Mental health in patients with pemphigus: an issue to worth consideration

Background:

There exists a high prevalence of psychiatric disorders in dermatological patients. Although, investigators have evaluated psychiatric aspects of the patients suffering from skin diseases; there are rare studies concerning mental health in pemphigus patients. The objective of this study was to evaluate mental health status and quality of life of newly diagnosed pemphigus patients.

Materials and Methods:

Between April 2007 and June 2008, all newly diagnosed pemphigus patients attending the outpatient clinic of a dermatological hospital were given a questionnaire comprising the GHQ-28 and DLQI to fill out.

Results:

Of 283 patients, 212 complete forms were returned. The bimodal score of GHQ ranged from 0 to 26 (Mean = 9.4) and the Likert score of GHQ ranged from 6 to 68 (Mean = 31.9). The DLQI score ranged between 0 and 30 (Mean of 13.8). A total of 157 patients (73.7%) were yielded to be possible cases of mental disorder considering GHQ-28 bimodal scores. Significant correlation was detected between the DLQI score and bimodal and Likert scoring of GHQ-28.

Conclusion:

Our study has depicted high prevalence of psychiatric comorbidity in pemphigus patients. It underlines the fact that physicians, who are in-charge of care for these patients, are in an exceptional position to distinguish the psychiatric comorbidity and to take appropriate measures.     

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.     

天疱疮和类天疱疮患者生活质量评定及影响因素分析

目的评价天疱疮和类天疱患者的生活质量,探讨影响其生活质量的相关因素。方法选择本科门诊84例大疱病患者(天疱疮44例,类天疱疮40例),并以银屑病患者作为对照组,采用世界卫生组织生活质量测定量表简表(WHOQOL-BREF)、皮肤病生活质量指数(DLQI)及简明健康调查问卷(SF-36)对寻常性银屑病、天疱疮和类天疱疮三组患者的生活质量进行评定,并对影响其生活质量的相关因素进行分析。结果天疱疮组和类天疱疮组WHOQOL-BREF总体感受得分低于银屑病组,差异有统计学意义(P0.05);天疱疮组和类天疱疮组与银屑病组DLQI总分比较,差异无统计学意义(P0.05);类天疱疮组在生理机能领域的得分低于天疱疮组,天疱疮和类天疱疮患者中皮损累及皮肤黏膜者在社会功能领域的得分低于累及皮肤者,疾病重度者在生理职能和情感职能领域的得分低于轻、中度者,有合并症者在生理机能和情感职能领域的得分低于无合并症者,差异均有统计学意义(P均0.05)。结论天疱疮和类天疱疮患者较银屑病患者生活质量低,疾病类型、皮损分布、疾病严重度和有无合并(并发)症对两种大疱病患者的生活质量有明显的影响。     

TNF‐α and IL‐10 gene polymorphisms show a weak association with pemphigus vulgaris in the Slovak population

Backround Pemphigus vulgaris is a rare chronic autoimmune disease of skin and mucous membranes, with several cytokines participating in its development. The role of their gene polymorphisms in susceptibility to the disease is, however, not fully understood. Objective The aim of our case‐control study was to investigate whether some of 22 single nucleotide polymorphisms (SNPs) in 13 cytokine genes (IL‐1α, IL‐1β, IL‐1RI, IL‐1Ra, IL‐4Rα, IL‐12, IFN‐γ, TGF‐β1, TNF‐α, IL‐2, IL‐4, IL‐6 and IL‐10) are associated with pemphigus vulgaris in the Slovak population. Methods DNA samples were obtained from 34 pemphigus vulgaris patients and 140 healthy controls of Slovak origin. Cytokine gene SNPs were determined using the polymerase chain reaction with sequence‐specific primers (PCR‐SSP) method. Results We found a weak association between pemphigus vulgaris and polymorphic variants in TNF‐α and IL‐10 genes only, with haplotypes TNF‐α–308G/–238G and IL‐10 –1082A/–819C/–592C being significantly overrepresented in pemphigus vulgaris patients (TNF‐α GG: 94.12% vs. 82.86%, P = 0.0216; IL‐10 ACC: 44.12% vs. 30.00%, P = 0.0309). Conclusions Our preliminary results suggest that certain TNF‐α and IL‐10 gene polymorphisms might contribute to genetic susceptibility to pemphigus vulgaris; however, their overall impact on disease development will be rather limited.     

Quality of Life Assessment in Korean Patients with Pemphigus

Background

Measuring the quality of life (QOL) is important in the evaluation of nonclinical aspects of diseases, for the discovery of functional and psychological limitations, and in choosing treatment in the initial phase of the disease. Pemphigus is a potentially fatal autoimmune bullous disease caused by autoantibodies against desmogleins (cadherin family proteins in desmosomes). Thus far, there has been no published study on QOL in Korean patients with pemphigus.

Objective

To study the impact of pemphigus on the QOL in a large number of Korean patients.

Methods

Sixty-six patients enrolled at the Gangnam Severance Hospital from March 2012 to March 2013 were assessed for QOL by using the Dermatology Life Quality Index (DLQI), and for anxiety and depression by using the General Health Questionnaire (GHQ). Spearman''s rank-order correlation, t-test, and ANOVA were used to identify the relations between the DLQI score and other clinical variables.

Results

Pemphigus vulgaris and pemphigus foliaceus significantly reduced the QOL of patients. The average DLQI score for all patients was 10.18. The mean DLQI score was 13.45 in patients in the active state and 5.15 in the patients in the remission state. The DLQI score highly correlated with disease severity, titer of anti-desmoglein 1 in enzyme-linked immunosorbent assay, and the corticosteroid dose. However, the QOL was not affected by sex, age, subtype of pemphigus, duration of disease, or comorbidities. Forty-two percent of the patients showed a positive result in the GHQ, reflecting probable minor psychiatric nonpsychotic conditions, and the GHQ score positively correlated to the DLQI score.

Conclusion

Pemphigus significantly impairs the QOL of patients. The QOL of Korean pemphigus patients significantly correlates with clinical severity. Therefore, considerable attention should be paid to the patients'' QOL and psychological states as well as clinical status.     

Aufbau einer interdisziplinären Vulvasprechstunde

Background: Diseases of the vulva often cause severe impairment and long‐term problems for the affected women. Adequate treatment requires expert knowledge on the part of treating dermatologists and gynecologists. This was the reason for the initiation of an interdisciplinary consultation service for vulvar diseases at the University Hospital of Lübeck. Patients and methods: Over a period of 2½ years, 208 patients were seen in the new consultation service. Cases were classified as inflammatory diseases, neoplastic diseases, infectious diseases, vulvodynia, or genodermatoses. The effectiveness of treatment was documented by photography, biopsy and – whenever applicable – a quality of life assessment using the Dermatology Life Quality Index (DLQI). Results: Inflammatory dermatoses were diagnosed in 133 patients and neoplas‐tic diseases in 32 patients. Infection was diagnosed in 25 patients, vulvodynia in 8, genodermatoses in 3 and other diseases in 7. The DLQI was assessed in 140 patients. Of these, 55 patients had a DLQI > 10 (0–30), indicating severe or extreme impairment of quality of life. A follow‐up DLQI was collected in 81 patients, showing a significant improvement. Conclusions: The patients and both hospital facilities benefitted from the interdisciplinary consultation service. The initial high costs in terms of medical staff and time was compensated by the development of diagnostic and treatment algorithms. Overall, the concept received positive feedback from patients and medical staff members.     

Validity of the EQ-5D in patients with pemphigus vulgaris and pemphigus foliaceus

Pemphigus is a group of autoimmune skin diseases that cause blisters and wounds on the skin and/or mucous membranes such as inside the mouth. It is an uncommon disease with around 15 new cases yearly per million people. The two most common clinical forms are pemphigus vulgaris and foliaceus. Pemphigus can still be life threatening, and the symptoms may cause significant difficulties for patients in their everyday life. This study, from Hungary, aimed to assess the health status and quality of life in patients with pemphigus vulgaris and foliaceus. The authors surveyed 109 pemphigus patients, and collected data on demographics and clinical characteristics. Quality of life was assessed by the EQ-5D general health status questionnaire. Overall, 50%, 43%, 43%, 42% and 19% of pemphigus patients reported problems regarding pain/discomfort, mobility, anxiety/depression, usual activities and self-care, respectively. Patients having skin and mucosal symptoms, a larger number of comorbidities (other diseases occurring alongside pemphigus) and more severe disease experienced greater impairment of quality of life. No difference was found in quality of life scores between pemphigus vulgaris and foliaceus patients or between females and males. This study demonstrates that the EQ-5D questionnaire is an accurate measure of quality of life in pemphigus patients. The EQ-5D questionnaire allows comparisons between patients across a broad range of disease areas and people without those diseases. The EQ-5D scores reported in this study can be useful for cost-effectiveness analyses of new pemphigus treatments such as rituximab, and might help to improve patients’ access to more effective medicines, in a condition for which only limited treatment options have been available so far.     

Pemphigus

Pemphigus is a group of organ-specific autoimmune mucocutaneous disorders with an established immunologic basis. The presence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous membranes characterize its three major variants, pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Prior to the use of corticosteroids in the 1950s, the natural history of pemphigus vulgaris was relentless progression, with a 50% mortality at 2 years, and almost 100% at 5 years. Today, with mortality rates less than 5%, the focus has changed towards reducing corticosteroid side effects and maintaining optimal quality of life under treatment. This can be achieved by the appropriate use of steroid-sparing agents. This article addresses the comprehensive management of patients with pemphigus.     

Health‐related quality of life among Darier’s disease patients

Background Darier’s disease (DD) is an autosomal dominant skin disorder characterized by persistent eruption of hyperkeratotic papules. The effect of DD on quality of life (QOL) has been assessed in only one study, which found no correlation between the Dermatology Life Quality Index (DLQI) score and clinical severity of the disease. The correlation between health‐related quality of life (HRQL) and other diseases and patient characteristics has not been studied. Objectives To examine the HRQL of patients with DD and to evaluate the association between HRQL scores and disease and patient characteristics. Methods A total of 74 DD patients completed three QOL questionnaires: DLQI, EQ‐5D, and one specially designed for the study. The data reported in this study were collected as part of a larger study on the clinical characteristics of DD; the socio‐demographic and clinical data were used in the statistical analysis of the current study. Results Mean DLQI was 5.41 ± 5.57 and the mean EQ‐Visual Analogue Scale (VAS), was 70.84 ± 19.25. DLQI and EQ‐VAS were significantly associated with skin area affected, disease severity, age at onset of DD and a seborrhoeic distribution pattern of DD. Stepwise linear regression showed skin area affected to be the most significant variable in the predication of DLQI (beta = 0.183; SE = 0.04; P < 0.001), and disease severity the most significant variable in the predication of EQ‐VAS (beta = ?9.15; SE = 3.21; P < 0.006). Conclusions Darier’s disease has a negative impact on HRQL of patients and the HRQL is associated with various disease characteristics, mainly skin area affected and clinical severity.     

Disease severity and quality of life in a follow-up study of patients with occupational contact dermatitis

Background Few studies have investigated how occupational contact dermatitis and its severity affect patients' quality of life (QoL). Objectives To investigate the impact of occupational contact dermatitis and its severity on patients' QoL, and to examine the association between different QoL measures. Method Patients previously diagnosed with occupational contact dermatitis completed the Short Form Health Survey (SF‐36) and Dermatology Life Quality Index (DLQI). Disease severity was rated both by the patient and by the physician. Results Of a possible 725, a total of 119 patients (16.4%) were included in the study, and, at follow‐up, just over 21% had normal skin and 18.3% had progressed to persistent dermatitis. The SF‐36 median physical component summary score was 52 [interquartile range (IQR): 43–56] and the mental component summary median score was 51 (IQR: 44–57). The correlation between patient‐rated and physician‐rated disease severity was moderate (r = 0.708, p < 0.00001). The correlation between the disease severity measures and DLQI was moderate, whereas the SF‐36 correlated poorly with both the DLQI and disease severity measures. Conclusions QoL was generally reduced in this group of patients with occupational contact dermatitis, with some correlation between severity and DLQI. The DLQI was a more sensitive measurement of QoL than the SF‐36 in this patient population. Incorporating disease severity rating with a QoL questionnaire is valuable in occupational contact dermatitis and is recommended.     

Diagnosis and clinical features of pemphigus vulgaris

Autoimmune bullous diseases are associated with autoimmunity against structural components that maintain cell-cell and cell-matrix adhesion in the skin and mucous membranes. They include those where the skin blisters at the basement membrane zone and those where the skin blisters within the epidermis (pemphigus vulgaris, pemphigus foliaceus, and other subtypes of pemphigus). The variants of pemphigus are determined according to the level of intraepidermal split formation. There are 5 main variants of pemphigus: pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus, drug-induced pemphigus, and paraneoplastic pemphigus. This review focuses only on pemphigus vulgaris.     

成人寻常型银屑病瘙痒特征及对生活质量的影响调查

【摘要】 目的 调查成人寻常型银屑病患者瘙痒的流行程度及临床特征,评估瘙痒对睡眠和生活质量的影响。方法 2020年1 - 12月在兰州大学第二医院采用医师评估和问卷调查相结合的方式,评估291例寻常型银屑病患者的瘙痒特征、睡眠及生活质量。运用二元Logistic回归分析影响瘙痒的危险因素,多元线性回归分析影响睡眠及生活质量的相关因素。结果 291例寻常型银屑病患者,男184例（63.23%）,女107例（36.77%）,年龄M（Q1,Q3）为37.00（28.00,50.00）岁。258例（88.64%）有瘙痒症状,瘙痒程度以中度多见。皮损严重程度（OR = 1.252,95% CI 1.114 ~ 1.407,P＜0.001）是患者瘙痒的主要危险因素。银屑病面积和严重程度指数与瘙痒数字评价量表（NRS）评分（r = 0.65,P＜0.001）、5-D′S评分（r = 0.65,P＜0.001）、匹兹堡睡眠质量指数量表（PSQI）评分（r = 0.48,P＜0.001）及皮肤病生活质量指数量表（DLQI）评分（r = 0.63,P＜0.001）均存在正相关,瘙痒严重程度NRS评分与DLQI评分（r = 0.61,P＜0.001）和PSQI评分（r = 0.55,P＜0.001）存在正相关,5-D′S评分与DLQI评分（r = 0.62,P＜0.001）和PSQI评分（r = 0.64,P＜0.001）均存在正相关。结论 大部分成人寻常型银屑病患者存在中等程度瘙痒;疾病严重程度显著影响患者的瘙痒严重程度,瘙痒对睡眠和生活质量有较大影响。     

Immunologic and histopathologic characterization of an active disease mouse model for pemphigus vulgaris

Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes that is caused by anti-desmoglein 3 IgG autoantibodies. Recently, we generated an active disease mouse model for pemphigus vulgaris by adoptive transfer of splenocytes from immunized desmoglein 3-/- mice to Rag2-/- mice. In this study, we performed immunologic and histopathologic studies using this pemphigus vulgaris model in mice and compared the gross and microscopic phenotypes of pemphigus vulgaris model mice and desmoglein 3-/- mice. Pemphigus vulgaris model mice showed strong in vivo IgG, and weak IgA deposition on keratinocyte cell surfaces in stratified squamous epithelia, and produced circulating anti-desmoglein 3 IgG antibodies without apparent cross-reactivity to desmoglein 1, in enzyme-linked immunosorbent assays. The predominant IgG subclass was IgG1. Pemphigus vulgaris model mice and desmoglein 3-/- mice were almost indistinguishable in terms of both gross and microscopic findings. Both types of mice showed suprabasilar acantholysis in the stratified squamous epithelia, including the oral mucous membranes and traumatized skin around the snout or paws; however, some pemphigus vulgaris model mice demonstrated a more severe phenotype than desmoglein 3-/- mice. The esophagus and forestomach were affected in some pemphigus vulgaris model mice, but not in desmoglein 3-/- mice. Furthermore, eosinophilic spongiosis, which is found in early pemphigus vulgaris lesions in patients, was observed in pemphigus vulgaris model mice but not in desmoglein 3-/- mice. Pemphigus vulgaris model mice reflect several of the histopathologic and immunologic features seen in pemphigus vulgaris patients, and provide a valuable tool to investigate the pathophysiologic mechanisms of pemphigus vulgaris.     

The health impact of pemphigus vulgaris and pemphigus foliaceus assessed using the Medical Outcomes Study 36-item short form health survey questionnaire

BACKGROUND: Pemphigus vulgaris and pemphigus foliaceus are rare, potentially life-threatening, autoimmune disorders characterized by antibodies to epidermal adhesion molecules. Clinical characteristics are painful chronic erosions of mucous membranes and of the skin. There are only few published studies on the impact of the disease on the health status (HS) of patients with these conditions. OBJECTIVES: To assess the impact of disease on the HS of patients with pemphigus. METHODS: Fifty-eight patients enrolled at the Bullous Skin Diseases Unit of IDI-IRCCS in the period January-June 2006 were assessed for their HS using the Medical Outcomes Study 36-item short form health survey (SF-36) questionnaire and for anxiety and depression using the Institute for Personality and Ability Testing questionnaires. RESULTS: A compromised HS on both the physical and the psychosocial scales was observed, similar to other chronic dermatological diseases such as psoriasis. Patients with anxiety had severe disease as measured by the Physician's Global Assessment and the degree of mucocutaneous involvement, as well as those with a more recent disease onset. A better HS was observed in patients whose clinical condition was rated as nonsevere, and also in patients with pemphigus foliaceus. In pemphigus vulgaris, antidesmoglein 3 antibody levels directly correlated with clinical severity and with lower SF-36 scores. CONCLUSIONS: HS evaluated with the SF-36 can be a very useful additional outcome criterion in clinical studies with pemphigus. The management of the disease must take into account its impact on various aspects of life of the patient.     

Costs and quality of life in patients with moderate to severe plaque‐type psoriasis in Germany: A multi‐center study

Background: This study evaluated costs, disease severity and health‐related quality of life (QoL) in patients with moderate to severe plaque‐type psoriasis. Patients and Methods: Patients with a ‘psoriasis area and severity index’ (PASI) > 12 and/or a body surface area (BSA) > 10 were enrolled in dermatological practices and hospital outpatient departments (n = 184) and the total costs of illness generated during the last 12 months were retrospectively calculated. QoL was assessed using the SF‐36 and the DLQI. Participants were stratified into three subgroups according to the treatment received during the 1 year documentation period; a) patients without and b) patients with phototherapy or standard systemic therapy, and c) patients who had failed, were intolerant or had contraindications to at least two standard systemic therapies. The study was performed before biologics became available for the treatment of psoriasis in Germany. Results: Included patients had severe skin symptoms (mean PASI 18.2) and a highly impaired QoL (mean DLQI 10.6). Total annual costs amounted to € 6,709. Patients belonging to subgroup C had the most severe skin symptoms (mean PASI 22.2), the lowest QoL (mean DLQI 12.6), the highest hospitalization rate and largest loss of productivity.These patients produced the highest total costs of 8.831 €/y. Conclusions: Patients who cannot (or can no longer) be adequately managed with standard treatments are characterized by high disease activity, high costs and reduced QoL. Improved treatment options particularly for these patients are medically necessary and appear economically sensible.     

Occupational contact dermatitis has an appreciable impact on quality of life

In this study, we examined the impact of occupational contact dermatitis on quality of life. 181 patients, diagnosed with occupational contact dermatitis over a period of 3 years (1996–1999 inclusive), were sent a questionnaire based on the Dermatology Life Quality Index (DLQI) and the Short Form‐36 (SF‐36). 60 (32%) patients were in industrial occupations and 27 (14%) in health care. An overall response rate of 39% (n=70) was obtained. The median DLQI score was 5, with a mean score 6.6 (SD 6.4), which is similar to that seen in Behçet’s syndrome and urticaria. There was no statistically‐significant difference between male and female median scores (p=0.98) and no significant correlation between age and DLQI score nor between DLQI score and time from diagnosis. The most problematic quality of life areas were symptoms and feelings. Males scored highest in problems associated with work, relationships and treatment, whereas females scored highest in problems associated with symptoms and feelings, daily activities and leisure. The SF‐36 scores showed an association between physical problems and emotional problems affecting work. From this study, it can be seen that occupational contact dermatitis has an appreciable impact on quality of life.     

Pemphigus vulgaris: environmental factors. Occupational, behavioral, medical, and qualitative food frequency questionnaire

BACKGROUND: There have been reports suggesting the involvement of environmental factors in the disease process of pemphigus. Factors suggested include exposure to pesticides or certain drugs. OBJECTIVE: To analyze the association of pemphigus with environmental exposure to various agents, including smoking, recreational and occupational insults, drugs, and food. DESIGN AND SETTING: In-person interviews of pemphigus patients and control subjects were conducted by trained medical investigators using a structured questionnaire. Questions included occupational, behavioral, medical, and qualitative food frequency details. The multicenter study was conducted at outpatient services of teaching hospitals in Bulgaria, Brazil, India, Israel, Italy, Spain, and the USA. PARTICIPANTS: A total of 126 pemphigus patients (55 men, 71 women; age, 54 +/- 17 years) and 173 healthy controls (87 men, 86 women; age 50 +/- 19 years) were interviewed in the period between October 1, 1999 and March 31, 2000. The diagnosis of pemphigus was based on clinical, histologic, immunohistologic, and immunohistochemical criteria. The disease duration was 2-27 years (8.4 +/- 7.2 years). Individuals with skin diseases other than pemphigus were selected as control subjects. MAIN OUTCOME MEASURE: Information on drugs, foods, and occupational, environmental, constitutional, and other possible risk factors was analyzed by t-tests and chi-squared tests as applicable. A multivariate logistic regression model was applied to the data to study simultaneously the independent relationship between each risk factor and pemphigus vulgaris. RESULTS: The risk for pemphigus vulgaris was lower for ex-smokers and current smokers than for patients who had never smoked. Exposure to pesticides and occupational exposure to metal vapor were associated with an increased risk of pemphigus. Pemphigus patients had more pregnancies than controls. There were differences in environmental factors between countries, with exposure to gardening materials and pesticides being highest among patients from Bulgaria, followed by Israel. Disease characteristics also exhibited differences between countries. Bulgarian patients less frequently had oral mucous membrane lesions: 66% compared to 92% for Israeli patients and 83% for Italians. The distribution of the disease in skin and mucous membranes was similar among patients from all countries. Exclusive skin involvement was seen in 50% of patients, mucous membranes alone in 23% of patients, and both skin and mucous membranes in 27% of patients. CONCLUSIONS: The beneficial effect of smoking on pemphigus might be explained by its effect on the immune system. In addition, smoking has an antiestrogenic effect, while pesticides have an estrogenic effect. The lower numbers of smokers among patients, the higher exposure rates to pesticides, and the higher number of female patients who had been pregnant may point to the contribution of estrogens to the disease process. It remains to be determined whether measures, such as avoiding exposure to pesticides or metal vapor, may be beneficial in the clinical context. As the present study was a survey, more definitive studies should be conducted to validate the results.     

Klinik und Immunpathologie bei 48 Patienten mit Pemphigus

Background and Objective. Pemphigus is a rare intraepidermal autoimmune bullous disease. Two major variants, pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are distinguished. The aim of this study was to document the clinical and immunpathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Würzburg over the past 10 years. Patients/Methods. Based on a retrospective study, clinical and immunpathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunfluorescence microscopy. Results. Between January 1989 and August 1998, 48 patients were diagnosed with pemphigus at our institution; 31 patients had PV and 17 PF. The average age (±standard deviation) of PV patients was 55 (±17) and of PF patients 60 (±12) years. All PV patients showed involvement of mucous membranes and in 65% of cases, the skin was also involved. In contrast, PF patients had involvement only of the skin. By direct immunfluorescence microscopy, intercellular deposits of IgG and C3 were detected in 89% and 78% of PV cases, respectively. In PF, intercellular deposits of IgG were found in 94% and of C3 in 75% of cases. By indirect immunfluorescence microscopy on monkey esophagus, 94% of the PV and 88% of the PF patients revealed circulating serum antibodies. In 30 patients, we characterized the immune response by ELISA using recombinant desmoglein 1 and 3. All PF sera showed autoantibodies against desmoglein 1 and all PV sera against desmoglein 3. In PV with both mucous membrane and skin involvement, antibodies to both desmoglein 3 and 1 were detected. Conclusions. Our results confirm the correlation of the autoantibody profile with the clinical phenotype of pemphigus.     

Cost‐effectiveness of psoriasis therapy with etanercept in Germany

Background: We estimated the cost‐effectiveness of intermittent therapy with etanercept in patients with moderate‐to‐severe plaque‐type psoriasis in comparison to non‐systemic therapy in Germany. Patients and Methods: We performed a cost‐utility analysis using the endpoint costs per quality‐adjusted life year gained (costs/QALY). For this purpose, we adapted a UK‐based Markov model by means of resource use data that we derived from a German cost study. Efficacy data, information on frequency of adverse events and changes in quality of life were derived from three pooled clinical trials. We extrapolated the further course of the disease and its treatment over a 10 year course. Results: For patients with an initial Psoriasis Area and Severity Index (PASI) > 10 and a Dermatology Life Quality Index (DLQI) > 10 the incremental cost‐effectiveness ratio (ICER) for etanercept compared to non‐systemic therapy was 45,491 €/QALY. For patients with PASI and DLQI > 15 costs/QALY were 32,058 € and among patients with severe plaque psoriasis (DLQI and PASI > 20) 18,154 € . Conclusions: According to internationally accepted levels of cost‐effectiveness thresholds, the intermittent treatment of (moderate to) severe plaque‐type psoriasis with etanercept is a cost‐effective measure within the German healthcare system.     

The impact of acne vulgaris on the quality of life and psychologic status in patients from upper Egypt

Background  Acne vulgaris is a common skin disease. Although neither life threatening nor physically debilitating, acne can severely affect social and psychologic functioning.
Aim  To study the overall impact of acne vulgaris on the quality of life and psychologic functioning of patients in Egypt.
Methods  One hundred and fifty patients with acne vulgaris and 50 healthy controls were evaluated. Acne patients were evaluated using the Dermatology Life Quality Index (DLQI), Culture Free Self-Esteem Inventory – Adult Version (CFSEI-AD), and Symptom Check List-90 – Revised (SCL-90-R). The controls were evaluated using SCL-90-R and CFSEI-AD.
Results  The mean DLQI scores of male acne patients were found to be higher than those of female patients with regard to symptoms and feelings, daily activities, personal relationships, and treatment; however, the differences were significant for leisure ( P  = 0.005) and total score ( P  = 0.022) only. The CFSEI-AD score of patients was significantly lower than that of controls ( P  = 0.001). Acne patients had significantly higher scores than controls in all items of the SCL-90-R. Significant positive correlations were found between the duration and severity of illness and DLQI and SCL-90-R scores. There was a significant negative correlation between the duration and severity of acne and CFSEI-AD scores.
Conclusion  Acne vulgaris has a significant effect on the quality of life. Our results highlight the importance of recognizing psychiatric comorbidity in acne patients.