EuroGuiderm guideline on lichen sclerosus-introduction into lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

EuroGuiderm guideline on lichen sclerosus-Treatment of lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

Serum autoantibody reactivity in bullous pemphigoid is associated with neuropsychiatric disorders and the use of antidiabetics and antipsychotics: a large, prospective cohort study.

BACKGROUND: Bullous pemphigoid (BP), the by far most frequent autoimmune blistering skin disease (AIBD), is immunopathologically characterized by autoantibodies against the two hemidesmosomal proteins BP180 (collagen type XVII) and BP230 (BPAG1 or dystonin). Several comorbidities and potentially disease-inducing medication have been described in BP, yet a systematic analysis of these clinically relevant findings and autoantibody reactivities has not been performed. OBJECTIVE: To determine associations of autoantibody reactivities with comorbidities and concomitant medication. METHODS: In this prospective multicenter study, 499 patients diagnosed with BP in 16 European referral centers were included. The relation between anti-BP180 NC16A and anti-BP230 IgG ELISA values at the time of diagnosis as well as comorbidities and concomitant medication collected by a standardized form were analysed. RESULTS: An association between higher serum anti-BP180 reactivity and neuropsychiatric but not atopic and metabolic disorders was observed as well as with the use of insulin or antipsychotics but not with dipeptidyl peptidase-4 (DPP4) inhibitors, inhibitors of platelet aggregation and L-thyroxine. The use of DPP4 inhibitors was associated with less anti-BP180 and anti-BP230 reactivity compared with BP patients without these drugs. This finding was even more pronounced when compared with diabetic BP patients without DPP4 inhibitors. Associations between anti-BP180 and anti-BP230 reactivities were also found in patients using insulin and antipsychotics, respectively, compared with patients without this medication, but not for the use of inhibitors of platelet aggregation, and L-thyroxine. CONCLUSION: Taken together, these data imply a relation between autoantibody reactivities at the time of diagnosis and both neuropsychiatric comorbidities as well as distinct concomitant medication suggesting a link between the pathological immune mechanisms and clinical conditions that precede the clinically overt AIBD.

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. OBJECTIVES AND METHODOLOGY: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. RESULTS: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. CONCLUSIONS: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.

[Dermatitis herpetiformis and other forms of wheat sensitivity]. / Dermatitis herpetiformis und sonstige Formen der Weizensensitivität.

BACKGROUND: Wheat sensitivity is a collective term for several, especially gastrointestinal, diseases that occur as part of a hypersensitivity reaction after wheat consumption. The symptoms, which are mostly similar to those of irritable bowel syndrome, are often accompanied by skin lesions. In addition to celiac disease and dermatitis herpetiformis, the cutaneous manifestation of celiac disease, wheat sensitivity also includes nonceliac gluten sensitivity (NCGS), allergic nickel contact mucositis, wheat allergy, amylase-trypsin inhibitor intolerance, and fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAP) intolerance. OBJECTIVES: This review article aims to provide an overview of the clinical, especially dermatological and gastrointestinal features of the different forms of wheat sensitivity. Diagnosis and therapeutic management are also discussed. MATERIALS AND METHODS: A selective literature search was carried out with evaluation of our own clinical data. RESULTS: The skin lesions in dermatitis herpetiformis are very disease-specific. In contrast, wheat allergy often shares signs and symptoms with many other diseases. Other forms of wheat sensitivity cause primarily gastrointestinal abnormalities, but extra-intestinal manifestations can also occur. Their diagnosis is often complex and requires cross-disciplinary collaboration with experts in gastroenterology. The therapy consists of a wheat- or gluten-free diet. CONCLUSIONS: Knowledge of the different and frequently occurring dermatological signs of wheat sensitivity is of great importance, because dermatological manifestations associated with gastrointestinal pathology, intolerance reactions, and allergies appear more and more frequently.

S2k guidelines (consensus statement) for diagnosis and therapy of dermatitis herpetiformis initiated by the European Academy of Dermatology and Venereology (EADV).

INTRODUCTION: Dermatitis herpetiformis (DH) is a chronic, pruritic, gluten-induced skin disorder characterized by subepidermal granular IgA deposition and a variable degree of enteropathy identical to that seen in coeliac disease. So far, there has been no European consensus about the management of DH. METHODS: The guidelines were created by small subgroups of a guideline committee consisting of 26 specialists from various medical fields and one patients' representative. The members of the committee then discussed the guidelines and voted for the final version at two consensus meetings. The guidelines were developed under the support of the European Academy of Dermatology and Venereology (EADV) and in collaboration with the European Dermatology Forum (EDF). RESULTS: The guidelines summarize evidence-based and expert-based recommendations (S2 level) for the management of DH (see Appendix). CONCLUSION: These guidelines will improve the quality of management of DH and support dermatologists in their diagnostic and therapeutic decisions.

Ca2+ signalling is critical for autoantibody-induced blistering of human epidermis in pemphigus.

BACKGROUND: Pemphigus is a severe bullous autoimmune skin disease. Pemphigus foliaceus (PF) is characterized by antidesmoglein (Dsg) 1 IgG causing epidermal blistering; mucosal pemphigus vulgaris (mPV) by anti-Dsg3 IgG inducing erosions in the mucosa; and mucocutaneous pemphigus vulgaris (PV) by affecting both, with autoantibodies targeting Dsg1 and Dsg3. OBJECTIVES: To characterize the Ca2+ flux pathway and delineate its importance in pemphigus pathogenesis and clinical phenotypes caused by different antibody profiles. METHODS: Immunoprecipitation, Ca2+ flux analysis, Western blotting, immunofluorescence staining, dissociation assays and a human skin ex vivo model were used. RESULTS: PV IgG and PF IgG, but neither Dsg3-specific monoclonal antibody (AK23) nor mPV IgG, caused Ca2+ influx in primary human keratinocytes. Phosphatidylinositol 4-kinase α interacts with Dsg1 but not with Dsg3. Its downstream target - phospholipase-C-γ1 (PLC) - was activated by PV IgG and PF IgG but not AK23 or mPV IgG. PLC releases inositol 1,4,5-trisphosphate (IP3) causing IP3 receptor (IP3R) activation and Ca2+ flux from the endoplasmic reticulum into the cytosol, which stimulates Ca2+ release-activated channels (CRAC)-mediated Ca2+ influx. Inhibitors against PLC, IP3R and CRAC effectively blocked PV IgG and PF IgG-induced Ca2+ influx; ameliorated alterations of Dsg1 and Dsg3 localization, and reorganization of keratin and actin filaments; and inhibited loss of cell adhesion in vitro. Finally, inhibiting PLC or IP3R was protective against PV IgG-induced blister formation and redistribution of Dsg1 and Dsg3 in human skin ex vivo. CONCLUSIONS: Ca2+ -mediated signalling is important for epidermal blistering and dependent on the autoantibody profile, which indicates different roles for signalling complexes organized by Dsg1 and Dsg3. Interfering with PLC and Ca2+ signalling may be a promising approach to treat epidermal manifestations of pemphigus.

Disease burden of patients with pemphigus from a societal perspective.

Introduction: Cost-of-illness studies are widely used for healthcare decision-making; however, no such study is available in pemphigus from the societal perspective. The purpose of this analysis was to estimate annual cost-of-illness per patient with pemphigus from a societal perspective. Areas covered: Between 2014 and 2017, a multicenter, cross-sectional study was carried out. Consecutive pemphigus patients aged ≥18 years were recruited at all four university dermatology departments in Hungary. Direct and indirect costs were calculated, including costs for treatments, outpatient visits, hospital admissions, informal care, travel costs and productivity loss. Generalized linear model was used to analyze predictors of costs. Atotal of 109 patients with pemphigus enrolled with amean age of 57.1 (SD 14.8) years. Total cost per pemphigus patient was €3,995 (SD €7,526) peryear, with productivity loss (58%) and informal care (19%) accounting for the majority. Annual means of 189 and 41 working hours were lost due to absence from work and reduced productivity, respectively. Younger age and pemphigus vulgaris were associated with higher costs (p < 0.05). Expert opinion: This is the first cost-of-illness study applying the societal perspective in pemphigus. Our results indicate a substantial economic burden on society, mainly driven by productivity loss and informal care.

Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV).

BACKGROUND: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States. OBJECTIVES: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. RESULTS: The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.

Validity of EQ-5D-5L, Skindex-16, DLQI and DLQI-R in patients with hidradenitis suppurativa.

BACKGROUND: Numerous generic, skin- and disease-specific health-related quality of life (HRQoL) measures are available for patients with hidradenitis suppurativa (HS). Yet, robust psychometric evidence is lacking in many aspects of these outcome measures. OBJECTIVES: We sought to determine convergent and known-groups validity of multiple generic and skin-specific HRQoL measures and to identify predictors of impaired HRQoL in patients with HS. METHODS: Between 2017 and 2019, a multicentre cross-sectional study was carried out involving 200 consecutive HS patients. HRQoL outcomes included the EQ-5D-5L, EQ visual analogue scale (EQ VAS), Skindex-16, Dermatology Life Quality Index (DLQI) and DLQI-Relevant (DLQI-R). Disease severity was graded by HS-Physician's Global Assessment (HS-PGA) scale and the Modified Sartorius scale (MSS). RESULTS: Overall, 77%, 56%, 51%, 46% and 28% reported problems in the pain/discomfort, usual activities, anxiety/depression, mobility and self-care dimensions of EQ-5D-5L. Mean ± SD EQ VAS, DLQI and DLQI-R scores were 64.29 ± 22.68, 11.75 ± 8.11 and 12.19 ± 8.33, respectively. Skindex-16 responses indicated that the emotional burden of HS (64.55 ± 29.28) far exceeded those of functioning (49.40 ± 34.70) and physical symptoms (46.74 ± 29.36). EQ-5D-5L, EQ VAS, DLQI, DLQI-R and Skindex-16 total scores had moderate or strong correlations with each other (range: |0.487| to |0.993|), weak or moderate correlations with HS-PGA (|0.350| to |0.433|) and weak correlations with MSS (|0.324| to |0.389|). DLQI-R slightly outperformed DLQI both in terms of convergent and known-groups validity. Being female, lower education level, more severe disease and genital involvement were associated with worse HRQoL (P < 0.05). CONCLUSION: This study provides high-quality evidence that among skin-specific outcomes, the DLQI, DLQI-R and Skindex-16, and among generic instruments, the EQ-5D-5L are suitable to be used in HS patients. In future research, we recommend the use of existing well-validated HRQoL tools instead of developing new measures for each study. The development of composite measures that combine physician- and patient-reported outcomes is not supported by evidence in HS. [Correction added on 25 July 2020, after first online publication: in the Abstract section, the ± signs were missing and have been added to this version.].

DLQI-R scoring improves the discriminatory power of the Dermatology Life Quality Index in patients with psoriasis, pemphigus and morphea.

BACKGROUND: The Dermatology Life Quality Index (DLQI) rates 'not relevant' responses (NRRs) as the item on the questionnaire having no impact on the patients' lives at all. The DLQI-Relevant (DLQI-R) is a recently developed scoring that adjusts the total score of the questionnaire for the number of NRRs indicated by a patient. OBJECTIVES: To compare the discriminatory power of the original and DLQI-R scoring approaches in terms of absolute and relative informativity. METHODS: Cross-sectional data from 637 patients with morphea, pemphigus and psoriasis were used for the analyses. To assess absolute and relative informativity, Shannon's index and Shannon's evenness index were calculated for the 10 items on the questionnaire and for DLQI and DLQI-R total scores. RESULTS: Mean DLQI and DLQI-R scores of patients were 6·13 vs. 6·91. In the subset of patients with NRRs (n = 261, 41%), absolute informativity was higher with the DLQI-R scoring for all eight items with NRR options in all three conditions. The DLQI-R exhibited a better relative informativity in 8, 8 and 6 items in pemphigus, morphea and psoriasis, respectively. The DLQI-R led to an improvement in average item-level informativity in all DLQI score bands up to 20 points. Regarding total scores, the DLQI-R produced both a higher absolute and relative informativity in all three conditions. CONCLUSIONS: In patients with morphea, pemphigus and psoriasis, DLQI-R scoring improves the discriminatory power of the questionnaire by benefiting from the additional information in NRRs. DLQI-R scoring may be useful both in clinical practice and research. A scoring chart has been developed to aid physicians with scoring. What's already known about this topic? The original scoring of the Dermatology Life Quality Index (DLQI) rates 'not relevant' responses as the item of the questionnaire having no impact on the patients' lives at all. DLQI-Relevant (DLQI-R) is a new scoring developed in 2018 that adjusts the total score of the questionnaire for the number of 'not relevant' responses indicated by patients. The discriminatory power of the DLQI-R compared with the DLQI has not yet been investigated. What does this study add? In patients with psoriasis, pemphigus and morphea, DLQI-R scoring improves the discriminatory power of the questionnaire by benefiting from the additional information in 'not relevant' responses. What are the clinical implications of this work? DLQI-R scoring may help to more accurately quantify patients' health-related quality of life both in clinical practice and research. A scoring chart has been developed to aid physicians with scoring.

Patient-assigned health utility values for controlled and uncontrolled pemphigus vulgaris and foliaceus.

BACKGROUND: The assessment of health-related quality of life (HRQoL) in patients with pemphigus is now of increasing interest due to the availability of highly effective new therapies. Preference-based HRQoL values or health utilities required for medical and financial decision-making are not yet available directly from pemphigus patients. OBJECTIVE: To obtain health utility values for current health and hypothetical health states from the perspective of pemphigus patients. METHODS: A cross-sectional questionnaire survey was carried out with pemphigus patients. Disease severity was rated by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Patients were asked to evaluate their current health as well as three common hypothetical pemphigus health states [uncontrolled pemphigus vulgaris (PV), uncontrolled pemphigus foliaceus (PF) and controlled PV/PF] by using composite time trade-off (cTTO). Multiple regression was applied to explore determinants of utility values. RESULTS: Responses of 108 patients (64.8% women, mean age 57.4 years) were analysed. Mean ABSIS score was 11.6. The mean utility values for the hypothetical uncontrolled PV, uncontrolled PF and controlled PV/PF health states were 0.41, 0.52 and 0.66 with cTTO. The mean cTTO scores for current health were higher compared with the hypothetical health states (0.76; P < 0.001). Patients with higher ABSIS, worse pain intensity scores and those having a caregiver reported lower utility values for current health (P < 0.05). CONCLUSIONS: In pemphigus, HRQoL impairment expressed in utility values seems to be considerable, especially in comparison with other chronic dermatological conditions (e.g. psoriasis, atopic eczema, chronic hand eczema). These health utilities inform physicians, policymakers and funders about the overall extent of health loss in pemphigus and provide evidence to guide medical decisions and cost-effectiveness analyses of treatment strategies. Future research is needed to evaluate the caregiver burden in pemphigus.

Ex vivo confocal laser scanning microscopy for bullous pemphigoid diagnostics: new era in direct immunofluorescence?

BACKGROUND: Ex vivo confocal laser scanning microscopy (ex vivo CLSM) is a novel diagnostic method allowing rapid, high-resolution imaging of excised skin samples. Furthermore, fluorescent detection is possible using fluorescent-labelled antibodies. OBJECTIVE: To assess the applicability of ex vivo CLSM in the detection of basement membrane (BM) fluorescence in bullous pemphigoid (BP) and to compare its diagnostic accuracy with direct immunofluorescence (DIF) microscopy. METHODS: A total of 81 sections of 49 BP patients with positive DIF microscopy findings were examined using ex vivo CLSM in reflectance and fluorescence mode following staining with fluorescent-labelled IgG and C3 antibodies. RESULTS: Ex vivo CLSM showed an overall performance of 65.3% in identifying BM fluorescence in BP patients. IgG and C3 deposition along the BM was detected in 50% and 45.5% of the patients, respectively. The sensitivity of ex vivo CLSM in detecting BM fluorescence was low (IgG: 50%, C3: 45.5%), but the specificity was high (IgG: 100, C3: 90%). In addition to immunoreactivity, ex vivo CLSM could display subepidermal inflammatory cells similar to histological examination in 84% of patients. CONCLUSIONS: Basement membrane fluorescence could be identified with ex vivo CLSM in the skin sections of BP patients. Ex vivo CLSM enables simultaneous and rapid detection of histopathological and immunofluorescence findings in the same session, albeit with a lower sensitivity than DIF in detecting BM fluorescence.

[Dermatitis herpetiformis]. / Dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a genetically determined, gluten sensitive autoimmune bullous dermatosis related to celiac disease in which granular, insoluble aggregates in the papillary dermis of epidermal transglutaminase (TG3), immunoglobulin A (IgA), and fibrinogen are present. Detection of the dermal IgA-TG3 immune complex is the gold standard of diagnosis. DH develops in a subpopulation of patients with gluten sensitive enteropathy, characterized by itching, erythematous, excoriated papules showing characteristic distribution over the knees, elbows and buttocks; vesicles are rarely seen. The primary therapy of DH is a strict, lifelong gluten-free diet, and it may be necessary to temporarily give dapsone in case of severe symptoms.

[Linear IgA bullous dermatosis]. / Lineare IgA-Dermatose.

Linear IgA bullous dermatosis is a rare autoimmune blistering disease that occurs in both children and adults. Strings of pearls, crowns of jewels, rosettes and urticarial plaques can occur on the whole integument with emphasis on the face (particularly perioral area) and genitalia. Pruritus is common and may be severe. The presence of IgA deposits along the basement membrane can usually be identified using direct immunofluorescence (DIF) microscopy. The histological and clinical features of this disorder may mimic those of dermatitis herpetiformis.

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

BACKGROUND: No studies to date have employed the EuroQoL EQ-5D questionnaire to assess health-related quality of life (HRQoL) in patients with pemphigus. OBJECTIVES: To evaluate the HRQoL of patients with pemphigus by the EQ-5D and to analyse the convergent and known-groups validity of the EQ-5D in this patient population. METHODS: Between 2014 and 2017, a multicentre cross-sectional study was carried out. Outcome measures included the five-level EQ-5D (EQ-5D-5L), Dermatology Life Quality Index (DLQI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and an average pain intensity visual analogue scale (VAS) for the past 3 months. RESULTS: In total, 109 consecutive patients with pemphigus participated in the study (mean age 57 years; 64% women). Among the EQ-5D dimensions, the most problems were reported regarding pain/discomfort (50%), mobility (43%) and anxiety/depression (43%). No significant difference was found in mean EQ-5D index scores between patients with pemphigus vulgaris and those with pemphigus foliaceus (0·81 vs. 0·86, P = 0·14). The mean EQ-5D index scores of patients with limited, moderate, significant and extreme pemphigus were 0·88, 0·82, 0·72 and 0·67, respectively (P = 0·001). The number of comorbidities was associated with greater impairment in EQ-5D index scores (P < 0·001). DLQI (rs = -0·62, P < 0·001) and the average pain intensity VAS (rs = -0·59, P < 0·001) more strongly correlated with the EQ-5D index scores than did ABSIS (rs = -0·40, P < 0·001). CONCLUSIONS: This is the first study employing the EQ-5D questionnaire in pemphigus. The EQ-5D is a valid measure of HRQoL in patients with pemphigus that can be useful both in clinical practice and in economic evaluations to assess the health gains associated with new effective treatments.

Proposal of a new scoring formula for the Dermatology Life Quality Index in psoriasis.

BACKGROUND: 'Not relevant' responses (NRRs) on the Dermatology Life Quality Index (DLQI) may occur in up to 40% of patients with psoriasis. As these responses are scored as the item of the questionnaire having no impact on the patients' lives at all, it is more difficult for these patients to fulfil the DLQI > 10 criterion required by clinical guidelines to become candidates for systemic treatment including biologics. OBJECTIVES: We propose a new scoring system for the DLQI that corrects for the bias in the NRR option and test its construct validity in a sample of patients with psoriasis. METHODS: Data from 242 patients (104 of whom marked at least one NRR) from two earlier cross-sectional surveys were reanalysed. For each patient, the DLQI score was calculated in two ways: (i) according to the original scoring and (ii) by applying a new scoring formula (DLQI-R) that adjusts the total score for the number of NRRs. The construct validity of the DLQI-R was tested against the Psoriasis Area and Severity Index (PASI) and EQ-5D-3L. RESULTS: The mean DLQI and DLQI-R scores were 9·99 ± 7·52 and 11·0 ± 8·02, respectively. The DLQI-R allowed eight more patients (3·3%) to achieve the 'PASI > 10 and DLQI > 10' threshold. The results were robust when limiting the maximum number of NRRs allowed to two or three. Compared with the DLQI, DLQI-R correlated slightly better with PASI (rs = 0·59 vs. 0·57) and EQ-5D-3L index scores (rs = -0·58 vs. -0·54). CONCLUSIONS: The DLQI-R seems to be a valid scoring system for avoiding the bias in the NRR option and can help to improve patients' access to biologics.