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Abstract Background Sjögren syndrome (SS) is a systemic autoimmune disease characterised by oral and ocular mucosal dryness. Objectives Our study focused on skin manifestations of SS. The differences between primary SS (pSS) and secondary SS (sSS) were investigated. Furthermore, we elucidated factors predicting internal organ involvement. Methods This was a single centre retrospective cohort study. We analysed clinical and laboratory data from 185 Ro‐SSA positive SS patients between January 2000 and December 2016. Results Skin manifestations were present in 74.9% (n = 137) of SS patients. Dermatitis and Lupus erythematosus (LE)‐specific skin manifestations were present in 34.1% (n = 63) and 21.6% (n = 40) of SS patients respectively. 65.9% (n = 122) of SS patients had internal organ involvement, with 53% (n = 98) presenting neuropsychiatric manifestations. Several risk factors for internal organ involvement were identified: presence of myalgia/arthralgia (odds ratio [OR] = 3.05, confidence interval [CI] 1.29−7.22), fatigue (OR = 2.53, CI 1.11−5.73) and age older than 50 years (OR = 2.23, CI 1.01−4.93). Conclusions Internal organ involvement is frequent in SS, however there is no significant difference between pSS and sSS concerning its occurrence.

Abstract Introduction Plaque psoriasis is a chronic relapsing inflammatory skin disease that is associated with extensive disease burden that often requires long-term therapy. Treatment of psoriasis with 4 weeks of the aerosol foam formulation of calcipotriol/betamethasone dipropionate (Cal/BD; Enstilar®, LEO Pharma) has been demonstrated to be effective, well tolerated, and associated with high patient satisfaction. Cal/BD foam is approved as a first-line treatment in multiple countries, where several non-interventional studies (NIS) have corroborated the beneficial efficacy and safety profiles determined in the randomized clinical trials. Heterogenicity in these NIS, however, prevents the use of a data pooling strategy for comparisons of effectiveness outcomes across different patient populations. Methods Therefore, here, we report on a post hoc analysis of effectiveness data consolidated from six prospective NIS to discern any differences in improvement in signs and symptoms of psoriasis attributable to Cal/BD foam treatment across the countries. In addition, we provide real-world experience of clinicians with Cal/BD foam treatment, factoring in changes in usage since these NIS were performed in their local markets. Results This post hoc analysis of Cal/BD foam NIS brings together data outside of randomized clinical trials from six countries to provide real-world evidence in 1388 patients showing that 4 weeks of Cal/BD foam is an effective and safe treatment option with quick onset of action for patients with psoriasis. Conclusion These results show that regardless of NIS location, Cal/BD foam remains a well-tolerated, efficacious option for patient care that could be used as a first-line topical therapy for mild-to-severe psoriasis.

Abstract Background For the topical treatment of plaque psoriasis, vitamin D analogues and corticosteroids are used as a fixed combination, which is available in different galenic preparations. Objectives The aim of this study was to investigate the effectiveness and tolerability of calcipotriol/betamethasone (Cal/BD) cutaneous foam in a real‐world setting. Methods Overall, 107 adult patients with at least mild plaque psoriasis as measured by the Investigator Global Assessment (IGA) were included and treated for 4 weeks. Affected body surface area (BSA), plaque locations, and Psoriasis Area and Severity Index (PASI) were documented at baseline, after 4 weeks and at the end of the study. Pruritus, overall patient satisfaction, tolerability and convenience of use, as well as quality of life (DLQI) were also evaluated. Results In total, 96% of the patients suffered from mild to moderate psoriasis vulgaris. Of all patients included, 76% had previously received topical therapy. Under treatment with Cal/BD aerosol foam, the mean IGA score was reduced from 2.49 to 1.28 points (p

Background: Little is known about the effectiveness and drug survival associated with apremilast under real-world conditions. Objective: To investigate the influence of patient and disease characteristics on drug survival associated with apremilast and to elucidate clinical effectiveness with regard to the psoriasis area and severity index (PASI) reduction. Methods: This was an observational, retrospective, multicenter analysis from the Austrian Psoriasis Registry. Results: Data from 367 patients were eligible for analysis. The 12-month drug survival rate associated with apremilast (ie, the proportion of patients on the drug) was 57.3% and decreased significantly in patients younger than 40 years (relative hazard ratio = 1.49, P = .007918). Sex; concomitant arthritis; previous biologic therapy; obesity; and palmoplantar, scalp, nail, and intertriginous involvement did not significantly affect drug survival. At 12 months, the response rates in patients receiving apremilast per protocol with a PASI of 50, 75, 90, and 100 were 80.0%, 56.4%, 38.2%, and 22.7%, respectively. Limitations: Inclusion of a substantial number of patients with no record of absolute PASI at study entry and lack of PASI reduction follow-up data of 103 patients (28.1%) after starting apremilast treatment. Conclusion: Apremilast is a robust antipsoriatic drug for which the drug survival is not strongly influenced by most patient- or disease-related factors except age. Drug survival is significantly shorter in patients younger than 40 years.

Background: Little is known about psychological discomfort and quality of life (QoL) in early stage mycosis fungoides (MF) and the effect of psoralen plus UV-A (PUVA) on it.Objective: To evaluate QoL, anxiety, and depression with validated instruments in early stage MF patients and whether PUVA treatment improves it.Methods: Patients with stage IA to IIA MF were treated with PUVA twice weekly for 12–24 weeks, followed by maintenance treatment or not, in a prospective randomized clinical trial. Patients completed a questionnaire on DLQI as well as the Hospital Anxiety and Depression Scale (HADS) prior to therapy, after their last PUVA exposure, and after the PUVA maintenance or observance phase.Results: For 24 patients with early stage MF, completed questionnaires were available and analyzed. Prior to treatment, 17% reported strong (DLQI > 10) and 29% moderate impairment (DLQI 6–10) in QoL; 33% of patients reported HADS scores indicating anxiety, and 21% reported scores indicating depression. PUVA significantly improved overall QoL by reducing mean DLQI scores by 58.6% (p = 0.003), HADS-A by 30% (p = 0.045), and HADS-D by 44% (p = 0.002). Improvements in QoL and psychological well-being seemed to be sustained, irrespective of maintenance treatment or not.Limitations: Small sample size.Conclusions: PUVA sustainably improves QoL and psychological well-being in patients with early stage MF.Clinical trial registration:ClinicalTrials.gov identifier: NCT01686594.

We report on three exceptional courses of sinus histiocytosis Rosai-Dorfman. Patient one developed regional lymph-node disease subsequent to two independent malignancies in the right head and neck region. Patient two suffered from extensive extranodal disease with more than 100 mucocutaneous lesions over 17 years, which spontaneously resolved. Patient three showed exclusively extranodal disease, including bilateral conjunctival/scleral lesions, before he developed lung cancer. Our cases are unique for three reasons: the association of the disease with solid malignancies in two cases, the extent and persistence of exclusively extranodal disease in one patient and the appearance of thus far undescribed conjunctival/scleral lesions. [ABSTRACT FROM AUTHOR]