Comorbid mental health issues in patients with pemphigus vulgaris and pemphigus foliaceus.

The term 'pemphigus' refers to chronic autoimmune skin disorders that cause blistering erosions on the skin and oral mucosa. The two major clinical forms are pemphigus vulgaris and pemphigus foliaceus. Although rare, they confer a stark symptomatic burden upon patients that significantly impacts daily life. Comorbid mental health issues are not routinely screened for in patients with pemphigus, and current UK guidance provides no formal provision for the identification and treatment of psychological issues. This review is the first of its kind, to our knowledge, to systematically examine the available evidence on mental health issues in pemphigus. Published work suggests that the incidence of anxiety and depression is much higher in patients with pemphigus compared with both the general population and with patients having other chronic skin disorders. Disease severity appears to be closely linked to mental health, with worsening of pemphigus associated with deteriorations in psychological wellbeing. Corticosteroids, which are associated with depression in chronic use, are the current first-line therapy for pemphigus and have been identified as a potential confounder and independent risk factor for mental health comorbidity in pemphigus. Current evidence is unclear whether a bidirectional relationship exists between mental health and pemphigus severity, and more thorough research is required to develop understanding of this issue. In conclusion, we have identified a high incidence of mental health comorbidity in pemphigus, and recommend routine screening of patients with pemphigus for mental health issues and signposting toward mental health services as an initial measure to address this.

Family dermatology life quality index in patients with pemphigus vulgaris: A cross-sectional study.

BACKGROUND AND AIMS: Pemphigus vulgaris is a rare autoimmune intraepidermal vesiculobullous disease involving the skin and mucosa. It impacts the quality of life of both patients and their families. METHODS: A total of 70 patients with pemphigus vulgaris (either outpatient or hospitalized) were enrolled using the simple sampling method between 2016 and 2017 from the dermatology clinic at Faghihi Hospital, Shiraz, Iran. A validated Persian version of the Family Dermatology Life Quality Index (FLDQI) questionnaire was filled by a family caregiver. The questionnaire contained 10 items assessing the quality of life of the family. Demographic variables were recorded in a separate form. RESULTS: The mean age of the patients was 51 ± 11.3 years and that of the family caregivers was 32 ± 8.8 years. The FLDQI score was higher (poorer quality of life) if the patient was male, older, had shorter disease duration or had fewer disease recurrences (P = 0.046, 0.01, 0.001 and >0.001, respectively). Higher scores were also obtained in the less-educated caregivers (P = 0.026) but there was no association with either gender or age (P = 0.399, 0.1). CONCLUSION: Pemphigus vulgaris significantly affects the Family Dermatology Life Quality Index. Education and counseling of family caregivers by various support groups such as Pemphigus Family Associations could be effective in improving the quality of life of the caregivers. LIMITATIONS: This study did not assess the effect of comprising domain analysis, severity of disease, patients' Dermatology Life Quality Index (DLQI), mucosal involvement, response to treatment, outpatient or admitted status, socioeconomic status, or the quality of life among the various family members.

Assessment of the Quality of Life, Prevalence of Depression, and the Level of Interleukin 6 in Patients with Pemphigus Vulgaris.

Pemphigus vulgaris (PV) is a life-threatening, autoimmune blistering disease affecting the skin and mucous membranes, exerting a detrimental effect on the quality of life (QOL). Our aim was to evaluate the psychological status and QOL of patients with PV and investigate Interleukin-6 (IL-6) as a possible contributor to the pathogenesis of pemphigus and associated depression. The study included 22 patients with PV, 21 patients with depression, and 20 normal controls. All the 63 participants were subjected to assessment of their QOL, psychiatric profile, as well as estimation of serum level of IL-6. All (100%) of the included patients with PV had a negative effect on their QOL, which was significant compared with controls (P<0.001). Among patients with PV, 13 patients (59.1%) had depression. IL-6 was non-significantly elevated in the pemphigus group when compared with the controls (P=0.057). QOL was significantly worse in the depressed pemphigus subgroup compared with the non-depressed pemphigus subgroup (P=0.006 and <0.001) respectively. However, IL-6 was non-significantly elevated in the depressed pemphigus subgroup compared with the non-depressed pemphigus subgroup (P=0.095). A marked deterioration in the QOL was observed in patients with pemphigus. More than 50%, but not all, of patients with pemphigus had depression. IL-6 was non-significantly elevated in patients with pemphigus.

The Risk of Depression in Patients with Pemphigus: A Nationwide Cohort Study in Taiwan.

Pemphigus is a chronic dermatological disorder caused by an autoimmune response and is associated with a high proportion of comorbidities and fatalities. The aim of this study was to investigate the risk of depression in patients with pemphigus. Data were derived from the National Health Insurance Research Database recorded during the period 2000-2010 in Taiwan. Multivariate Cox proportional hazards regression models were used to analyze the data and assess the effects of pemphigus on the risk of depression after adjusting for demographic characteristics and comorbidities. Patients with pemphigus were 1.98 times more likely to suffer from depression than the control group (pemphigus, adjusted HR: 1.99, 95% CI = 1.37-2.86). People aged ≥65 years were 1.69 times more likely to suffer from depression than those aged 20-49 years (≥65 years, adjusted HR: 1.42, 95% CI = 0.92-2.21). Female and male patients with pemphigus were respectively 2.02 and 1.91 times more likely to suffer from depression than the control group (female, adjusted HR: 2.09, 95% CI = 1.24-3.54; male, adjusted HR: 1.87, 95% CI = 0.97-3.60). People with HTN, hyperlipidemia, asthma/COPD, and chronic liver disease were respectively 1.73, 2.3, 2.2, and 1.69 times more likely to suffer from depression than those without these comorbidities (HTN, adjusted HR: 0.75, 95% CI = 0.41-1.42; hyperlipidemia, adjusted HR: 1.48, 95% CI = 0.78-2.82; asthma/COPD, adjusted HR: 1.4, 95% CI = 0.72-2.69; and chronic liver disease, adjusted HR: 1.61, 95% CI = 1.07-2.43). There was a significant association between pemphigus and increased risk of depression. Female patients had a higher incidence of depression.

Subjective well-being in patients with pemphigus: a path analysis.

BACKGROUND: Pemphigus is a chronic autoimmune blistering disease of the skin and mucosa severely impairing patients' health-related quality of life (HRQoL). To date, no studies have measured subjective well-being in terms of life satisfaction in pemphigus. Our main objective was to evaluate satisfaction with life in patients with pemphigus, and to analyse its relationship with clinical severity and HRQoL. METHODS: A cross-sectional survey was carried out enrolling 77 patients with pemphigus. Subjective well-being was measured using the Satisfaction with Life Scale (SWLS). HRQoL was assessed by the Dermatology Life Quality Index (DLQI) and EQ-5D-5L. Disease severity was measured by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). RESULTS: Mean ABSIS, DLQI, EQ-5D-5L and SWLS scores of patients were 11.7 (SD 17.3), 5.4 (6.8), 0.84 (0.22) and 4.76 (SD 1.52), respectively. The proportion of patients indicating extreme dissatisfaction, dissatisfaction, slightly below average in life satisfaction, average satisfaction, high satisfaction and very high satisfaction with life was 6 (7.8%), 5 (6.5%), 14 (18.2%), 16 (20.8%), 21 (27.3%) and 15 (19.5%), respectively. Life satisfaction was independent from age, gender, level of education and type of disease. A path analysis revealed that there was no direct relationship between ABSIS and SWLS (beta = - 0.09; p = 0.428); however, the following indirect path was confirmed: ABSIS → DLQI → EQ-5D-5L → SWLS. CONCLUSIONS: Disease severity and HRQoL measures regularly used to assess patients' health status may be complemented with a measure of subjective well-being, such as SWLS, to achieve a more holistic assessment of patients' lives and optimise pemphigus care.

Individuals With the Rare Disease Pemphigus: A Quest for Diagnostic.

This qualitative study conducted in France of "individuals living with a pemphigus" (ILPs; N = 54) highlights the taxing diagnostic trajectory of those suffering from these rare autoimmune diseases. Beyond enduring a diagnostic period that may prove long, during their numerous medical appointments, these individuals internalize the expectations of the medical professionals who are treating them. In some cases, numerous inconclusive medical tests and, at times, a doctor's condescension may push the patient toward a process of renunciation. This article relates the ILPs' critiques of the medical work conducted during the trying diagnostic period.

Rituximab treatment of pemphigus in women of childbearing age: experience with two patients.

Pemphigus vulgaris is an autoimmune blistering disorder treated with systemic steroids and immunosuppressive agents. Treatment of this disorder in young women of childbearing age must take into consideration the patient's desire for pregnancy and effects of the treatment on both mother and child. We report two young women with pemphigus, initially treated with standard immunosuppressive medications, who expressed their wishes for pregnancy. The immunosuppressive agents were tapered and both patients were treated with Rituximab and IVIG, permitting discontinuation of other medications, conception and pregnancy without any oral steroids or immunosuppressive agents. Both patients maintained normal pregnancies and delivered healthy babies, supporting the use of this treatment early in the disease course of this population.

Valuation of pemphigus vulgaris and pemphigus foliaceus health states: a convenience sample experiment.

BACKGROUND: Health-related quality of life (HRQoL) in pemphigus has been widely investigated; nevertheless, utility values for economic evaluations are still lacking. OBJECTIVES: To estimate health utilities for hypothetical pemphigus vulgaris (PV) and pemphigus foliaceus (PF) health states in a general population sample. METHODS: Three health states (uncontrolled PV, uncontrolled PF and controlled pemphigus) were developed based on a systematic literature review of HRQoL studies in pemphigus. Utilities were obtained from a convenience sample of 108 adults using a visual analogue scale (VAS) and 10-year time trade-off (TTO). Lead-time TTO was applied for health states regarded as worse than dead with a lead time to disease time ratio of 1 : 1. RESULTS: The mean VAS utility scores for PV, PF and controlled pemphigus were 0·25 ± 0·15, 0·37 ± 0·17 and 0·63 ± 0·16, respectively. Corresponding TTO utilities were as follows: 0·34 ± 0·38, 0·51 ± 0·32 and 0·75 ± 0·31. Overall, 14% and 6% judged PV and PF as being worse than dead. For both VAS and TTO values, significant differences were observed between all health states (P < 0·001). VAS utilities were rated significantly lower compared with TTO in each health state (P < 0·001). CONCLUSIONS: This is the first study that reports health utility values for PV and PF. Successful treatment of pemphigus might result in significant utility gain (0·24-0·41). These empirical findings with respect to three health states in pemphigus may serve as anchor points for further utility studies and cost-effectiveness analyses.

Psychiatric morbidity and pemphigus: a review of theories and literature on pathogenesis.

Skin diseases are very common in the community and a large number of general practice visits are due to dermatological problems. Most dermatologists believe that psychiatric problems are frequent among subjects coming to their attention and several studies on dermatological patients have revealed that they prevalently suffered from psychiatric disorders. However, there are only few articles in the literature dealing with stress-induced pemphigus or psychiatric troubles associated with pemphigus. The relationship between pemphigus and psychiatric disorders is still a matter of debate. The first question is whether the association is circumstantial or causal. Subsequently, it should be clarified if psychogenic factors can be considered an inducing factor for pemphigus onset, or a possible complication of this skin disease, or a comorbidity of it, or, in the end, an adverse reaction to conventional therapy for pemphigus. In any case, further studies are needed to investigate the underlying mechanism linking psychiatric diseases and pemphigus.

Burden of disease during quiescent periods in patients with pemphigus.

BACKGROUND: Studies conducted using different tools have invariably observed that physical and mental components of health status are seriously compromised in patients with pemphigus. An improvement in quality of life (QoL) has been commonly observed over the treatment period. OBJECTIVES: The aim of the study is to verify whether the patients' wellbeing is affected by pemphigus also in absence of cutaneous and mucosal lesions. MATERIALS AND METHODS: The clinical records of 203 patients were analysed. A total of 47 patients were without bullae/erosions and reported a score = 0 for both the Patient Global Assessment and the Ikeda index. In order to assess the QoL we used the Skindex-17 and the 12-item General Health Questionnaire (GHQ-12). RESULTS: Patients without bullae/erosions had a better QoL when compared with patients with active lesions. This difference, with a reduction of approximately 30% of the Skindex-17 scores in the patients without lesions, was statistically significant, for both the symptoms and the psychosocial scales. The proportion of patients at risk of anxiety/depression (GHQ-positive cases) was 44% lower in patients without lesions compared with patients with lesions. In a multiple linear regression model the presence of bullae/erosions negatively influences QoL with an average increase of Skindex-17 symptoms and psychosocial scale scores of 11·7 and 10·6 points, respectively. Female patients had a statistically significantly worse QoL than males on the symptoms but not on the psychosocial Skindex-17 scales. CONCLUSIONS: While patients without lesions reported a better QoL than patients with bullae/erosions, their Skindex-17 scores remained elevated. Dermatologists should be aware that a clearing of the skin manifestations does not mean 'perfect health' for the patient.

Depression in patients with pemphigus: is it a major concern?

It is suggested that some dermatological diseases due to their chronicity, impact on the body image, unlikelihood of complete recovery and frequent recurrences are one of the major predisposing factors towards depression. Therefore, we aimed to evaluate the rate and level of depression among pemphigus vulgaris and pemphigus foliaceus patients, two of the most common causes of hospitalization in dermatology units. This research was conducted on 55 patients with active pemphigus vulgaris and pemphigus foliaceus referring to pemphigus clinics or admitted as inpatients to the dermatology ward of Qaem and Imam Reza hospitals, Mashhad, Iran, from April 2008 to September 2009. The research tool was the Beck Depression Inventory. Collected data was analyzed by χ(2)-test Student's t-test. Twenty-six (47.3%) patients were female and 29 (52.7%) were male. The mean age was 42.34 ± 18.98 years. The prevalence rate of clinical depression was 28% in pemphigus vulgaris and 20% in pemphigus foliaceus cases. Depression prevalence showed no significant difference between these two groups (P = 0.873). In conclusion, pemphigus patients are at risk for mild depression.

Psychiatric morbidity in pemphigus and psoriasis: a comparative study from India.

AIM: This study was designed to examine the prevalence of psychiatric morbidity and its psychosocial and psychopathological correlates in patients with pemphigus in comparison to those with psoriasis. MATERIALS AND METHODS: Patients with pemphigus (n=50), group matched for demography, with those with psoriasis (n=30), and healthy controls (n=30), were subjected to cross-sectional assessment for duration, severity, and impact of dermatological disorder, attitude to appearance, social support, coping strategies, disability, quality of life, and psychiatric morbidity and diagnosis. RESULTS: The pemphigus group recorded the psychiatric morbidity rates at 40% by GHQ-12 and 26% by ICD-10; the ICD-10 diagnoses included adjustment disorder (16%), depressive episode (8%), and acute and transient psychosis (2%). This comorbidity was not very different from that of the psoriasis group at 46.7% by GHQ-12 and 36.7% by ICD-10; the ICD-10 diagnoses including adjustment disorder (13.3%), depressive episode (10.0%), alcohol dependence (6.6%), paranoid schizophrenia (3.3%), and delusional disorder plus severe depressive episode with psychotic symptoms (3.3%). The pemphigus group scored higher on disability, despite the dermatological severity and psychosocial profile being similar. Dermatological severity, psychopathology, and certain psychosocial variables were correlated in the pemphigus group, as also in the psoriasis group. CONCLUSIONS: The high psychiatric and psychosocial morbidity in pemphigus and other chronic and severe dermatologic disorders indicates a need for more studies on the psychosocial aspect of these disorders and for sensitization by the dealing physicians with this aspect.

Reliability and validity of the Chinese version of the chronic oral mucosal diseases questionnaire.

INTRODUCTION: The aim of this study was to evaluate the reliability and validity of the Chinese version of the Chronic Oral Mucosal Diseases Questionnaire (COMDQ). METHODS: A consecutive sample of 200 patients with chronic oral mucosal diseases was enrolled to complete the questionnaire after its translation and cross-cultural adaptation. The reliability of the Chinese version of COMDQ was determined through internal consistency and test-retest methods. The construct validity of COMDQ was analysed by exploratory factor analysis (EFA). RESULTS: Cronbach's alpha value for the total COMDQ score was 0.894, and the test-retest intraclass correlation coefficient value for the total COMDQ score was 0.83. The EFA extracted four factors, which accounted for 67.89% of the variance. All items showed adequate factor loadings, ranging from 0.487 to 0.947. CONCLUSION: The results suggest that the Chinese version of the COMDQ has satisfactory psychometric properties and is applicable to patients with chronic oral mucosal diseases in China.

[Prolonged corticosteroid-therapy and anxiety-depressive disorders, longitudinal study over 12 months]. / Corticothérapie prolongée et troubles anxieux et dépressifs. Étude longitudinale sur 12 mois.

UNLABELLED: To date, there is little data in the literature describing the anxiety and depressive disorders iatrogenic to corticosteroids. These disorders are common, underestimated, with potentially serious consequences that may jeopardize the patient's prognosis; their management is not consensual. OBJECTIVES: The objective of our work is to determine the prevalence of anxiety and depressive disorders induced by corticosteroids, assessing their accountability to the corticosteroids and studying their risk factors. METHODS: We conducted a prospective longitudinal study over 12months evaluating the prevalence of anxiety and depressive disorders in patients followed for chronic skin diseases treated with prolonged corticosteroid-therapy. Our patients were assessed using standardized instruments: the Mini International Neuropsychiatric Interview (MINI), the Hamilton Anxiety Scale (HAS) and the Beck Depression Inventory (BDI). RESULTS: Of 54 patients included, our study showed a high prevalence of anxiety and depressive disorders estimated at 27%. These disorders were divided into depressive disorder in 16% of cases, and anxiety disorder in 11% of cases. The early onset of these disorders was found during the first weeks of treatment. According to the Beck Depression Inventory (BDI), depression was moderate in 67% of cases; severe with suicide attempts in 22% of cases, and mild in 11% of cases. According to the Hamilton Anxiety Scale (HAS), anxiety was mild in 33% of cases and moderate in 67% of cases. The disorders observed were mainly distributed into: 33% deep pemphigus, 27% lupus, 13% bullous pemphigoid and 13% dermatomyositis. In this study the statistically significant risk factors are dose of corticosteroids and personal psychiatric history of the patient; in addition, there is a high prevalence of disorders in patients whose age exceeds 40 years, female gender, and patients treated for deep pemphigus. The evolution after pharmacological treatment and supportive psychotherapy was favorable in most patients. CONCLUSION: The psychiatric examination prior to prescription of long-term corticosteroid-therapy use should be standard practice to identify patients at risk, discuss the treatment modalities, and provide comprehensive care.

Quality of life in patients with pemphigus receiving adjuvant therapy.

BACKGROUND: Pemphigus has a strong effect on patients' quality of life (QOL). AIM: To analyze QOL and psychological well-being within patient groups, subdivided according to their different adjuvant treatments. METHODS: All adult patients with pemphigus enrolled in the study were assessed using the Short Form (SF)-36, the Skindex-29, and the General Health Questionnaire (GHQ)-12 for health status, effect of dermatology-specific aspects, and the presence of psychological comorbidity, respectively. The study population was subdivided into the following treatment groups: (i) those who were untreated or were treated only with corticosteroids (CS) at a dose of ≤ 5 mg/day (no adjuvant treatment, NAT); and patients receiving or not receiving CS ≤ 5 mg/day who also received either (ii) azathioprine (AZ), (iii) cyclophosphamide (CY), (iv) mycophenolate mofetil (MM) or (v) rituximab (RTX). RESULTS: In total, 113 patients were recruited. There were no significant differences between the treatment subgroups in either the SF-36 or Skindex-29 results. However, for the GHQ, there were large differences in QOL scores between patients scoring > 4 points (GHQ+) and those scoring < 4 points (GHQ-), especially for the more 'physical' components of QOL. The overall observed proportion of GHQ+ patients was 33.6%. CONCLUSIONS: We found no significant differences in QOL impairment between the treatment subgroups; however, we observed a strong association between psychiatric morbidity and poorer QOL within each of the treatment groups. This should be of concern for dermatologists, as psychiatric morbidity is associated with poor treatment adherence and dissatisfaction with care.

Further reliability and responsiveness of the Chronic Oral Mucosal Diseases Questionnaire.

OBJECTIVE: To test the reliability and responsiveness of the Chronic Oral Mucosal Diseases Questionnaire (COMDQ), in measuring the quality of life (QofL) in patients with chronic oral mucosal conditions. METHODS: A random sample of 160 patients with the following chronic oral mucosal conditions, recurrent aphthous stomatitis, oral lichen planus, the more common vesiculobullous conditions (mucous membrane pemphigoid and pemphigus vulgaris) and orofacial granulomatosis received a copy of the COMDQ. A subset of 100 patients received the questionnaire on two further occasions, 2 weeks and 3 months later. Statistical tests were carried out to evaluate the test-retest reliability and responsiveness of this instrument. RESULTS: This study has demonstrated that the COMDQ has good test-retest reliability with an intraclass correlation coefficient of 0.81 and is responsive to changes in the patients' overall conditions. CONCLUSION: In conclusion, this study has further demonstrated the reliability and responsiveness of the COMDQ in assessing QofL in patients with chronic oral mucosal diseases.

Quality of life and psychological status of patients with pemphigus vulgaris using Dermatology Life Quality Index and General Health Questionnaires.

Pemphigus vulgaris with painful chronic blisters and/or erosions on skin and mucosa can impair quality of life (QOL). Therapeutic modalities in the long run can have additional negative impact. There are few studies that have focused on QOL of such patients except in treated cases. The aim of this study was to describe the effect of the disease per se on QOL before receiving treatment and evaluation of psychological status of the patients and its effect on their QOL. A total of 61 patients with newly diagnosed non-treated pemphigus vulgaris participated in the study. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate their QOL and the 28-item General Health Questionnaire (GHQ-28) for their psychological status. In this study, the mean DLQI score was 10.9 ± 6.9. QOL was worse in patients with nasal and pharynx involvement, with positive Nikolsky sign, patients with severe skin involvement and those who showed the symptom of itching. There was a negative correlation between DLQI score and duration of the disease. More than 77% of patients experienced anxiety and depression with more impaired QOL. In conclusion, pemphigus vulgaris is responsible for great alteration in QOL, especially in its severe form. The disease in its initial stage may have greater impact on the QOL. The high probability of anxiety and depression in these patients and its negative effect on QOL should be taken into account in the management of these patients right from the start of the treatment.

Stress and serum TNF-alpha levels may predict disease outcome in patients with pemphigus: a preliminary study.

The aim of the current preliminary case-control study was to estimate the initial serum levels of tumor necrosis factor alpha (TNF-alpha) in case patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) and correlate them with history of stress, body surface area (BSA) affected, disease severity, and disease outcome. Ten PV and 4 PF case patients as well as 7 healthy matched controls had their serum levels of TNF-alpha measured by an enzyme-linked immunosorbent assay. Case patients were treated and followed up for 2 months. A statistically significant elevation in serum levels of TNF-alpha in PV case patients compared with controls and in PV case patients compared with PF case patients was detected (P < .05), with no significant difference between PF case patients and controls (P > .05). No significant correlation was detected between the serum levels of TNF-alpha and the BSA affected (P > .05). Four PV case patients had a bad disease outcome, of which 3 had severe emotional stress a month prior to the onset of the attack. All 4 showed significantly elevated initial serum levels of TNF-alpha compared with those who had a good disease outcome (P < .05). Emotional stress is a factor affecting prognosis of the disease. Pretreatment assessment of serum TNF-alpha levels in patients with pemphigus may be a guide to the expected prognosis and selection of the proper treatment regimen.