Quality-of-Life Measurement in Blistering Diseases

Bullous pemphigoid (BP) autoantibody levels are generally elevated in patients with BP but can be present nonspecifically in patients without BP.

To analyze the clinical findings of patients with elevated BP180 or BP230 autoantibody levels and negative direct immunofluorescence (DIF) study findings.

We retrospectively reviewed records of patients seen at our institution during January 1, 2005-December 31, 2015, who were positive for BP180 or BP230 autoantibodies and had a negative DIF study finding. These patients' demographic characteristics and BP180 and BP230 levels were compared with those of a BP control group who were positive for BP180 or BP230 autoantibodies and had positive DIF study findings.

We identified 208 patients with BP autoantibodies but without positive DIF study findings. These patients' mean age and enzyme-linked immunosorbent assay values were significantly lower than those of the control group. Dermatitis was the most common final clinical diagnosis. Of the 208 patients, 41 (19.7%) had at least 2 years' follow-up. Four patients had positive DIF results upon repeating the test and ultimately received pemphigoid diagnoses.

Retrospective design with limited follow-up.

Patients might harbor serum BP autoantibodies in the context of a wide range of dermatoses. Low positive BP180 and BP230 autoantibody levels should not be overinterpreted as evidence for BP in the setting of a negative DIF.

The Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity-Score (ABSIS) scores have been proposed to provide an objective measure of pemphigus activity. These scores have been evaluated only on already treated patients mainly with mild to moderate activity. The objective was to assess the interrater reliability of ABSIS and PDAI scores and their correlation with other severity markers in a large international study.

Consecutive patients with newly diagnosed pemphigus were enrolled in 31 centers. Severity scores were recorded during a 24-month period by the same two blinded investigators. Serum was collected at each visit for ELISA measurement of anti-desmoglein antibodies. The intraclass correlation coefficient (ICC) and Spearman rank correlation coefficient were calculated.

A total of 116 patients with pemphigus vulgaris (n = 84) or pemphigus foliaceus (n = 32) were included. At baseline, the ABSIS and PDAI ICCs were 0.90 (95% confidence interval [CI] = 0.85–0.93), and 0.91(95% CI = 0.87–0.94), respectively. The ICCs for PDAI were higher in moderate and extensive pemphigus (ICC = 0.82, 95% CI = 0.63–0.92 and ICC = 0.80, 95% CI = 0.62–0.90, respectively) than in patients with intermediate (significant) extent (ICC = 0.50, 95% CI = 0.27–0.68). Conversely, the ICCs for ABSIS were lower in patients with moderate extent (ICC = 0.44, 95% CI = 0.004–0.74) than in those with intermediate or extensive forms, (ICC = 0.69, 95% CI = 0.51–0.81 and ICC = 0.75, 95% CI = 0.51–0.88, respectively). During patients’ follow-up, the ICCs of both ABSIS and PDAI scores remained higher than 0.70.

ABSIS and PDAI skin (r = 0.71 and r = 0.75) but not mucosal (r = 0.32 and r = 0.37) subscores were correlated with the evolution of anti-DSG1 and anti-DSG3 ELISA values, respectively. ABSIS and PDAI scores are robust tools to accurately assess pemphigus activity.

This review examines the work productivity in patients with autoimmune blistering diseases (AIBDs). Work productivity and employment are important aspects of a patient’s life, which can be affected by diseases. The Work Productivity and Activity Impairment Questionnaire (WPAIQ) is a validated instrument that can measure work productivity and assess the impact of disease on patients’ work lives. There is currently a paucity of research that investigates the reason why AIBDs cause such a large impact on work productivity and whether AIBDs affect employment status. Using quality of life (QoL) instruments in conjunction with the creation of an adapted WPAIQ to examine the reasons behind work impairment may further characterize these effects and unveil a deeper understanding of stigmatization in the workplace as a factor of loss of work productivity.

Inherited and autoimmune blistering diseases are rare, chronic, and often severe disorders that have the potential to significantly affect patients’ quality of life. The effective management of these conditions requires consideration of the physical, emotional, and social aspects of the disease. Self-esteem is integral to patients’ ability to cope with their illness, participate in treatment, and function in society. This article discusses quality-of-life studies of patients with blistering diseases with a particular focus on self-esteem issues that patients may face.

There is a paucity of literature on quality of life (QoL) in patients with oral potentially malignant disorders (OPMDs) despite these conditions being relatively common, chronic, and potentially debilitating. The aim of this paper is to systematically review the literature on QoL in patients with OPMDs. A search from electronic databases PUBMED, MEDLINE, and CINAHL Plus retrieved 180 titles after removing duplicates, and a further 4 papers were identified by hand searching. Study of the abstracts identified 25 truly relevant articles, which were studied in full. Of these, 14 met our strict inclusion criteria. Most studies were cross-sectional; most were from Europe and have evaluated QoL in patients with oral lichen planus (OLP). The findings differ but, overall, do not provide evidence that patients with OPMDs have a poorer QoL compared with healthy patients. Several things may explain this apparently surprising conclusion. First, the quality of most articles was moderate or weak; second, most studies assessed QoL only in patients with OLP and cannot be generalized to all patients with OPMDs; last, direct comparisons between patients with OPMD and healthy controls were rarely included. The validity of the QoL instrument used for patients with OLP was frequently inadequate.