Impact of Generalized Pustular Psoriasis from the Perspective of People Living with the Condition: Results of an Online Survey.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare disease characterized by episodic worsening (flares). Knowledge of the burden of GPP and the experience of affected individuals is limited. AIMS: To conduct a survey of people living with GPP to understand how they experience GPP flares, which therapies they have received and are receiving, and how GPP impacts their activities of daily living. METHODS: The online survey consisted of 43 questions answered by individuals recruited from an opt-in market research database. The research team performed a targeted outreach to identify individuals with GPP. The survey included screening questions to determine if potential participants qualified for inclusion. Eligible individuals were US residents aged ≥ 18 years who self-reported that they had been diagnosed with GPP. Respondents provided consent to participate and received compensation (fair market value) for their time. RESULTS: Between August 4 and 14, 2020, 66 people living with GPP in the USA were surveyed. Most participants were female, aged 40-59 years, had been diagnosed ≥ 1 year previously, and had experienced ≥ 2 flares in the past year. A substantial proportion of respondents had symptoms for years, had consulted multiple healthcare professionals, and experienced misdiagnoses before receiving a diagnosis of GPP. Emotional stress was the most common cause of flares and many respondents reported a fear of flares. Respondents defined flares by the presence of itching, an increase in the size of the affected area, more crusts or pustules, and fatigue. A change in mood was the most burdensome symptom. Most respondents were receiving topical corticosteroids and only approximately one-third felt their condition was well controlled. GPP had an impact on activities of daily living even in the absence of flares and many respondents felt that their physician did not understand the level of emotional, psychological, or physical pain caused by GPP. CONCLUSIONS: GPP imposes a substantial emotional burden on patients, with wide-ranging impacts on activities of daily living beyond the physical discomfort of skin lesions.

Oral health related quality of life and disease severity in autoimmune bullous diseases.

BACKGROUND: There is an increased risk of long-term dental and periodontal disease in autoimmune bullous diseases (AIBD). AIMS: In this cross-sectional study, we aimed to determine whether the oral health-related quality of life status (OHRQoL) was associated with disease severity and activity in patients with AIBD. SUBJECTS AND METHODS: 67 patients with AIBD were enrolled in this study. Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) was used to evaluate the disease severity. The score was categorized as a significant course (≥17) and moderate course (<17). Oral health impact profile-14 (OHIP-14) questionnaire was filled to assess the OHRQoL. Self-reported oral health status and oral lesion related pain score were also evaluated in the study group. RESULTS: OHIP-14 score was significantly higher in active patients (42.28 ± 13.66) than inactive patients (29.08 ± 12.25) (P = 0.004) and it was correlated with the pain score (6.33 ± 2.78; r = 0.409, P = 0.013). Furthermore, OHIP-14 score was higher in patients with a significant disease course (45.18 ± 15.08) (P = 0.010) than in patients with a moderate course (36.09 ± 9.73). CONCLUSIONS: OHRQoL may be useful in the disease management and treatment. Since it can be affected by both presence of oral erosions and disease severity, a collaboration between dermatologists and dentists could be crucial to the disease management in AIBD.

The effect of autoimmune blistering diseases on work productivity.

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.

Chinese version of the treatment of autoimmune bullous disease quality of life questionnaire: Reliability and validity.

BACKGROUND: Treatments for autoimmune blistering disease carry significant risks of medical complications and can affect the patient's quality of life. Recently, the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was developed in Australia. OBJECTIVE: The objective of this study was to evaluate the reliability and validity of the Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire in Chinese patients with autoimmune blistering diseases. METHODS: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was produced by forward-backward translation and cross-cultural adaptation of the original English version. Autoimmune blistering disease patients recruited in the study self-administered the Chinese Treatment of Autoimmune Bullous Disease Quality of Life questionnaire, the Dermatology Life Quality Index and the 36-item Short-Form Health Survey. Reliability of the Chinese Treatment of Autoimmune Bullous Disease Quality of Life was evaluated using internal consistency and test-retest (days 0 and 7) methods. Validity was analyzed by face, content, construct, convergent and discriminant validity measures. RESULTS: A total of 86 autoimmune blistering disease patients were recruited for the study. Cronbach's alpha coefficient was 0.883 and the intraclass correlation coefficient was 0.871. Face and content validities were satisfactory. Convergent validity testing revealed correlation coefficients of 0.664 for the Treatment of Autoimmune Bullous Disease Quality of Life and Dermatology Life Quality Index and -0.577 for the Treatment of Autoimmune Bullous Disease Quality of Life and 36-item Short-Form Health Survey. With respect to discriminant validity, no significant differences were observed in the Treatment of Autoimmune Bullous Disease Quality of Life scores of men and women (t = 0.251, P = 0.802), inpatients and outpatients (t = 0.447, P = 0.656), patients on steroids and steroid-sparing medications (t = 0.672, P = 0.503) and patients with different autoimmune blistering disease subtypes (F = 0.030, P = 0.971). LIMITATIONS: Illiterate patients were excluded from the study. The patients were from a single hospital and most of their conditions were in a relatively stable status. CONCLUSION: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire is a reliable and valid instrument to measure treatment burden and to serve as an end point in clinical trials in Chinese autoimmune blistering disease patients.

Quality of Life in Greek Patients with Autoimmune Bullous Diseases Assessed with ABQOL and TABQOL Indexes.

is missing (Short communication).

Reliability and validity of the Chinese version of the autoimmune bullous disease quality of life (ABQOL) questionnaire.

BACKGROUND: The autoimmune bullous diseases quality of life (ABQOL) questionnaire was recently developed by an Australian group and has been validated in Australian and North American patient cohorts. It is a 17-item, multidimensional, self-administered English questionnaire. The study aimed to validate the Chinese version of the ABQOL questionnaire and evaluate the reliability in Chinese patients. METHODS: The Chinese version of the ABQOL questionnaire was produced by forward-backward translation and cross-cultural adaptation of the original English version. The ABQOL questionnaire was then distributed to a total of 101 patients with autoimmune bullous diseases (AIBDs) together with the Dermatology Life Quality Index (DLQI) and the 36-item Short Form Health Survey (SF-36). Validity was analyzed across a range of indices and reliability was assessed using internal consistency and test-retest methods. RESULTS: The Chinese version of the ABQOL questionnaire has a high internal consistency (Cronbach's alpha coefficient, 0.88) and test-retest reliability (the intraclass correlation coefficient, 0.87). Face and content validity were satisfactory. Convergent validity testing showed that the correlation coefficients for the ABQOL and DLQI was 0.77 and for the ABQOL and SF-36 was -0.62. In terms of discriminant validity, there was no significant difference between the proportions of insensitive items in ABQOL and DLQI (p = 0.236). There was no significant difference between the proportions of insensitive items in ABQOL and SF-36 (p = 0.823). CONCLUSIONS: The Chinese version of the ABQOL questionnaire has adequate validity and reliability. It may constitute a useful instrument to measure disease burden in Chinese patients with AIBDs.

Measuring of quality of life in autoimmune blistering disorders in Poland. Validation of disease - specific Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires.

PURPOSE: Autoimmune bullous dermatoses (AIBD) are rare, severe diseases resulting from some antibodies activity against the different adhesion structures within the skin and/or mucosa. Few studies investigated quality of life (QOL) in AIBD by generic and dermatology-specific instruments, all reporting strong impact on QOL. Recently, disease-specific measurement tools have been developed: Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The aim of this study was to test the reliability and validity of ABQOL and TABQOL by developing the first foreign language versions and to evaluate ABQOL and TABQOL in Polish patients. MATERIAL AND METHODS: The study enrolled 80 patients from the tertiary referral center for AIBD at the outpatient clinic or on admission to the hospital. Sixty six patients completed the 17-item questionnaires of each ABQOL and TABQOL at day 0 and after 5-7 days. Both questionnaires were translated into Polish according to protocol. RESULTS: The internal consistency and test-retest reliability were high (Cronbach α=0.95 for ABQOL, α=0.87 for TABQOL), (R=0.98 for ABQOL, R=0.86 for TABQOL). In convergent validity, the correlation of ABQOL and TABQOL was strong (R=0.81), but low with objective disease activity scales. The strongest impact of AIBD on QOL has been observed in flares and in patients with the onset below 70 years of age. The patients with bullous pemphigoid had the highest QOL compared to other AIBD patients. CONCLUSIONS: The ABQOL and TABQOL are reliable and valid instruments for the assessment of QOL in AIBD.

Loss of Work Productivity and Quality of Life in Patients With Autoimmune Bullous Dermatoses.

BACKGROUND: Little is known about quality of life and work productivity in autoimmune bullous dermatoses (AIBDs). OBJECTIVE: To determine the impact of AIBDs on quality of life and work productivity. METHODS: An observational cross-sectional study took place between February and May 2013 at an AIBD tertiary referral centre. Ninety-four patients were included. All participants completed the Dermatology Life Quality Index and the Work Productivity and Activity Impairment-Specific Health Problem questionnaires. RESULTS: Responders to treatment had less impairment (P<.001) than nonresponders. Patients with severe AIBD had significantly more impairment that those with mild (P<.001) and moderate (P=.002) AIBD. Greater impairment was associated with higher percentage of work missed. Those with a higher Dermatology Life Quality Index score had greater work impairment and overall activity impairment (P=.041, P=.024). Nonresponders had increased impairment while working (P<.001), overall work impairment (P<.001), and activity impairment (P<.001). Severely affected patients had worse impairment in all Work Productivity and Activity Impairment Questionnaire domains. CONCLUSIONS: AIBD has the potential to be a large burden on ability to work and quality of life. Larger studies are needed to clarify how these domains change over time and whether or not they improve with treatment.

Quality of life index in autoimmune bullous dermatosis patients.

BACKGROUND: Autoimmune bullous dermatoses are complex diseases triggered by autoantibodies action against epidermal antigens or the dermoepidermal junction. Blisters and vesicles that evolve with erosion areas characterize them. Although rare, they present high morbidity, affecting the quality of life of patients. OBJECTIVES: To assess the magnitude of autoimmune bullous dermatoses on life quality of patients treated in a public university service in countryside of Brazil. METHODS: This cross-sectional study was based on an inquiry with autoimmune bullous dermatoses patients assisted at outpatient university referral service. Elements related to quality of life were evaluated by the Dermatology Life Quality Index, as well as clinical and demographic data. RESULTS: The study evaluated 43 patients with pemphigus foliaceus, 32 with pemphigus vulgaris, 6 with bullous pemphigoid and 3 with dermatitis herpetiformis. The average age was 48 ± 16 years and 34 (40%) were female. The median score (p25-p75) of the Dermatology Life Quality Index was 16 (9-19), classified as "severe impairment" of life quality, in which the greater impact was related to symptoms and feelings, daily and leisure activities. CONCLUSIONS: Autoimmune bullous dermatoses inflict severe impairment of quality of life for patients followed by a public outpatient clinic in the countryside of Brazil.

Quality of life index in autoimmune bullous dermatosis patients

BACKGROUND: Autoimmune bullous dermatoses are complex diseases triggered by autoantibodies action against epidermal antigens or the dermoepidermal junction. Blisters and vesicles that evolve with erosion areas characterize them. Although rare, they present high morbidity, affecting the quality of life of patients. OBJECTIVES: To assess the magnitude of autoimmune bullous dermatoses on life quality of patients treated in a public university service in countryside of Brazil. METHODS: This cross-sectional study was based on an inquiry with autoimmune bullous dermatoses patients assisted at outpatient university referral service. Elements related to quality of life were evaluated by the Dermatology Life Quality Index, as well as clinical and demographic data. RESULTS: The study evaluated 43 patients with pemphigus foliaceus, 32 with pemphigus vulgaris, 6 with bullous pemphigoid and 3 with dermatitis herpetiformis. The average age was 48 ± 16 years and 34 (40%) were female. The median score (p25-p75) of the Dermatology Life Quality Index was 16 (9-19), classified as "severe impairment" of life quality, in which the greater impact was related to symptoms and feelings, daily and leisure activities. CONCLUSIONS: Autoimmune bullous dermatoses inflict severe impairment of quality of life for patients followed by a public outpatient clinic in the countryside of Brazil. .

Reliability of the autoimmune bullous disease quality of life (ABQOL) questionnaire in the USA.

PURPOSE: To evaluate the reliability of the autoimmune bullous diseases quality of life (ABQOL) questionnaire in a North American patient cohort. METHODS: Patients attending the dermatology clinics of the University of Pennsylvania with a histological diagnosis of an autoimmune bullous disease (AIBD) and self-reported proficiency in English were recruited to participate in the study. Patients completed the ABQOL questionnaire at Day 0 and Day 3. Internal consistency was calculated through Cronbach's alpha. Test-retest reliability was determined by the intraclass correlation coefficient. RESULTS: Of the 45 patients enrolled in the study, 39 patients (87 %) participated to completion. The mean age was 60.7 years with an equal sex distribution observed. Patients had a range of AIBD including pemphigus vulgaris, bullous pemphigoid, pemphigus foliaceus, epidermolysis bullosa acquisita, mucous membrane pemphigoid and linear IgA disease. Cronbach's alpha was calculated to be 0.90. The intraclass correlation coefficient was calculated to be 0.93 (95 % confidence interval 0.88-0.94). CONCLUSION: The ABQOL was found to be reliable tested by internal consistency and test-retest reliability in an American patient cohort. It represents a promising disease-specific outcome measure for patients with AIBD.

The development and validation of the treatment of autoimmune bullous disease quality of life questionnaire, a tool to measure the quality of life impacts of treatments used in patients with autoimmune blistering disease.

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.

Bullous dermatitis artefacta.

Bullous artefactual dermatoses are rare and may be induced by various techniques, including chemicals, heat, or electrical current. Proving a factitial etiology and identifying the mechanism of injury may be difficult. We describe the clinical features and histopathology of 2 patients with bullous disease induced by electrical current or heat. Physical examination in both patients demonstrated geometrically shaped tense bullae. Skin biopsies revealed epidermal necrosis overlying a pauci-inflammatory subepidermal cleft, with homogenization of underlying superficial dermal collagen. In 1 of the 2 patients, there was prominent vertical elongation of keratinocyte nuclei and also of cytoplasmic processes. Direct immunofluorescence study of skin plus testing of serum by indirect immunofluorescence and enzyme-linked immunosorbent assay for BP180 and BP230 antibodies revealed no evidence for immunobullous disease in either patient. Vertical elongation of keratinocyte nuclei, often attributed to a polarization effect of electrical current, is characteristic of electrical burn but also may be induced by thermal injury. These 2 patients highlight the importance of histopathology in confirming a diagnosis of bullous dermatitis artefacta.

Quality of life in patients with bullous dermatoses.

Genetic and acquired bullous dermatoses can severely affect multiple domains of a patient's quality of life (QOL). Integrating formal evaluation of QOL into the clinical evaluation of patients facilitates an objective assessment of disease severity, mapping of disease trajectory, and captures therapeutic intervention outcomes. There have been 5 studies evaluating QOL in autoimmune dermatoses and 4 studies reviewing QOL in the genodermatoses. All literature to date indicates a significant disease burden in this setting. The development of formal QOL instruments has facilitated quantification of QOL deficits in this arena and offers promising tools for patient assessment in the future.

Hospice health care: management of a case of bullous pemphigoid utilizing the hospice interdisciplinary team.

An association between bullous pemphigoid and internal malignant neoplasms has been reported by many authors, primarily in case report formats. Stone and Schroeter, however, compared cases of bullous pemphigoid with age and sex-matched control patients with contact dermatitis and psoriasis and found no evidence of an increased association between malignant neoplasms and bullous pemphigoid. A few cases of bullous pemphigoid occurring after radiation therapy for malignant neoplasms have been reported. A case of bullous pemphigoid which occurred after radiation therapy for squamous carcinoma of the cervix is presented. The interactive intervention of a hospice interdisciplinary team was crucial to the care and resolution of this patient's dermatological condition. Medical, psychological, and social aspects of the case are discussed.

[Retarded bullous dermographism]. / Dermografismo retardado ampollar.

A case of what we denominate Bullous Delayed Dermographism in a woman who had neither allergic nor urticarial antecedents is registered. We comment the features of the lesions which appeared after a period of 4 to 12 hs. This lesions appeared by friction or percussion. We describe the existence of an endocrine and psychological predisposition mechanism and the non specificity of the histopathological characters. The nonexistence of bibliographical antecedents of the illness is noted. Also its difference with bullous and pressure urticaria. This differentiation is based on clinical features, and the nonexistence of allergic antecedents and urticarial lesions. Also on the existence of friction mechanism different to the simple pressure and the nonexistence of immunoglobulins in the subepidermal blister.