Black patients with cutaneous lupus are associated with positive family history of cutaneous lupus and systemic lupus.

OBJECTIVES: Various genetic polymorphisms have been associated with an increased risk of cutaneous lupus erythematosus (CLE). However, it is not fully known how often positive family histories occur in patients with CLE. The aims of this study are to determine the rate of positive family history among patients with CLE and to identify risk factors associated with positive family history. METHODS: A retrospective cohort study was conducted among 338 patients with CLE seen in outpatient dermatology clinics in a tertiary referral centre in Dallas, Texas. The primary outcome was positive family history of CLE and/or SLE, as defined by the presence of self-reported CLE and/or SLE in first-degree or more distant relatives of a patient. Univariate analyses were performed to identify risk factors associated with positive family history of CLE and/or SLE in patients with CLE. Multivariable logistic regression analyses were performed to determine significant predictors of positive family history of CLE and/or SLE. RESULTS: 34% (n=114) of patients reported positive family history of CLE and/or SLE. 7% (n=23) of patients with CLE had relatives with CLE, with 5% (n=18) having a first-degree relative with CLE. 30% (n=102) of patients with CLE had relatives with SLE, and 15% (n=52) had a first-degree relative with SLE. Black patients were more likely to have positive family history of CLE and/or SLE (OR 2.13, 95% CI 1.23 to 3.69, p=0.007). CONCLUSIONS: More patients with CLE had positive family history of SLE than CLE. Black patients with CLE were more likely to have a relative with CLE and/or SLE. Providers can use this information to counsel patients with CLE on the risk of other family members having CLE and/or SLE. These data may help identify potentially new genetic polymorphisms associated with positive family history.

Robust measurement of clinical improvement in patients with cutaneous lupus erythematosus.

Objective: The severity and disease course of cutaneous lupus erythematosus (CLE) are highly variable. Consequently, outcome measures for CLE clinical improvement are heterogeneous, complicating treatment decisions and therapeutic development. This study characterises CLE outcome measures and identifies the influence of clinical improvement thresholds on strengths of associations with patient demographic and clinical factors. Methods: In this pilot cohort study, multivariable models identified factors associated with CLE activity and skin damage improvement, defined as relative decreases in Cutaneous Lupus Activity and Severity Index (CLASI) activity (CLASI-A) and damage (CLASI-D) scores, over ranges of response thresholds. Results: 66 patients with 119 visit-pairs were included in the CLASI-A analysis. 74 patients with 177 visit-pairs were included in the CLASI-D analysis. Factors associated with CLE activity and damage improvement depended on the response threshold. Some associations were stronger at more stringent thresholds, including subacute CLE predominance with increased likelihood of CLASI-A improvement (R2=0.73; 50% reduction: OR 1.724 (95% CI 0.537 to 5.536); 75%: 5.67 (95% CI 1.56 to 20.5)) and African-American race with decreased likelihood of CLASI-D improvement (R2=0.80; 20%: 0.40 (95% CI 0.17 to 0.93); 40%: 0.25 (95% CI 0.08 to 0.82)). Other associations were stable across multiple thresholds, including older age of CLE development with increased likelihood of CLASI-A improvement (R2=0.25; 50%: 1.05 (95% CI 1.01 to 1.09]; 75%: 1.05 (95% CI 1.00 to 1.10)) and higher initial disease activity with decreased likelihood of CLASI-D improvement (R2=0.55; 20%: 0.91 (95% CI 0.84 to 0.98); 40%: 0.88 (95% CI 0.79 to 0.97)). Conclusions: Examining a range of CLASI threshold outcomes can comprehensively characterise changes in disease course in patients with CLE. Insufficiently stringent thresholds may fail to distinguish meaningful clinical change from natural fluctuation in disease activity.

Association of African-American ethnicity and smoking status with total and individual damage index in systemic lupus erythematosus.

INTRODUCTION: Smoking has been associated with increased incidence, severity of cutaneous lupus, and lupus activity. We looked at the association of both smoking and ethnicity with the individual damage items from the SLICC/ACR Damage Index. METHODS: Poisson regression was used to model the total SLICC/ACR Damage Index score against ever smoking. Cox regression was used to assess the relationship between time to individual damage items and ever smoking. Furthermore, we compared SLICC/ACR Damage Index items among African-American and Caucasian ever smokers. RESULTS: The study included 2629 patients, 52.6% Caucasian and 39.3% African-American. The prevalence of ever smokers was 35.8%. There was no significant difference in total SLICC/ACR Damage Index score between ever smokers and never smokers after adjustment for ethnicity, gender, age at diagnosis, and years of education. Ever smokers had more atherosclerotic cardiovascular damage and skin damage compared to non-smokers. Caucasian SLE patients who ever smoked were more likely to have muscle atrophy and atherosclerosis compared to Caucasian non-smokers. African-American patients who ever smoked were more likely to have skin damage compared to African-American non-smokers. African-Americans who smoked were more likely to have many more damage items (cataract, renal damage, pulmonary hypertension, cardiomyopathy, deforming or erosive arthritis, avascular necrosis, skin damage, and diabetes) compared to Caucasians who smoked. CONCLUSION: Our analysis proved the major effect of smoking on cardiovascular and cutaneous damage. Surprisingly, cardiovascular damage items had higher hazard ratios in Caucasian smokers than non-smokers while skin damage items hazard ratios were higher in African-American smokers compared to non-smokers.Key Points• This study is the largest cohort study to date evaluating the effect of smoking on the cumulative SLICC/ACR Damage Index and its individual damage items.• It is the only study that examined the effect of smoking on individual items of the SLICC/ACR Damage Index in terms of Caucasians vs. African-American ethnicity.• Our analysis proved the major effect of smoking on cardiovascular and cutaneous damage. Compared to non-smokers, Caucasian smokers had higher risk of cardiovascular damage while African-American smokers had more skin damage.• African-Americans who smoked were more likely to have many more damage items (cataract, renal damage, pulmonary hypertension, cardiomyopathy, deforming or erosive arthritis, avascular necrosis, skin damage, and diabetes) compared to Caucasians who smoked.

Spectrum of cutaneous lupus erythematosus in South Africans with systemic lupus erythematosus.

BACKGROUND: Cutaneous involvement is very common in systemic lupus erythematosus. We describe the prevalence and spectrum of lupus-specific (cutaneous lupus erythematosus) and non-specific cutaneous features amongst mostly black South Africans with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective record review of 298 South Africans (262 blacks and 36 non-blacks) with systemic lupus erythematosus was carried out. Cutaneous features were classified according to the Gilliam and Sontheimer classification of cutaneous lupus. RESULTS: Most (81.5%) patients were black African females. The mean (SD) age at diagnosis and follow-up duration were 35.0 (11.8) and 8.0 (5.9) years, respectively. Cutaneous lupus erythematosus was seen in 76.1% of patients, mainly chronic cutaneous lupus erythematosus with the discoid lupus erythematosus subtype seen in 52.1% of patients. Acute cutaneous lupus erythematosus was seen in 30.2% of patients and was more common in non-blacks than blacks (odds ratio = 3.8 (1.9-7.9)); localized acute cutaneous lupus erythematosus was more common than generalized acute cutaneous lupus erythematosus (odds ratio = 2.6 (1.6-4.4)). Non-specific cutaneous features occurred in 77.2%, with oral/nasal ulcers and Raynaud's phenomenon each occurring in approximately 40% of patients. Diffuse melanonychia at initial diagnosis was present in 37.4% of patients and was more common in blacks than non-blacks (odds ratio = 3.1 (1.3-7.3)). Acute cutaneous lupus erythematosus was associated with renal disease (odds ratio = 2.8 (1.6-4.7)) and chronic cutaneous lupus erythematosus with arthritis (odds ratio = 2.02 (1.24-3.29)). Diffuse melanonychia was associated with less renal disease and anti-dsDNA antibody positivity (odds ratio = 0.4 (0.3-0.7) and 0.4 (0.2-0.6), respectively) and significantly lower lupus severity index scores (mean (SD) = 5.99 (1.11) vs 6.56 (1.36) in patients with no melanonychia, p < 0.05)). CONCLUSION: In this study of South Africans with systemic lupus erythematosus, the skin was the most commonly affected organ. In general, cutaneous lupus erythematosus was associated with less severe systemic disease. Acute cutaneous lupus erythematosus was less common in blacks, whereas discoid lupus erythematosus was more common than reported in Caucasians. Diffuse melanonychia was a distinctive finding and was associated with milder systemic disease.

Not Just Skin Deep: Systemic Disease Involvement in Patients With Cutaneous Lupus.

Cutaneous lupus erythematosus, specifically discoid lupus erythematosus, disproportionately affects those with skin of color and may result in greater dyspigmentation and scarring in darker skin types. In this article, we review investigations relevant to cutaneous lupus patients with skin of color at University of Texas Southwestern Medical Center, associations and risk of progression to systemic lupus, and recommendations for monitoring for systemic disease spread. Between 5% and 25% of patients with cutaneous lupus can develop systemic lupus. If they progress to systemic disease, patients often develop mild systemic disease with primarily mucocutaneous and musculoskeletal manifestations. Patients with cutaneous lupus should be followed up closely to monitor for systemic disease involvement. The University of Texas Southwestern Cutaneous Lupus Erythematosus Registry, of which almost two thirds of participants are those with skin of color, is a part of an ongoing effort to better understand the pathophysiologic mechanisms of CLE and to identify prognostic indicators of risk of progression to systemic lupus.

Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

Vitamin D levels in 87 Asian patients with cutaneous lupus erythematosus: a case-control study.

BACKGROUND: Cutaneous lupus erythematosus (CLE) is an autoimmune disease, often exacerbated by sun exposure. Patients are encouraged to avoid sun exposure, therefore predisposing them to vitamin D deficiency. AIM: To investigate the prevalence of and risk factors for vitamin D deficiency in patients with CLE. METHODS: Total serum 25-hydroxy vitamin D (25(OH)D) was measured in 87 consecutive patients with CLE and in 79 controls. Clinical characteristics, disease severity, medications used and lifestyle factors were analysed and compared to determine risk factors for inadequate (25(OH)D), defined as a serum (25(OH)D) level of < 20 µg/L. RESULTS: We found that 51% (n = 44) of the patients with CLE had 25(OH)D levels of < 20 µg/L compared with 73% (n = 58) of the controls (P < 0.01). No significant differences in (25(OH)D) levels were found between cases and controls with regard to age, sex, ethnicity, smoking, sun exposure, sunblock use or vitamin D supplementation. Treatment with antimalarials showed a statistically significant association with lower vitamin D levels. CONCLUSION: Low levels of vitamin D were found in both patients with CLE and controls. Despite being on vitamin D supplementation and living in an equatorial location, our Asian patients with CLE still had low levels of vitamin D. It is therefore important to ensure adequate vitamin D supplementation in patients with CLE, especially for those who are on antimalarial therapy.

Ethnic differences in the epidemiology of cutaneous lupus erythematosus in New Zealand.

Background The prevalence and variation by ethnicity of cutaneous lupus in New Zealand is not known. Therefore, a cross-sectional study to determine the prevalence and variation by ethnicity of cutaneous lupus in the ethnically diverse community of South Auckland, New Zealand, was undertaken. Methods Multiple sources were examined to determine the prevalence of acute cutaneous lupus erythematosus, subacute cutaneous erythematosus and discoid lupus erythematosus. Ethnicities examined were European, Maori/Pacific and Indian/Asian. Capture-recapture was used to determine the overall population prevalence of cutaneous lupus. Results A total of 145 cases of cutaneous lupus were identified. There were 22 men and 123 women, with an average age (standard deviation), respectively, of 46.4 (±21.5) and 43.1 (±14.8) years. There were 53 cases of acute cutaneous lupus erythematosus, 19 cases of subacute cutaneous erythematosus and 66 cases of discoid lupus erythematosus. The age and sex adjusted relative risk (95% confidence interval; CI) of Maori/Pacific compared to the European population was 2.47 (95% CI 1.67-3.67) for all types of cutaneous lupus, 1.60 (95% CI 0.84-3.18) for acute cutaneous lupus erythematosus, 0.09 (95% CI 0.01-1.1) for subacute cutaneous erythematosus and 5.96 (95% CI 3.06-11.6) for discoid lupus erythematosus. The overall prevalence of cutaneous lupus was 30.1 (95% CI 25.5-35.4) per 100,000. However, capture-recapture estimated the unadjusted prevalence of cutaneous lupus to be 86.0 (95% CI 78.1-94.7) per 100,000. Conclusion Maori and Pacific people in Auckland, New Zealand, have a greater relative risk of all types of cutaneous lupus compared to the European population and a particularly high risk of discoid lupus erythematosus.

Epidemiology of systemic lupus erythematosus and cutaneous lupus erythematosus in a predominantly white population in the United States.

OBJECTIVE: Epidemiologic studies comparing the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE) are few. Olmsted County, Minnesota provides a unique setting for such a study owing to resources of the Rochester Epidemiology Project. We sought to describe and compare the incidence and prevalence of SLE and CLE from 1993-2005. METHODS: SLE cases were identified from review of medical records and fulfilled the 1982 American College of Rheumatology classification criteria. CLE cases included patients with classic discoid lupus erythematosus, subacute CLE, lupus panniculitis, and bullous lupus erythematosus. Age- and sex-adjusted incidence and prevalence were standardized to the 2000 US white population. RESULTS: The age- and sex-adjusted incidence of SLE (2.9 per 100,000; 95% confidence interval [95% CI] 2.0-3.7) was similar to that of CLE (4.2 per 100,000; 95% CI 3.1-5.2, P = 0.10). However, the incidence of CLE was 3 times higher than SLE in men (2.4 versus 0.8 per 100,000; P = 0.009). The age- and sex-adjusted prevalence of CLE on January 1, 2006 was higher than that of SLE (70.4 versus 30.5 per 100,000; P < 0.001). The prevalences of CLE and SLE in women were similar, but the prevalence of CLE was higher in men than in women (56.9 versus 1.6 per 100,000; P < 0.001). The incidence of CLE rose steadily with age and peaked at 60-69 years. CONCLUSION: The incidences of CLE and SLE are similar, but CLE is more common than SLE in men and in older adults. These findings may reflect differences in genetic or environmental etiology of CLE.

The impact of skin damage due to cutaneous lupus on quality of life.

BACKGROUND: Patients with more severe cutaneous lupus erythematosus (CLE) have a poorer quality of life (QoL). Racial and ethnic disparities have been reported in disease activity and outcomes in systemic lupus erythematosus, but similar information is not available for CLE. OBJECTIVES: To evaluate the impact of lupus-related skin damage on skin-specific QoL, and to analyse differences stratified by ethnic background. METHODS: Data collected included sex, race, diagnosis and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Skindex-29 scores. These parameters were analysed at the initial and last visits. CLASI damage scores (dyspigmentation and scarring) and activity scores were collected, grouped by ethnicity, and correlated with Skindex-29. Overall, 223 patients were analysed at baseline, with 141 completing more than one study visit. RESULTS: The majority of patients were white (63·7%), followed by African American (29·1%) and Asian American (4·0%). African American patients accounted for a disproportionate percentage of both localized (50%) and generalized discoid lupus erythematosus (DLE) (49%). Median CLASI damage scores differed significantly between the African American, white and Asian American patients, at both the first (8·5, 4·0, 7·0, respectively; P < 0·0001) and last visit (10·0, 6·0, 8·5, respectively; P < 0·01). CLASI damage scores in African Americans correlated with CLASI activity scores (Spearman r = 0·45, P = 0·0003). CONCLUSIONS: There was no significant correlation between CLASI damage scores and Skindex domains overall. Individually, dyspigmentation and scarring also did not have a significant effect on QoL. Ethnic differences in patients with CLE were found: African American patients exhibited a high rate of DLE and experienced damage early in their disease course, frequently in conjunction with disease activity.

Multisystemic diseases and ethnicity: a focus on lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease.

We present an overview of the association between ethnicity and the clinical and epidemiological aspects of four multisystemic diseases: lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease. In particular, we highlight observed ethnic differences in cutaneous manifestations of these diseases. This article should help guide clinical management, as well as serve to highlight future areas for research.

Hair and scalp disorders in women of African descent: an overview.

We present an overview of hair and scalp disorders in women of African descent, discussing the biological features of afro-textured hair, as well as hair-grooming practices in this cohort and their association with specific hair and scalp disorders. A practical approach to diagnosing and managing common hair and scalp disorders in this cohort is also presented.

Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre.

BACKGROUND: Paediatric cutaneous lupus erythematosus (CLE) is uncommon and inadequately described in the literature. Similar to adults, children with CLE develop LE-specific and/or LE-nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult CLE have not been sufficiently described. OBJECTIVES: To detail the demographic and clinical features of paediatric CLE and compare these findings with those reported in the adult literature. METHODS: A retrospective chart review was performed of 53 children seen in a paediatric dermatology clinic with cutaneous manifestations of LE. RESULTS: Patients presented with all five major subtypes of CLE, with some notable differences from adult CLE and previously published reports of paediatric CLE. Progression from discoid LE to systemic LE (SLE) did not occur in our cohort. Patients with subacute CLE were more likely than adults to have lesions below the waist as well as concomitant SLE. Sex distribution for CLE in our study was equal prior to puberty and female predominant in post-pubertal patients. CONCLUSIONS: Children with CLE have variable clinical presentations and progression to SLE that may be different from adult disease. Specifically, children with acute and subacute CLE may be more likely than adults to have systemic disease; therefore, patients with these subtypes should be monitored closely for evidence of SLE. Study limitations included small patient numbers that may limit the ability to generalize these data and relatively short follow-up intervals.

Pure cutaneous lupus erythematosus in a population of African descent in French Guiana: a retrospective population-based description.

The objective of this study was to examine the characteristics of cutaneous lupus erythematosus, excluding systemic lupus erythematosus (SLE), in patients of African descent. Indeed, since the description of subacute cutaneous lupus erythematosus (SCLE), which had been included in chronic cutaneous lupus erythematosus (CCLE), there has been no description of the disease in black patients. In 2000, we performed a retrospective epidemiological study by querying multiple sources to identify all patients with lupus in French Guiana--a part of France in South America having western living conditions, free healthcare and 157,000 inhabitants, most of whom are of African origin. We found 45 patients with pure cutaneous lupus, which included CCLE (mostly discoid), SCLE and bullous lupus. The disease characteristics of these patients exhibited few differences compared with those of the Caucasian patients cited in the literature. However, the age of onset for our patients of African descent was younger than that of Caucasian patients. In contrast to the race-related differences reported for SLE, we found no major differences in terms of demographic, clinical and biological presentation between this cohort of pure cutaneous lupus erythematosus patients of African origin and Caucasian patients with similar forms of lupus.

25-Hydroxyvitamin D levels in African-American and Caucasian/Hispanic subjects with cutaneous lupus erythematosus.

BACKGROUND: Because exposure to ultraviolet radiation accounts for a significant portion of endogenous vitamin D production, subjects with cutaneous lupus (CLE) who practise sun-protective measures are at risk for vitamin D insufficiency. Previous studies have shown light-skinned subjects with CLE to have lower serum 25-hydroxy (25-OH) vitamin D levels than normal controls. OBJECTIVES: To assess the status of vitamin D insufficiency in dark-skinned individuals with CLE. METHODS: We performed a cross-sectional study comparing serum 25-OH vitamin D levels in 25 African-American (AA) subjects with CLE and 26 normal AA subjects matched by age, sex and season in Dallas, Texas. A questionnaire on demographics, medical history and lifestyle habits was administered to determine factors potentially affecting vitamin D levels. Findings were contrasted to a similar comparison in 26 Caucasian and Hispanic (C/H) subjects with CLE and 24 normal C/H subjects matched by age, sex and season. RESULTS: We found similar mean±SD 25-OH vitamin D levels in AA subjects with CLE (52·0±18·5nmolL(-1) ) and normal AA subjects (54·8±21·2 nmolL(-1) ) (P=0·62). Almost half of AA subjects in both groups were vitamin D insufficient. A larger difference in 25-OH vitamin D levels was found between C/H subjects with CLE (59·4±21·0nmolL(-1) ) and normal C/H subjects (70·5±27·4nmolL(-1) ) (P=0·12). Two-way anova demonstrated that skin colour (AA vs. C/H) had a significant effect on 25-OH vitamin D levels (P=0·008), although CLE status (CLE vs. normal) did not (P=0·13). CONCLUSIONS: Providers are encouraged to address vitamin D insufficiency concerns in all dark-skinned individuals. Future studies should stratify subjects by skin colour in determining differences between subjects with CLE and normal controls.

Emerging chronic illness: women and lupus in Ecuador.

This article reports on an ethnographic investigation of the experiences of urban Ecuadorian women suffering from the chronic illness, lupus. Chronic illness is "emerging" in Ecuador, and cultural models and the health care delivery system are struggling to adapt to the increasing burdens brought by life-long illness. Based on extensive qualitative interviewing of lupus patients and doctors and participant observation, we identify three areas of concern including a weak health infrastructure and unequal access to care, gender models that increase the emotional burdens, and cultural understandings about illness and morality that add to social stress.

Aspectos prácticos de la quinacrina como tratamiento del lupus eritematoso cutáneo: serie de casos / Quinacrine in the treatment of cutaneous lupus erythematosus: practical aspects and a case series

Los antimaláricos de primera elección en el tratamiento del lupus cutáneo son la hidroxicloroquina (HDQ) y la cloroquina (CQ). La quinacrina (Qn) se emplea poco, fundamentalmente por la ausencia de información sobre su utilización y por no estar comercializada en España. Es eficaz en combinación con otros antimaláricos y en monoterapia. La Qn parece carecer de toxicidad retiniana y ésta es una de sus ventajas sobre la CQ y la HDQ.Se utiliza cuando existen alteraciones oculares previas al tratamiento que contraindican el uso de otros antimaláricos (al valorar la opción con mejor relación riesgo-beneficio), y en tratamiento combinado con otros antimaláricos en pacientes resistentes o parcialmente respondedores a CQ o HDQ. Presentamos una serie de 8 casos de pacientes con lupus cutáneo que han recibido tratamiento con Qn en monoterapia o combinada con otros antimaláricos, se obtuvo resolución de las lesiones en 5 pacientes y mejoría de éstas en 3 pacientes. En uno de los pacientes fue necesario suspender el tratamiento por la aparición de un brote de psoriasis (AU)

Hydroxychloroquine and chloroquine are antimalarials used as first-line treatment of cutaneous lupus. Quinacrine is not often employed by Spanish physicians due to a lack of information about its use and the fact that it is not marketed in Spain. It is effective in monotherapy or in combination therapy with other antimalarials. One of the advantages of quinacrine over chloroquine and hydroxychloroquine is that it does not appear to cause retinal toxicity. Quinacrine is used as second-line therapy in patients with pre-existing eye problems that contraindicate treatment with chloroquine or hydroxychloroquine (after evaluation of which drug has the better risk-benefit relationship), and in combination therapy with other antimalarials in patients with resistance or only a partial response to chloroquine or hydroxychloroquine. We report 8 cases of patients with cutaneous lupus who received treatment with quinacrine in monotherapy or in combination with others antimalarials. Lesions resolved in 5 patients and improved in 3. Therapy had to be withdrawn in 1 patient due to an exacerbation of his psoriasis (AU)

Race differences in immunogenetic features and photosensitivity of cutaneous lupus erythematosus from the aspect of Japanese studies.

Skin lesions of collagen diseases are influenced by environmental triggers, such as UV light, and are variable in cutaneous lupus erythematosus (LE), such as systemic LE (SLE), chronic discoid LE (CDLE), subacute cutaneous LE (SCLE), and LE tumidus (LET). Although there are a few conflicting reports on photosensitivity in collagen diseases, many Japanese dermatologists feel there are photosensitivity differences in LE between Asians and Caucasians with SCLE and LET. To address this issue, we have carried out genetic studies of Japanese SLE and CDLE patients and reviewed the race differences in photosensitivity of cutaneous LE from Japanese studies. Human leukocyte antigen (HLA) studies in Japanese patients revealed that HLA-DRB1*1501 association was with CDLE and SLE. The association between HLA-Cw6 and CDLE was first reported in a Japanese population, and a HLA-A33-B44-DRB1*1302 haplotype showed a positive association in CDLE. However, these results are not compatible with those from Caucasian subjects. There are no significant associations among HLA studies, photosensitivity, and anti-Ro/SS-A antibodies in Japanese CLE patients. Photosensitivity will be a key factor to dissolve multifactorial complexes of LE etiopathogenesis. An axis of photosensitivity, anti-Ro/SS-A antibodies, and apoptosis via tumor necrosis factor-alpha is the best marker to verify the contribution of genetics in CLE patients. The incidence and degree of photosensitivity of SCLE and LET are much lower in Japanese than in Caucasians. This discrepancy may lead to investigations of CLE pathogenesis through global collaborations.

Ethnic differences in immunogenetic features and photosensitivity of cutaneous lupus erythematosus.

Genetic differences are involved in the development of lupus erythematosus (LE). Skin lesions are influenced by environmental triggers such as ultraviolet light, temperature, and chemical stresses, and the patterns of skin lesion are variable in cutaneous LE such as systemic LE (SLE), chronic discoid LE (CDLE), subacute cutaneous LE (SCLE), and LE tumidus (LET). Although there are a few conflicting reports, many Japanese dermatologists feel there are photosensitivity differences in lupus erythematosus between Asian and Caucasian subjects with SCLE and LET. HLA studies in Japanese subjects revealed that HLA-DRB1*1501 association was with both CDLE and SLE. The association between HLA-Cw6 and CDLE was first reported in Japanese population, and a HLA-A33-B44-DRB1*1302 haplotype showed a positive association in CDLE. However, these results are not compatible with those from Caucasian subjects. There are no significant associations among HLA studies, photosensitivity, and anti-Ro/SS-A antibodies in Japanese CLE patients. Photosensitivity will be a key factor to dissolve multi-factorial complexes of LE etiopathogenesis. Our present understanding is that an axis of photosensitivity, anti-Ro/SS-A antibodies and apoptosis via TNF are the best (markers) to verify the contribution of genetics in SCLE, LET, and other CLEs. The incidence and photosensitivity of SCLE and LET are much lower in Japanese than in Caucasian subjects. However, this discrepancy may open the window for investigating CLE pathogenesis through global collaborations. For this purpose and goal, a new and more conventional method should be developed for the examination of so-called photosensitivity.