A useful practical definition of the quality of life (QOL) is “the individual’s perception of their position in life, in the context of the cultural and value systems in which they live and in relation to their goals, expectations, standards, and concerns.” QOL is an abstract construct; it reflects the broad range of facets of an individual’s condition (physical, psychological, social), complementing the concept of health as “a state of complete physical, mental, and social well-being and not merely the absence of disease or infirmity [1, 2].” Skin diseases are rarely lifexthreatening; however, they are capable of a massive impact on QOL; for instance, psoriasis has been found to affect QOL in a comparable way to heart failure [3]. Contributing factors encompass physical discomfort, time spent on treatment, staining of linen and clothes with skin scales or blood, and the obvious nature of skin disease, with its adverse impact on body image and its consequences on social function, which may be not commensurate with clinical severity. Physical symptoms such as pain and itching can strongly affect QOL [4]; related problems such as functional limitations, financial burden, and side effects of treatment all contribute to a poor QOL [5]. Accordingly, an assessment of QOL helps evaluate the impact of a patient’s dermatosis on well-being.

Quantification of this impact is a relatively recent endeavor prompted by the desire to gauge the outcomes of medical intervention. Several QOL measurement tools have been developed to assess disease impact on patient well-being in a reliable, statistically validated way. The literature stresses that patients and dermatologists often have different perceptions of a patient’s QOL and that this discrepancy is associated to a lower degree of satisfaction with medical care [6]. The findings of the application of QOL tools can be used together with clinical assessment and disease activity parameters to score disease severity objectively. Indeed, some tools afford analysis of the aspects of disease that distress a patient the most (e.g., symptoms such as itching or pain, the esthetic problem, the financial burden), facilitating the provision of personalized care. QOL assessments can also be used to evaluate the effectiveness of therapeutic interventions and monitor disease course over time, besides providing a valuable patient-based endpoint for clinical trials, to quantify changes in the disease process that may not correlate with changes in clinical disease activity [7]. Quantifying the burden of disease is also important for its patient advocacy implications, given its influence on the allocation of research support and clinical resources as well as the public perception of dermatological conditions [8]. Moreover, a greater awareness of QOL may enhance communication and shared decision making between physician and patient. Greater patient satisfaction involves greater adherence to treatment, a more stable relationship with the physician, relief from anxiety, and an increased sense of personal control on events, all of which contribute to the recovery process [9].

However, QOL assessments are not routinely implemented by a number of healthcare professionals. The reasons include inadequate resources, limited time, or lack of appropriate instruments. Many clinicians are less familiar with QOL assessment administration or evaluation than with other diagnostic tests; QOL is sometimes viewed as providing “soft” data as opposed to “hard” laboratory data. Many practitioners report that it is more difficult to conduct QOL assessments on the elderly, because of higher rates of illiteracy, lower completion rates, cognitive disorders, and comorbidities. Patients find QOL questionnaires more acceptable if they offer fewer answer options and if questions/answers are in the form of words rather than numbers. Questionnaires that did not ask overly personal, sensitive, or irrelevant questions also seem to be favored. QOL assessments are not yet widely used in dermatology, although the interest in them is growing.

Studies exploring QOL in autoimmune bullous dermatosis (AIBD) have focused predominantly on pemphigus (i.e., pemphigus vulgaris, PV, and pemphigus foliaceus, PF) and bullous pemphigoid. Studies regarding other forms of pemphigus as well as other acquired bullous diseases such as mucous membrane pemphigoid, epidermolysis bullosa acquisita (EBA), and linear IgA bullous dermatosis are few and sparse.

67.2 Instruments Used to Study QOL in Pemphigus

A range of instruments, usually classified into generic, dermatology-specific, and disease-specific, have been used to assess QOL in clinical settings [10–12]. Generic QOL measures have been used by the greater medical community to assess life quality across general medical problems [13]. The benefits of generic tools include their breadth and applicability across different conditions and interventions; however, assessment of QOL requires instruments capable of detecting even minor changes in patient distress. Generic QOL instruments are not endowed with this degree of sensitivity, but are used nonetheless, because they provide a suitable means to compare cohorts across the greater community [14]. Generic tools make it possible to conduct comparisons across all diseases; for instance, the Medical Outcome Study 36-item Short-form Survey (SF-36) measures health-related QOL and is thus used to compare QOL in AIBD patients and in the general population [15]. Albeit devised as a generic health status indicator for use in population surveys and health policy studies, the SF-36 can also be combined with disease-specific tools as an outcome measure. It includes 36 items in a Likert-type or forced-choice format and measures eight dimensions: physical functioning (limitations in performing physical activities, e.g., bathing or dressing), role-physical (RP) (limitations in work and other daily activities connected with physical health), bodily pain (BP) (how severe and limiting pain is), GH (how general personal health is perceived by the patient), vitality (feeling tired and worn out vs energetic), social functioning (interference of physical or emotional problems with normal social activities), role-emotional (RE) (limitations in work and other daily activities due to emotional problems), and mental health (feeling nervous and depressed vs peaceful, happy, and calm). In addition, question 2 covers changes in health status during the past year; this is not counted in scoring the eight dimensions, but is used to estimate change in health from a cross-sectional administration of the SF-36. Scores for each domain range from 0 to 100, higher scores indicating better health.

Skin-specific measures used in dermatology allow for comparisons across cutaneous diseases. Several skin-specific QOL instruments have also been developed and have the advantage of being more sensitive and responsive than generic measures [16]. Although generic tools have been tested and validated repeatedly for their psychometric properties, it has been claimed that they do not necessarily highlight issues that are specific to patients with skin conditions. Dermatology-specific instruments generally explore a given domain more thoroughly than a generic measure, resulting in greater responsiveness to change, thus allowing comparison of patients with different skin conditions within dermatology. The Dermatology Life Quality Index (DLQI) was derived from patients with a variety of skin diseases and provides a simple tool to score the influence of skin disease on QOL [17]. It envisages ten questions addressing symptoms and feelings (items 1 and 2), daily activities (items 3 and 4), leisure (items 5 and 6), work and school (item 7), personal relationships (items 8 and 9), and treatment (item 10). Each question has four possible responses (“not at all,” “a little,” “a lot,” or “very much”) that are assigned a score of 0, 1, 2, or 3 respectively; “not relevant” is also assigned a score of 0. The sum of the scores of all responses gives a DLQI score that ranges from 30 to 0 points. Higher scores reflect greater QOL impairment; healthy subjects have an average score of 0.5 [18].

The Skindex, another skin-specific measure, was designed to assess the effect of symptoms and the burden on social function and emotional state. The original Skindex consisted of 61 items, but it has now been reduced to questionnaires of 29, 17, and 16 items (Skindex-29, Skindex-17, and Skindex-16, respectively), to decrease respondent burden and improve discrimination and evaluation. Higher values correspond to a worse QOL [19–21]. Used in conjunction with dermatological measures focusing specifically on skin-related issues, they are useful, for instance, in comparing patients with different skin diseases or in detecting changes in the effects of skin disease on patients in clinical trials or longitudinal studies [22].

Psychiatric-related measures are also used to investigate QOL in AIBD. Psychiatric morbidity is quite common among patients with skin diseases, and the prevalence of depression ranges between 25 and 40 % compared to only 2–5 % in the general population [18, 23]. The GHQ-12 is a self-administered 12-item questionnaire designed to measure psychological distress and to detect current nonpsychotic psychiatric disorders, such as depression and anxiety [24, 25]. The reliability and validity of the Italian version have been tested in several diseases, including dermatological conditions [26, 27]. Answers are scored on a four-point scale; for instance, the question “in the last weeks, did you feel under strain?” can be answered as “no,” “no more than usual,” “more than usual,” and “much more than usual.” When scored with the binary method (0–0–1–1), the GHQ-12 can be used as a screening tool to detect minor nonpsychotic psychiatric disorders, yielding final scores that range from 0 to 12. Patients scoring 4 or more are considered to be GHQ positive.

The Japanese Ministry of Health, Labor, and Welfare applies its own measure of disease burden. The questionnaire inquires about the respondent’s ability to perform common activities of daily living (ADL) unaided, including bathing, eating, cooking, shaving or applying makeup, using the toilet, using public transportation, and driving.

Disease-specific QOL instruments are even more perceptive than skin-specific tools, because they are sensitive to specific issues associated to a particular disease. The advantages of disease-specific instruments are that questions are perceived as being of direct relevance to the patient, having a high face validity, and that question specificity maximizes the ability to detect any changes in the disease process [28, 29]. However, the fact that investigators cannot apply the more specific instruments across diverse populations or different conditions hampers comparisons between groups [30–32]. An AIBD-specific measure would greatly benefit both patients and health practitioners. Researchers often use a combination of specific and generic QOL instruments to maximize responsiveness and comparability; a recent systematic review has suggested that harnessing a generic and a dermatology-specific tool may be the best combination of QOL instruments in dermatology [33].

Investigations into the adverse impact of AIBD on patients’ QOL have addressed primarily pemphigus, in particular PV. As a consequence, data regarding QOL in other forms of pemphigus and in other acquired bullous diseases, such as bullous pemphigoid, mucous membrane pemphigoid, EBA, and linear IgA bullous dermatosis, are quite scanty [34–36]. However, very few studies, using a variety of instruments, have explored QOL in patients with pemphigus.

67.3 Previous QOL Studies on Pemphigus

The earliest paper investigating QOL in AIBD involved 380 pemphigus patients in Japan [37]. Participants were stratified into those with mild (67.7 %), moderate (25.9 %), or severe (6.3 %) disease and were asked to complete a questionnaire regarding ADL and the financial implications of their condition. The survey found that patients retained good ADL independence, since 99.7 % were able to feed themselves and use the toilet unaided, 97 % were able to shave or apply makeup, 96.5 % were able to bathe, 90.8 % were able to use public transportation, 85.2 % could cook, and 82.3 % could drive without help. However, the economic burden was considerable, as 41.9 % (60 % of those with severe disease) reported a diminished income due to lost work time, and 41.3 % (57.9 % of those with severe disease) described their financial status as “poverty.” Since only 2.2 % of patients were experiencing active disease—the remainder being in varying phases of remission—the data show that pemphigus does not significantly impair ADL if appropriately treated. In contrast, its financial burden may be heavy despite clinical improvement, something that should be considered when assessing these patients.

Another study of QOL in pemphigus used the SF-36 to compare patients with pemphigus and the wider population in Morocco [38]. The study cohort included 30 consecutive pemphigus patients presenting to dermatology clinics or admitted to hospital; the control cohort consisted of 60 patients presenting with non-debilitating conditions such as verrucous warts or benign cutaneous tumors. Patient inclusion criteria were bullous dermatitis with fluid-filled bullae and intraepidermal division on a cutaneous biopsy, presence of acantholytic cells, and immunoglobulin deposition in the epidermis under direct immunofluorescence. Because of the high illiteracy rate, the questionnaires were completed by the investigators, although they had originally been designed either for self-administration or for administration by a trained examiner in person or by telephone. To minimize biases, a single interviewer completed the questionnaires for all participants, whether literate or illiterate. The authors identified significant differences between cohorts, with the pemphigus group displaying lower mean scores across all dimensions but “Physical Pain” and “Change Compared to the Previous Year.” Physical status and emotional status were the most affected domains, with facial involvement and lesion extent correlating well with overall scores. As many as 70 % reported being deeply ashamed of their appearance; 60 % were anxious about what others thought of their disease, and 63 % reported a significant loss of self-confidence. Repercussions on sexual function were also extensive, 81 % of patients reporting concern. Cultural factors were noted to play a large role in QOL deterioration due to rife misconceptions associating skin conditions to poor hygiene or improper sexual practices. Further considerations were the high cost of disease management and social issues such as the impairment of young women’s marriage prospects. The use of a generic QOL tool therefore identified a significant impact of pemphigus on QOL and highlighted that besides the functional aspects of disease burden, the SF-36 also explores social domains and self-image, which are very significant in this disease.

QOL was evaluated in 27 German patients with PV using a dermatology-specific tool [39]. Their mean Dermatology Life Quality Index (DLQI) was 10.1 ± 6.6 compared to healthy individuals, whose mean score was 0.5 ± 1.1, leading the authors to conclude that the disease greatly impairs QOL. The mean DLQI of patients with mucosal involvement was 10.4 ± 7.3, while that of patients whose mucosa was spared was 9.3 ± 5.1, indicating a poorer QOL in the former individuals. Itching was also associated to QOL deterioration, as these patients scored 11.5 ± 7.5 vs 7.9 ± 4.8 of those without itching. Burning was also found to impair QOL, since the index score of patients who suffered from it was almost double that of patients who did not (10.8 ± 6.6. vs 5.5 ± 5.7). None of these differences achieved statistical significance, possibly due to the small cohort size. Notably, 22.2 % of participants reported that their QOL was unchanged. The mean DLQI of pemphigus was higher than the mean scores of similar AIBD, such as bullous pemphigoid (6.92 ± 3.80), perhaps because of the low frequency of mucosal involvement in bullous pemphigoid or because its good response to lower drug doses entails fewer and less unpleasant adverse effects. For example, bullous pemphigoid typically responds to prednisone doses not exceeding 0.5 mg/kg/day, whereas pemphigus usually requires a starting dose of at least 1 mg/kg/day [40, 41]. The authors concluded that the DLQI may be useful as an additional measure of treatment response in patients with pemphigus. The mean DLQI of pemphigus was also higher than those of other dermatological conditions, such as basal cell carcinoma (2.0 ± 2.2), but it was lower than that of atopic eczema (12.5 ± 5.8)—an evaluation facilitated by the use of a skin-specific QOL instrument to compare severity [39]. However, QOL is a complex area influenced by several factors, and the DLQI does not appear to be an optimal tool to assess pemphigus patients. Indeed the questionnaire was devised using data from 120 patients, only one of whom had blisters, whereas there were at least 10 with eczema, 14 with psoriasis, and 15 with acne [42, 43]. Therefore, the DLQI is likely more appropriate to assess patients with these conditions than individuals with pemphigus. In addition, given that scores reflect tool construction, these patients may quite possibly place emphasis on aspects of their handicap that are not covered by the DLQI, and no emphasis at all on some that are. Moreover, questions regard only the previous week, which may miss flares in chronic disease.

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