Evaluation of health-related quality of life (QOL) has become increasingly important in recent years.1–3 In dermatology, various instruments have also been developed to evaluate the impact of skin disease on QOL. Measurement of QOL is useful in everyday clinical practice and research and for administrative purposes, political purposes (patient associations), and economic purposes (pharmaceutical companies, product evaluation).1,4 QOL also plays an important role in adherence, since both QOL and empathy on the part of the clinician contribute to patient satisfaction and, thus, adherence to treatment.5 In the case of lupus erythematosus, most publications focus on systemic lupus erythematosus (SLE), with less attention being paid to cutaneous lupus erythematosus (CLE). Both types have had a considerable impact on QOL.6

Our primary objective was to evaluate the impact of CLE on QOL. Our secondary objective was to determine the effect on QOL of other factors, such as disease severity, time since diagnosis, body surface area affected, systemic medication, and the presence of SLE criteria.

We performed a cross-sectional study between February and June 2012. The study population comprised patients diagnosed with CLE at the Department of Dermatology, Complejo Hospitalario de Pontevedra, Pontevedra, Spain (now known as Xestión Integrada Pontevedra-Salnés). We included patients aged more than 18 years with chronic CLE (CCLE), subacute CLE (SCLE), and acute CLE (ACLE). Classification was based on clinical criteria (morphology, location, progress of the lesions, and presence or absence of scarring). In addition, all the study patients presented histopathologic abnormalities consistent with CLE. Patients with lupus tumidus were excluded because of the clinical characteristics, unpredictable course, and problematic classification of this variant. We also excluded patients with a provisional or uncertain diagnosis and patients with overlap syndrome. Patients were informed about the aims and conduct of the study by telephone. If they verbally agreed to participate, an appointment was made at the dermatology clinic. All the participants signed an informed consent document. The Clinical Research Ethics Committee of Galicia, Spain approved the study. We recorded the following information: age, sex, date of diagnosis, CLE subtype, presence of SLE criteria, body surface area affected, previous and current treatment, score on the QOL evaluation scale, and disease severity. QOL was evaluated using the validated Spanish-language version of the Dermatology Life Quality Index (DLQI),7 a questionnaire comprising 10 questions on cutaneous involvement in terms of symptoms and feelings, activities of daily living, leisure and free time activities, work or studies, personal relationships, and treatment. The maximum score is 30 points. The scoring system was as follows: 0-1, no effect; 2-5, slight effect; 6-10, moderate effect; 11-20, large effect; 21-30, extremely large effect. The tool used to evaluate disease severity was the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). This scale takes into account 2 different scores, one referring to disease activity and the other to damage/scarring. The maximum score is 70 points. Scoring was as follows: 0-9 points, mild disease; 10-20 points, moderate disease; 21-70 points, severe disease.8,9 Both the DLQI and the CLASI were used with the authors’ permission.

Data were recorded on an Excel spreadsheet and analyzed using Stata 10.1.

CLE had a slight or no effect on the QOL of 50% of the patients (18/36). In the remaining 50%—an appreciable percentage—the impact on QOL of CLE was moderate (19.4%, 7/36), large (25%, 9/36), or extremely large (5.5%, 2/36). Taking into account only patients with CCLE, 47.1% (16/34) had a moderate to extremely large effect on QOL. The effect was greater in patients with SCLE and ACLE, and 75% (3/4) and 100% (3/3), respectively, presented a moderate to extremely large effect. The DLQI scores are shown in Table 1. The distribution of the DLQI score by disease subtype is shown in Table 2. The distribution of the DLQI scores in the total study population is shown in Figure 1. The mean (SD) DLQI value was 7.6 (SD 6.94) for the whole study population and 7.4 (SD 7.1) for patients with CCLE.

Figure 1.

Distribution of DLQI and CLASI scores in patients with cutaneous lupus erythematosus. DLQI indicates Dermatology Life Quality Index; CLASI, Cutaneous Lupus Erythematosus Disease Area and Severity Index.

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Disease severity was as follows: mild, 69.4% (25/36); moderate, 19.4% (7/36); and severe, 11.1% (4/36). The CLASI scores (activity scale, damage scale, total) are shown in Table 1. The distribution of the CLASI score in the whole study population is shown in Figure 1. The median (IQR) score was 4 (2-6) for CLASI activity, 3 (1-6) for CLASI damage, and 7 (4-11) for CLASI total.

Given the small sample size, the association between DLQI score and other factors was only analyzed in the CCLE group. No significant differences were found between the DLQI score and involvement of the face or scalp (Wilcoxon rank sum test: P=.57 and P=.97), DLQI and number of previous or current systemic treatments (nonparametric trend test: P=.64), DLQI and the presence of SLE criteria (Wilcoxon rank sum test: P=.32), or DLQI and time since diagnosis (linear regression: P=.97). The correlation between the DLQI and the CLASI scores was very low (Pearson r=0.18 for the CLASI activity score, 0.02 for the CLASI damage score, and 0.13 for the CLASI total score). When disease severity was taken into account, the mean DLQI scores were 7.1 (moderate effect on QOL) in patients with mild disease, 5.7 (slight effect on QOL) in those with moderate disease, and 14 (large effect on QOL) in those with severe disease.

A wide variety of questionnaires can be used to measure QOL. Some are valid for several diseases, whereas others are specific to a particular system or disease.1 The Short Form 36 health questionnaire (SF-36), the Sickness Impact Profile, the Nottingham Health Profile, and the General Health Questionnaire are examples of generic questionnaires,3 which enable comparisons to be made between different diseases affecting different systems.1 Dermatology-specific questionnaires include the DLQI, the Skindex, the Dermatology Quality of Life Scales, the Dermatology-Specific Quality of Life instrument, and the Qualita di Vita Italiana in Dermatologia (Italian Quality of Life in Dermatology) questionnaire.4

Despite the availability of more up-to-date, change-sensitive questionnaires such as the Skindex-29, which are useful, for example in clinical trials,4,10–12 the DLQI was the first to be developed and the most widely used in dermatology, as it is specifically designed for skin diseases and frequently used to evaluate the impact of a disease before systemic treatment is started. It is easy to complete, efficient, and reproducible, and a Spanish-language version has been validated.7,13 Consequently, the DLQI was the questionnaire we selected for our study.

We selected the CLASI to measure the severity of CLE, since it is currently the only formally validated questionnaire for this purpose.14 Despite its limitations (noninclusion of lesional induration, subjectivity in evaluation of alopecia, evaluation of the most severe lesion instead of providing a global evaluation of all the lesions, and inclusion of all forms of CLE),15 the CLASI activity score and the CLASI damage score show, respectively, an excellent correlation and a moderate correlation with general health status, as evaluated by the physician and the patient.8 The CLASI score also correlated with the improvement in general health status and has proven useful for measuring response to therapy.8,16 Independent evaluation of activity and damage is very useful, since the CLE damage score usually remains stable and generally does not improve with drugs.8

Owing to its chronic course and potential for causing disfiguring lesions, CLE (with CCLE as the most prevalent variant) can have a considerable effect on QOL, and involvement of photoexposed areas can diminish the patient's self-confidence and self-esteem.2 The presence of scarring and hypopigmentation, which are invariably persistent, means that improvement of the disease is not always correlated with an improvement in QOL.6 However, in our study, QOL in the CCLE group was no worse than in the other groups, although the low number of patients with SCLE and ACLE would only enable very large differences to be detected.

Most studies on QOL in lupus erythematosus focus on SLE. In patients with this disorder, QOL associated with body image has also been evaluated using the Body Image Quality of Life Inventory, whose score was significantly lower (worse QOL) in patients with SLE than in controls.17

Klein et al.9 evaluated the QOL (Skindex-29 and SF-36) and disease severity (CLASI) of 157 patients with CLE at a reference center in Pennsylvania in the United States. Since the SF-36 is not specific for dermatologic complaints (see above), it enables the results for patients with lupus erythematosus and those with other diseases to be compared. Patients with CLE were observed to have worse QOL than those with acne, nonmelanoma skin cancer, and alopecia. With respect to the psychological subdomain of QOL, patients with CLE had similar or worse scores than patients with hypertension, type 2 diabetes mellitus, myocardial infarction, and congestive heart failure. The factors related to worse QOL were female sex, generalized or severe disease, distribution of lesions (scarring alopecia), and younger age. Vasquez et al.18 performed a similar study with 91 patients from Texas in the United States in order to compare their results with those of the population from Pennsylvania in the study by Klein et al. and investigate differences in the QOL of patients from different geographic areas. They observed that the Skindex and SF-36 scores were very similar in both study populations, although significant differences were observed for the subdomains of physical functioning, lupus-specific subdomain, and general health status, all of which scored worse in the patients from Texas. The factors associated with worse QOL in this group were female sex, lower income, high educational level, presence of SLE, and greater disease activity.

The DLQI has also been used to evaluate QOL in patients with lupus erythematosus. Ferraz et al.19 evaluated QOL in 71 patients with lupus erythematosus and skin lesions (45 patients with CCLE and 26 patients with SLE) and found the mean DLQI score to be 6.5. Martins et al.20 performed a comparative study of QOL in patients with CCLE (only discoid type) and patients with SLE, including 26 patients in the first group and 38 in the second. Lower values were found in the DLQI in patients with discoid CCLE (mean value of 6.5 [moderate impact]) than in patients with SLE (mean value 12.4 [large impact]). Our study showed similar results with respect to the mean DLQI values, which fell within the range for moderate impact on QOL.

We found no association between QOL and disease severity, time since diagnosis, involvement of more visible areas, number or family of previous or current systemic drugs, or presence of SLE criteria. The absence of significant differences could be explained by the small sample size. QOL can also be influenced by changes in the location of the lesions (visible or nonvisible areas), in such a way that disease activity can increase owing to the appearance of lesions in covered areas, thus compromising QOL less than if the lesions were on the face or scalp. The need for stringent photoprotective measures and the adverse effects of systemic treatment can affect QOL despite the absence of skin lesions. Furthermore, scarring skin lesions do not improve QOL, even if disease activity has ceased. Consequently, the correlation between DLQI and CLASI is weak.

QOL is considerably compromised in patients with CLE, and outcome is determined by several factors. Given the visibility of the lesions, QOL is affected even if disease activity is low. The chronic course of CLE, the disfiguring scars it produces, and the need for stringent photoprotective measures are responsible for the lasting impact on QOL. Therefore, this field should not be overlooked in the global evaluation of patients with CLE. It seems reasonable to pay greater attention to the QOL of patients with CLE. In addition to drugs and sunscreen, these patients should be offered cosmetic treatment to improve the sequelae of the disease.

The authors declare that they have no conflicts of interest.