucocorticoids 85% Education on cardiovascular risk factors Education on cardiovascular risk factors (smoking, hypertension, high BMI) every 1 year with patient and parent if patient is 13 years or older. 86% Discussed lifestyle modifications (smoking cessation, weight control, exercise) every 2 years with parent and patient 13 years or older. 87% Pregnancy Anti‐SSA, anti‐SB and anti‐phospholipid antibodies have been assessed during pregnancy 100% CNS health Prescribed Immunosuppressive therapy if patient has major neuropsychiatric manifestations in last year of care (Optic neuritis, coma, psychosis … etc.) 94%Conclusion:Based on this initial benchmarking effort, the medical care of patients with cSLE at tertiary pediatric rheumatology centers is very good. Systematic planning and documentation of patient education on lifestyle modifications seems warranted and is expected to improve the self‐management skills of cSLE patients. Furthermore, increased focus on bone health, eye exams annually, and adequate vaccination of cSLE patients are areas to continue to monitor.

Living with xeroderma pigmentosum: comprehensive photoprotection for highly photosensitive patients

Xeroderma pigmentosum (XP) is a rare autosomal recessive disease of deoxyribonucleic acid (DNA) repair with ultraviolet (UV) radiation sensitivity and a 10 000-fold increased risk of skin cancer. Symptoms include: freckle-like pigmentation in sun-exposed skin before age 2 years, severe burns after minimal sun exposure (50% of patients) and damage to exposed surfaces of the eyes with loss of vision and ocular cancer. About 25% of patients develop a progressive neurodegeneration. The combination of an inherited inability to repair UV-induced DNA damage and environmental exposure to UV must occur for cutaneous and ocular symptoms to develop. There is no cure for XP, but many of its manifestations may be reduced or prevented through consistent UV protection; thus XP serves as a model for sun protection of patients with marked photosenstivity. Sun protective clothing including hats, sunglasses and face shields, sun screen lotions and avoidance of environmental sources of UV are cornerstones of prevention of skin and eye damage and cancer. Although XP is a serious disease with the potential for limitation of life expectancy, XP patients can live active lives while at the same time avoiding UV.