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Autoimmune blistering skin diseases (AIBD) encompass a group of diseases characterised by cutaneous and/or mucocutaneous fragility. Multiple risk factors contributing to osteoporosis exist in AIBD patients, including use of long‐term systemic corticosteroid therapy (SCT), decreased mobility and the presence of chronic inflammation. Despite this, there is no consensus on the prophylaxis of osteoporosis in AIBD, especially in the absence of SCT. To systemically review the current literature on the association between osteoporosis/osteopenia and AIBD, a comprehensive literature search was performed on six online databases with search terms related to bone mineral density (BMD) and AIBD. A total of 314 articles were screened by their abstract and/or full text. A total of 20 peer‐reviewed full‐text articles addressing BMD in patients with AIBD were identified. Eight articles examined the association between osteoporosis and pemphigus. Only two articles examined the association between osteoporosis and pemphigoid patients. Three articles examined the effectiveness of osteoporosis prophylaxis in AIBD patients. Seven papers examined the levels of vitamin D in AIBD patients. Few case‐control studies examine osteoporosis in pemphigus in the context of SCT, with consistent findings. However, there is scarce literature examining the risk of osteoporosis in pemphigoid, or in AIBD without SCT. Prophylaxis and screening of osteoporosis in AIBD is suboptimal and more attention in this area is required to avoid future complications related to osteopenia and osteoporosis. [ABSTRACT FROM AUTHOR]

Background: Autoimmune blistering diseases (AIBD) are known to negatively impact upon quality of life (QoL); however, there is a paucity of research on the effect of AIBD on work productivity. AIBD can be quite disfiguring in terms of a patient's appearance due to their blistering nature. Objective: To determine the impact of AIBD on work productivity and to determine whether patients are stigmatized at work due to their appearance. Methods: Sixty‐one patients with AIBD completed the Work Productivity and Activity Impairment Questionnaire‐Specific Health Problem (WPAIQ‐SHP), the Dermatology Life Quality Index (DLQI), the Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life questionnaires (TABQOL). Results: Non‐responders to treatment had more work and activity impairment compared to responders. Worse WPAIQ‐SHP scores were correlated with higher ABQOL, TABQOL and DLQI scores. Approximately 14.8% of subjects experienced stigmatization at work due to their appearance. The most common body areas stigmatized were easily visible sites, particularly the hands, arms and feet, with the majority of occurrences related to co‐workers; for some patients, this stigmatization occurred on a daily basis. Loss of productivity at work was statistically much higher in those with higher disease severity, ABQOL & TABQOL scores and in non‐responders to treatment. Conclusion: Autoimmune blistering diseases negatively impacts upon work productivity and activity. Stigmatization was common in the workplace which leads to increased stress, itself a stimulator of pemphigus. [ABSTRACT FROM AUTHOR]

Background Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score ( ABSIS) and Pemphigus Disease Area Index ( PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus. Objectives To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. Methods In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment ( PGA) and Dermatology Life Quality Index ( DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. Results Ninety-six patients with pemphigus vulgaris ( n = 77) or pemphigus foliaceus ( n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS ( P < 0·001), PDAI ( P < 0·001), PGA ( P < 0·001) and DLQI ( P = 0·03) scores. Conclusions This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to article "Inherited Epidermolysis Bullosa: Updated Recommendations on Diagnosis and Classification," that was published in the previous issue.

Background Treatments for autoimmune blistering diseases have significant risk of medical complications and quality of life impacts during treatment, and it is difficult to differentiate these impacts from disease burden or the effects of treatment. Objectives To develop a quality of life instrument specific to the effects of treatments used in patients with autoimmune bullous disease ( AIBD). Methods A comprehensive item generation process was used to build a 45-item pilot Autoimmune Bullous Disease Quality of Life ( ABQOL) questionnaire, distributed to 70 patients with AIBD. Experts in bullous disease refined the pilot ABQOL, selecting only those questions pertaining to the treatment effects. This pilot Treatment of Autoimmune Bullous Disease Quality of Life ( TABQOL) questionnaire was administered to 70 patients, before factor analysis was performed to yield the final questionnaire of 17 questions. Validity and reliability were evaluated across a range of indices. Results Face and content validity were established through a comprehensive patient interview process, expert review and summaries of treatments used. The questionnaire was found to have appropriate correlation with the Dermatology Life Quality Index ( r = 0·64) and the level of treatments used ( P < 0·01), and was found to be responsive to overall variations in treatment burden. The TABQOL was also found to be a reliable instrument as evaluated by internal consistency ( Cronbach α = 0·892) and test-retest reliability ( r = 0·99). Conclusions We have shown that the TABQOL questionnaire is a valid and reliable instrument that may to be used to measure treatment burden in AIBD and serve as an end point in clinical trials. [ABSTRACT FROM AUTHOR]

ORIGINAL ARTICLE, p 1307 [ABSTRACT FROM AUTHOR]