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Background There are various topical and systemic treatment options for the management of lichen planus. However, it is often difficult to achieve long-term disease control and many of the common therapies may be associated with unwanted side effects. Aims To evaluate the effectiveness of 8 mg oral methylprednisolone administered daily in lichen planus by the analysis of medical records. Methods In this retrospective cohort study, we compared the rates of improvement between two groups of patients. The first group received 8 mg oral methylprednisolone daily for at least one month. In the second group, patients with similar parameters to the first group (age, sex, disease manifestation) but without systemic glucocorticoid therapy were included. Fisher’s exact test was used to compare the rates of remission in the two groups. Results In the daily oral methylprednisolone (n = 24) and no systemic corticosteroids (n = 16) groups, 23 (95.8%) and 6 (37.5%) patients achieved partial or complete remission, respectively. The frequency of improvement was significantly higher in patients who received oral methylprednisolone (P < 0.0001). Limitations Limitations of this study include its retrospective design and the relatively small sample size. Conclusion Low dose oral glucocorticoid therapy may be an effective option for the systemic treatment of lichen planus. Based on our results and previous studies, instead of higher doses, longer therapy duration with low doses should be considered.

Intravenous immune globulin (IVIG) for the treatment of dermatomyositis has not been extensively evaluated.We conducted a randomized, placebo-controlled trial involving patients with active dermatomyositis. The patients were assigned in a 1:1 ratio to receive IVIG at a dose of 2.0 g per kilogram of body weight or placebo every 4 weeks for 16 weeks. The patients who received placebo and those without confirmed clinical deterioration while receiving IVIG could enter an open-label extension phase for another 24 weeks. The primary end point was a response, defined as a Total Improvement Score (TIS) of at least 20 (indicating at least minimal improvement) at week 16 and no confirmed deterioration up to week 16. The TIS is a weighted composite score reflecting the change in a core set of six measures of myositis activity over time; scores range from 0 to 100, with higher scores indicating greater improvement. Key secondary end points included at least moderate improvement (TIS ≥40) and major improvement (TIS ≥60), and change in score on the Cutaneous Dermatomyositis Disease Area and Severity Index.A total of 95 patients underwent randomization: 47 patients were assigned to the IVIG group, and 48 to the placebo group. At 16 weeks, 79% of the patients in the IVIG group (37 of 47) and 44% of those in the placebo group (21 of 48) had a TIS of at least 20 (difference, 35 percentage points; 95% confidence interval, 17 to 53; P0.001). The results with respect to the secondary end points, including at least moderate improvement and major improvement, were generally in the same direction as the results of the primary end-point analysis, except for the change in creatine kinase level (an individual core measure of the TIS), which did not differ meaningfully between the two groups. Over 40 weeks, 282 treatment-related adverse events occurred in the IVIG group, including headache (in 42% of patients), pyrexia (in 19%), and nausea (in 16%). A total of 9 serious adverse events that were considered to be related to IVIG occurred, including 6 thromboembolic events.In this 16-week trial involving adults with dermatomyositis, the percentage of patients with a response of at least minimal improvement based on a composite score of disease activity was significantly greater among those who received IVIG than among those who received placebo. IVIG was associated with adverse events, including thromboembolism. (Funded by Octapharma Pharmazeutika; ProDERM ClinicalTrials.gov number, NCT02728752.).

Few studies have investigated health-related quality of life (HRQoL) in patients with atopic dermatitis (AD) during the COVID-19 pandemic.The objectives of this study were to compare HRQoL in adult AD patients before and during the pandemic and to assess measurement performance of 4 HRQoL measures.Between 2018 and 2021, a multicenter, cross-sectional survey was conducted, involving 218 adult AD patients. Health-related quality of life outcomes included the EQ-5D-5L, Skindex-16, Dermatology Life Quality Index (DLQI), and DLQI-Relevant (DLQI-R). Severity was measured using objective SCORing Atopic Dermatitis, Eczema Area and Severity Index, and Investigator Global Assessment.The mean ± SD EQ-5D-5L utility, Skindex-16, DLQI, and DLQI-R scores were 0.82 ± 0.22, 56.84 ± 27.46, 13.44 ± 8.46, and 13.76 ± 8.60, respectively. The patients reported more problems during the pandemic ( P0.05) regarding pain/discomfort (odds ratio [OR], 1.78), worrying (OR, 1.89), concerns about persistence/reoccurrence of disease (OR, 1.88), and social relationships (OR, 1.69). The HRQoL outcomes showed strong correlations with each other (range of rs , |0.69| to |0.99|). The Skindex-16, DLQI, and DLQI-R were able to discriminate between severity groups with large (η 2 = 0.20-0.23), whereas the EQ-5D-5L with moderate effect sizes (η 2 = 0.08-0.11).Atopic dermatitis patients experienced significantly more problems in some areas of HRQoL during the pandemic. The EQ-5D-5L, Skindex-16, DLQI, and DLQI-R demonstrated good convergent and known-group validity and can be suitable instruments for HRQoL assessment in clinical and research settings.

Background Atopic dermatitis (AD) is a common chronic inflammatory skin disorder affecting up to 10% of adults. The EQ-5D is the most commonly used generic preference-accompanied measure to generate quality-adjusted life years (QALYs) for economic evaluations. Objectives We aimed to compare psychometric properties of the three-level and five-level EQ-5D (EQ-5D-3L and EQ-5D-5L) in adult patients with AD. Methods In a multicentre cross-sectional study, 218 AD patients with a broad range of severity completed the EQ-5D-3L, EQ-5D-5L, Dermatology Life Quality Index (DLQI) and Skindex-16. Disease severity outcomes included the Investigator Global Assessment, Eczema Area and Severity Index and the objective SCORing Atopic Dermatitis. Results A good agreement was established between the two EQ-5D versions with an intraclass correlation coefficient of 0.815 (95% CI 0.758–0.859, p η2 = 0.083–0.489). Conclusion Both instruments exhibited good psychometric properties in AD; however, the EQ-5D-5L was superior in terms of ceiling effects, informativity and convergent validity. We recommend the use of the EQ-5D-5L to measure health outcomes in clinical settings and for QALY calculations in AD.

Zusammenfassung Hintergrund Die Bevölkerung erreicht ein höheres Lebensalter. Begleitend steigt die Inzidenz der Hauterkrankungen. Ziel der Arbeit Dargestellt werden die wichtigsten Hauterkrankungen geriatrischer Patienten. Material und Methoden Es erfolgten sowohl eine Literaturrecherche in der Datenbank von PubMed als auch aus dermatologischen Standardlehrbüchern. Ergebnisse Die Haut geriatrischer Patienten reagiert empfindlicher auf Umwelteinflüsse und kann im Rahmen von internistischen Grunderkrankungen mitbetroffen sein. Aufgrund von verzögerter Diagnostik werden maligne Hauterkrankungen bei alten Patienten erst in höheren Stadien diagnostiziert. Diskussion Physiologische Hautveränderungen im Alter sind durch entsprechende Pflegemaßnahmen zu behandeln. Bei unklaren Hautveränderungen ist eine rasche dermatologische Abklärung anzustreben.

Introduction: Cost-of-illness studies are widely used for healthcare decision-making in chronic conditions. Our aim was to assess the cost-of-illness of adult atopic dermatitis (AD) from the societal perspective in Hungary. Areas covered: We conducted a multicentre, cross-sectional questionnaire survey. Consecutive AD patients aged ≥18 years were recruited at dermatology departments in Hungary. We calculated direct and indirect costs, including costs for treatments, outpatient visits, hospital admissions, informal care, travel costs and productivity loss. Generalized linear model was used to analyse predictors of total, direct and indirect costs. Expert Commentary: Altogether 218 patients completed the survey (57.8% female) with an average age of 31.3 (SD=11.7). Patients’ average Dermatology Life Quality Index (DLQI) score was 13.5 (SD=8.5). According to Eczema Area and Severity Index (EASI) score, 2.3% (n=5), 21.2% (n=46), 54.4% (n=118) and 22.1% (n=48) had clear, mild, moderate, and severe AD, respectively. We found that the average total, direct medical, direct non-medical and indirect annual costs per patients were €4,331, €1,136, €747, and €2450, respectively, with absenteeism and presenteeism being the main cost drivers, accounting for 24% and 29% of the total cost of AD. A one-year longer disease duration led to, on average, 1.6%, and 4.2% increase in total and direct non-medical costs, respectively. Patients with worse health-related quality of life (higher DLQI score) had significantly higher total, direct medical, direct non-medical costs, and indirect costs. Our results indicate a substantial economic burden of AD from a societal perspective, mainly driven by productivity losses.

The authors summarize up-to-date knowledge about the rare forms of autoimmune blistering diseases, focusing on their clinical features, diferential diagnosis and the modern therapy modalities. Besides the well known pemphigus and pemphigoid diseases, the knowledge about the rare autoimmune blistering diseases has been expanded. Although at this time, there is no signifcant diference between these diseases with regard to their therapy, the diferential diagnosis is important for knowing their exact prevalences and it may lead to individual targeted therapies in the future.

In the present review, the authors briefy describe the clinical features and pathomechanism of dermatitis herpetiformis and detail the diagnostic algorithm of the disease. The up-to-date protocol for the management of the disease is presented in light of the evidence- and consensus-based (S2) guideline published by the European Academy of Dermatology (EADV) in 2021.

Linear IgA bullous dermatosis is a rare autoimmune blistering disorder, afecting both children and adults. The goal of this review is to summarize the clinical, diagnostic, and therapeutic aspects of the disease. Subepidermal, tense blisters, urticariform plaques and erosions develop on the skin, typically in a grouped pattern. Annularly arranged lesions are referred to as “cluster of jewels”, while lesions forming a linear or arched pattern are named “string of pearls”. Mucosal involvement is also present in some cases, and scarring may lead to permanent complications. Specifc autoimmune diagnostic tests are essential for the defnitive diagnosis. The frst line treatment is dapsone and the disease shows a benign course in most cases.

Összefoglaló. A könnyűlánc-amyloidosis ritka, multidiszciplináris jelentőségű kórkép, melynek hátterében az esetek döntő hányadában egy amyloidogen fehérje, a csontvelő kóros plazmasejtjeiben termelődő monoklonálisimmunglobulin-molekula lambda típusú könnyűláncának felszaporodása áll. A klinikai tünetek az érintett szervek függvényében igen változatosak és gyakran nem specifikusak, ezért a betegség sok esetben későn kerül felismerésre. A diagnózis felállításának alapfeltétele a szövettani vizsgálat elvégzése és a kóros fehérjelánc kimutatása. A betegség jellegzetes alarmírozó bőrtüneteinek helyes értékelése fontos szereppel bír a korai diagnózisalkotásban. A jelen közlemény egy myeloma multiplexhez társult könnyűlánc-amyloidosis esetét mutatja be. A betegnél a pathognomicus, típusos bőrgyógyászati tünetek (periorbitalis, axillaris és inguinalis lokalizációjú petechiák, purpurák, ecchymosisok, suffusiók és viaszsárga papulák) mellett szív- és veseérintettség is igazolódott. Az alkalmazott ciklofoszfamid-, bortezomib- és dexametazonkezelési séma hatására a csontvelőben komplett morfológiai remisszió következett be, a beteg a jelenleg legjobb túlélést biztosító autológőssejt-transzplantáció előtt áll. Orv Hetil. 2021; 162(32): 1303–1308. Summary. Amyloid light-chain amyloidosis is a rare disease with diverse signs and symptoms according to the affected organs. The signs are often aspecific which can lead to delayed diagnosis. Considering the characteristic cutaneous manifestations of the disease, dermatologists have an important role in early identification. Additionally, histopathological examination is required for diagnosis. Here we present a rare case of light-chain amyloidosis in association with multiple myeloma. The patient presented with characteristic periocular, axillar and inguinal petechiae, purpurae, ecchymoses, suffusions, yellowish-brown waxy papules and plaques besides cardiovascular and renal involvement. In this case, the amyloidogenic proteins are the lambda-chains of monoclonal immunoglobulins secreted by the clonally expanded plasma cells of the bone marrow. The applied cyclophosphamide, bortezomib and dexamethason treatment induced complete morphological remission in the bone marrow and the patient currently awaits autologous stem cell transplantation which yields the longest possible survival. Orv Hetil. 2021; 162(32): 1303–1308.

Összefoglaló. A glutén, alimentáris környezeti antigénként, különböző szervrendszereket érintő autoimmun betegségeket tud kiváltani. A kórképek hátterében a gluténtolerancia veleszületett hiánya vagy az élet során bekövetkező elvesztése áll. A gluténindukált autoimmun betegségek között a leggyakoribb a coeliakia, melyet különböző súlyosságú enteropátia jellemez, és melynek a szöveti, 2-es típusú transzglutamináz az autoantigénje. A coeliakia extraintestinalis tünetei között azonban néha olyan bőr- és idegrendszeri kórképek jellegzetességei is megtalálhatók, melyek hátterében további transzglutamináz-autoimmunitás kialakulása áll. Idesorolható a hevesen viszkető, polimorf autoimmun bőrbetegség, a dermatitis herpetiformis (transzglutamináz-3-autoimmunitás) és a centrális és/vagy perifériás neurológiai károsodások egy jellegzetes csoportja (transzglutamináz-6-autoimmunitás). Az indukált autoimmunitás reverzibilis, a szigorúan tartott gluténmentes diéta mellett a coeliakia és a bőrtünetek elmúlnak, de az idegrendszeri tünetek egy része maradandó. Az elmúlt évtizedben beszámoltak gluténérzékeny, transzglutamináz-6-pozitív, nem coeliakiás (transzglutamináz-2-negatív) betegekről is. A gluténszenzitivitás sokféle megjelenését ma is erősen kutatják. Fontos a korai felismerés és a kórképek interdiszciplináris szemléletű kezelése. A coeliakia családi szűrővizsgálatokkal való korai felismerése és a tünetmentes egyének diétás kezelése is nagy jelentőségű a gluténérzékenység által kiváltott hiányállapotok és a társuló egyéb betegségek kialakulásának megelőzésében. Orv Hetil. 2021; 162(28): 1107–1118. Summary. Autoimmune diseases induced by digestion of gluten, an environmental antigen, can affect different organ systems. The diseases develop in individuals with congenital or acquired loss of gluten tolerance for life. Amongst the gluten-induced autoimmune diseases, celiac disease is the most common one, characterized by an enteropathy of varying severity. Here the target autoantigen is tissue (type 2) transglutaminase. While the extraintestinal manifestations of celiac disease are complex, they may include characteristics of certain skin and nervous system disorders that develop due to additional transglutaminase autoimmunities. Such diseases are the severely pruritic, polymorphic autoimmune skin disease, dermatitis herpetiformis due to epidermal (type 3) transglutaminase autoimmunity, and a distinctive group of gluten-sensitive neuropathies with central and/or peripheral neurological involvement caused by type 6 transglutaminase autoimmunity. While the celiac and skin autoimmune diseases gradually get into remission under a strict gluten-free diet, some neurological symptoms may persist. In the last decade, gluten-induced transglutaminase 6 positive but non-celiac (transglutaminase 2 negative) patients were reported. Today, various manifestations of gluten sensitivity are under extensive research. Early detection and interdisciplinary treatment of these disorders are important. Family screenings are of particular relevance in early recognition and dietary treatment of latent disease forms in order to prevent enteropathy-induced, malabsorption-related and other associated co-morbidities. Orv Hetil. 2021; 162(28): 1107–1118.

Systemic sclerosis is a collagen vascular disease which could lead to fibrosis of the skin and internal organs. It is characterized by a high disease burden and mortality. The 2013 classification criteria system helps to recognize the disease early, which allows prompt intervention and a better survival. In the past few year the recognition of the association between the presence of RNA polimerase III antibodies and malignancies was important. Treatment options of SSc have been improved. Early, intensive immunosuppressive treatment (cyclophosphamide, mycophenolate mofetil) of interstitial lung disease inhibits progression. There are several treatment modalities for the treatment of pulmonary arterial hypertension and digital ulcers (endothelin receptor antagonists, phosphodiesterase 5 inhibitors, prostaglandin analogues). Antifibrotic drugs (nintedanib, riociguate) are also available among former immunosuppressive and vasoactive agents. Lenabasum is a new promising selective cannabinoid receptor 2 agonist, which inhibits inflammation and fibrosis. Localized scleroderma presents a diverse clinical picture. The disease leads to fibrosis of the skin but it is not accompanied with serious inner organ involvement typically seen in systemic sclerosis. Capillaroscopy abnormalities, sclerodactyly and SSc specific antibodies are absent in localized scleroderma . The 2017 EDF guideline formulates a very useful classification, checkup and treatment recommendations.

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 Ca\(^{2+}\) flux pathway and delineate its importance in pemphigus pathogenesis and clinical phenotypes caused by different antibody profiles. Methods Immunoprecipitation, Ca\(^{2+}\) 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 Ca\(^{2+}\) 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 Ca\(^{2+}\) influx. Inhibitors against PLC, IP3R and CRAC effectively blocked PV IgG and PF IgG‐induced Ca\(^{2+}\) 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 Ca\(^{2+}\) signalling may be a promising approach to treat epidermal manifestations of pemphigus.

HPV associated diseases, which may range from banal conditions through sexually transmitted infections to malignant illnesses, are highly prevalent in the everyday dermatological practice. Our aim in this paper is to summarize the variety of their appearance and the possibilities of diagnostics and therapy.

Összefoglaló. Bevezetés: A nikkel széleskörűen elterjedt fém és kontaktallergén. Megtalálható mindennapi használati tárgyainkban, feldolgozza az ipari termelés, és az egészségügyben is rendre bővül alkalmazási köre. Egyidejűleg a társadalom növekvő hányadánál fordul elő nikkel-kontaktszenzibilizáció. Célkitűzés: Az epicutan tesztelt betegcsoport adatainak feldolgozása, kiemelve a nikkelpozitív betegek megoszlását nem, életkor, diagnózis, a klinikai tünetek lokalizációja és a társult fémérzékenység szerint, továbbá a 2004 óta érvényes európai uniós Nikkel Direktívák hatásainak tanulmányozása. Módszer: A közlemény a Semmelweis Egyetem Bőr-, Nemikórtani és Bőronkológiai Klinikájának Allergológia Laboratórium és Szakambulanciáján 1994-től 2014-ig 13 693 fő (10–87 év közötti) standard környezeti epicutan sorral tesztelt beteg adatait vizsgálja retrospektív módon. Eredmények: Az összes vizsgált bőrbeteg nikkelszenzibilizációs aránya 1994-ben 13,1%, 2004-ben 11,5%, 2014-ben 19,1% volt. A nikkel-kontaktdermatitis főként nőbetegeknél (93,0%) fordul elő. A klinikai tünetek elsősorban a karokra és az arcra lokalizálódnak. Nikkelérzékenyeknél az allergiás kontaktdermatitis diagnózisa 65,8%, atopiás dermatitis 9,7%-nál fordul elő. A nikkelérzékenységhez leggyakrabban társult fémallergének a kobalt és a króm. Az 1994–2004-es periódushoz képest az európai uniós Nikkel Direktívákat követő 10 évben a szenzibilizáció százalékos emelkedése szignifikáns volt, ugyanakkor a nikkelpozitívak évenkénti száma csökkent. 1994-ben a betegek legnagyobb hányada (26,5%) a 20–24 éves korcsoportba tartozott, 2004-ben szintén (20,8%), 2014-ben azonban a 35–39 éves korosztályhoz (15,1%). Következtetések: A nikkelszenzibilizáció korban eltolódást mutat az idősebb korosztály felé, a 35 évesnél fiatalabb betegek száma mérséklődött. A Nikkel Direktívák révén a fiatalabbak későbbi életkorban és kisebb mértékű nikkelexpozíciónak vannak kitéve. A vizsgált betegek nikkelérzékenységének százalékos emelkedése miatt azonban újabb szabályozások bevezetése és a hatályban lévők módosítása szükségszerű. Orv Hetil. 2021; 162(16): 629–637. Summary. Introduction: Nickel is a widely used metal and contact allergen. It can be found in our everyday objects and it is becoming more prevalent in healthcare. Simultaneously, nickel contact sensitization occurs more frequently. Objective: Analysis of data of patch tested patients by gender, age, diagnosis, localization of skin lesions, and associated metal sensitivity. Furthermore, to study the effects of the European Nickel Directives in force since 2004. Method: Retrospective analysis of data of 13 693 patients (aged 10–87) tested with a standard series of contact allergens at the Allergy Outpatient Unit and Laboratory of the Department, Venereology and Dermatooncology, Semmelweis University. Result: Nickel sensitization of all examined patients was 13.1% in 1994, 11.5% in 2004, and 19.1% in 2014. Contact dermatitis occurred mainly in females (93.0%). Skin lesions are primarily localized to the arms and face. Diagnosis of allergic contact dermatitis occurred in 65.8%, and atopic dermatitis in 9.7% of tested patients. Commonly associated metal sensitivities were cobalt and chromium. In the 10 years following the Nickel Directives, the increase of the ratio of sensitized patients was significant while the number of nickel-positives per year decreased. Both in 1994 and 2004, the largest proportion of patients belonged to the 20–24 age group (26.5% and 20.8%, respectively), but in 2014, to the 35–39 age group (15.1%). Conclusion: Nickel sensitization shifts towards the older age group, with a decrease in young patients. Because of the Nickel Directives, people are exposed to nickel at a later age and to a lesser extent. Due to the increase of the ratio of nickel-sensitive patients, it is necessary to introduce new regulations and amend the existing ones. Orv Hetil. 2021; 162(16): 629–637.

Purpose Eight of the ten items of the Dermatology Life Quality Index (DLQI) have a ‘not relevant’ response (NRR) option. There are two possible ways to interpret NRRs: they may be considered ‘not at all’ or missing responses. We aim to compare the measurement performance of the DLQI in psoriasis patients when NRRs are scored as ‘0’ (hereafter zero-scoring) and ‘missing’ (hereafter missing-scoring) using Rasch model analysis. Methods Data of 425 patients with psoriasis from two earlier cross-sectional surveys were re-analysed. All patients completed the paper-based Hungarian version of the DLQI. A partial credit model was applied. The following model assumptions and measurement properties were tested: dimensionality, item fit, person reliability, order of response options and differential item functioning (DIF). Results Principal component analysis of the residuals of the Rasch model confirmed the unidimensional structure of the DLQI. Person separation reliability indices were similar with zero-scoring (0.910) and missing-scoring (0.914) NRRs. With zero-scoring, items 6 (sport), 7 (working/studying) and 9 (sexual difficulties) suffered from item misfit and item-level disordering. With missing-scoring, no misfit was observed and only item 7 was illogically ordered. Six and three items showed DIF for gender and age, respectively, that were reduced to four and three by missing-scoring. Conclusions Missing-scoring NRRs resulted in an improved measurement performance of the scale. DLQI scores of patients with at least one vs. no NRRs cannot be directly compared. Our findings provide further empirical support to the DLQI-R scoring modification that treats NRRs as missing and replaces them with the average score of the relevant items.

ZusammenfassungIm Zuge der Neugründung medizinischer Fakultäten in Deutschland muss auch die dermatologische Forschung und Lehre aufgebaut werden. Diese großen Zusatzaufgaben neben der Krankenversorgung stellen eine Herausforderung für die Klinikleitungen dar. In diesem Übersichtsbeitrag beschreiben exemplarisch Chefärztinnen und Chefärzte neuer Universitätshautkliniken und dermatologischer Fachabteilungen die Auswirkungen, die die Umwandlung in eine Universitätsklinik auf die Forschung und Lehre sowie den klinischen Alltag mit sich gebracht hat.

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, the prognosis of 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 USA.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 organisations.

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 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.].

Absztrakt: Bevezetés: Az étel adverz reakciók gyakorisága egyre nő; a felnőtt populáció 10–20%-a tapasztal ilyen jellegű panaszokat, melyeket a legtöbbször ételallergiának hisznek, annak ellenére, hogy a nem immunmediált ételintoleranciák és az egyéb szerveket érintő (például gasztroenterológiai) kórfolyamatok nagyobb eséllyel okozzák ezeket. Célkitűzés: Célunk volt, hogy részleteiben megismerjük az étel adverz reakciókkal jelentkező felnőtt betegcsoportot és az ételallergiának vélt tünetek, tünetegyüttesek valós hátterét. Emellett, a kérdéskör kapcsán, az interdiszciplinaritás tükrében szeretnénk hangsúlyozni a differenciáldiagnosztika és a társszakmákkal történő együttműködés jelentőségét. Módszer: 406 felnőtt (>18) beteg allergodermatológiai vizsgálatára került sor élelmiszerekhez kapcsolódó tünetek felmérése, azonosítása és elkülönítése céljából. A páciensek változatos tünetekkel jelentkeztek, melyek mindegyikét „ételallergia” jeleként azonosították. Alapos anamnézisfelvétel és orvosi vizsgálat után, amennyiben indokoltnak bizonyult, specifikus-IgE-tesztek elvégzése történt. Szükség esetén a pácienseket társszakmák képviselőihez irányítottuk a kivizsgálás komplettálásához. Eredmények: 307 (75,6%) nő és 99 (24,4%) férfi vizsgálatára került sor. A betegcsoport átlagéletkora 43,6 év volt. A leggyakoribb objektivizálható bőrgyógyászati diagnózis az urticaria (44,1%) volt. Releváns IgE-mediált ételallergiát a 406 eset közül 6 betegnél (1,5%) tudtunk igazolni. Oralisallergia-szindrómát 35 (8,6%) esetben diagnosztizáltunk. Biogénamin-intolerancia 50,2%-ban, valamint egyéb társbetegségek (gasztroenterológiai: 62,1%, krónikus gyulladás: 22,2%) merültek fel provokáló faktorként a tünetek hátterében. A betegek által szedett és étkezéssel egyidejűleg bevett gyógyszerek mellékhatásait azonosítottuk 20 esetben. Következtetés: Adataink szerint a betegek által ételekhez kötött reakciók hátterében a biogénamin-intolerancia nagyon gyakori, viszont az ételallergiák száma túlbecsült. Az egészségügyben dolgozók és a betegek felé is fontos hangsúlyozni az adverz ételreakciók mögött meghúzódó változatos kóroktant. A differenciáció alapja mindig alapos anamnézisfelvétel, orvosi vizsgálat és személyre szabott kivizsgálás kell, hogy legyen. Orv Hetil. 2020; 161(25): 1042–1049.

Background Rosacea is a common chronic inflammatory cutaneous disorder affecting nearly 5.5 % of the adult population. Our aim was to evaluate the prevalence and epidemiology of rosacea and perioral dermatitis (POD) in an ambulatory care setting. Methods We retrospectively analyzed medical data of patients with a confirmed diagnosis of rosacea or perioral dermatitis (POD) presenting at our university hospital outpatient clinic during a 3‐year period. Results Out of 1032 patients, 81.5 % were diagnosed with rosacea and 18.5 % with POD. Overall prevalence was 1.4 % for rosacea and 0.3 % for POD. 69.3 % of the analyzed patients were female. Overall mean age was 49.3 ± 7.7 (1–92) years; the women’s average age was less than the men’s. Patients with POD were younger and predominantly female, whereas patients with phymatous rosacea were older and predominantly male. The most common phenotypes were papulopustular rosacea (68.4 %), erythematotelangiectatic rosacea (22.5 %), and phymatous rosacea (8.0 %). Special forms of rosacea were diagnosed in 15.8 % of the patients; the most frequent were ocular rosacea (6.9 %) and steroid‐induced rosacea (5.4 %). Conclusions The large patient cohort analyzed in our study provides a good estimate of the frequency of the rosacea subtypes, special forms and of perioral dermatitis in a hospital‐based outpatient care setting.

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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 wa...

Background: After the outbreak of the corona virus disease-19 (COVID-19) pandemic, teledermatology was implemented in the Hungarian public healthcare system for the first time. Our objective was to assess aggregated diagnostic agreements and to determine the effectiveness of an asynchronous teledermatology system for skin cancer screening. Methods: This retrospective single-center study included cases submitted for teledermatology consultation during the first wave of the COVID-19 pandemic. Follow-up of the patients was performed to collect the results of any subsequent personal examination. Results: 749 patients with 779 lesions were involved. 15 malignant melanomas (9.9%), 78 basal cell carcinomas (51.3%), 21 squamous cell carcinomas (13.8%), 7 other malignancies (4.6%) and 31 actinic keratoses (20.4%) were confirmed. 87 malignancies were diagnosed in the high-urgency group (42.2%), 49 malignancies in the moderate-urgency group (21.6%) and 16 malignancies in the low-urgency group (4.6%) (p < 0.0001). Agreement of malignancies was substantial for primary (86.3%; κ = 0.647) and aggregated diagnoses (85.3%; κ = 0.644). Agreement of total lesions was also substantial for primary (81.2%; κ = 0.769) and aggregated diagnoses (87.9%; κ = 0.754). Conclusions: Our findings showed that asynchronous teledermatology using a mobile phone application served as an accurate skin cancer screening system during the first wave of the COVID-19 pandemic.