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Competing Interests: Conflicts of interest The authors declare they have no conflicts of interest.

Background: European guidelines propose a 0·5 mg kg -1 per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients., Methods: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg -1 per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg -1 per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score., Results: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3-69.5]; 18 of 24 patients (75%, 95% CI 53·3-90·2), 75 of 110 patients (68·8%, 95% CI 59·2-77·3) and 26 of 56 patients (46.4%, 95% CI 33·0-60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan-Meier 1-year survival was 82·6% (95% CI 76·3-87·4) corresponding to 90·9%, 83·0% and 80·0% rates in patients with mild, moderate and severe BP, respectively (P = 0·5). Thresholds of 49 points for BPDAI score and 70 points for Karnofsky score yielded maximal Youden index values with respect to disease control at day 21 and 1-year survival, respectively., Conclusions: A 0·5 mg kg -1 per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous., (© 2021 British Association of Dermatologists.)

Background: Corticosteroids (CS) with or without adjuvant immunosuppressant agents are standard treatment for pemphigus vulgaris (PV). The efficacy of adjuvant therapies in minimizing steroid-related adverse events (AEs) is unproven., Objectives: To utilize data collected in a French investigator-initiated, phase III, open-label, randomized controlled trial to demonstrate the efficacy and safety of rituximab and seek approval for its use in PV., Methods: This was an independently conducted post hoc analysis of the moderate-to-severe PV subset enrolled in the Ritux 3 study. Patients were randomized to rituximab plus 0·5 or 1·0 mg kg -1 per day prednisone tapered over 3 or 6 months, or 1·0 or 1·5 mg kg -1 per day prednisone alone tapered over 12 or 18 months, respectively (according to disease severity). The primary end point was complete remission at month 24 without CS (CRoff) for ≥ 2 months, and 24-month efficacy and safety results were also reported., Results: At month 24, 34 of 38 patients (90%) on rituximab plus prednisone achieved CRoff ≥ 2 months vs. 10 of 36 patients (28%) on prednisone alone. Median total cumulative prednisone dose was 5800 mg in the rituximab plus prednisone arm vs. 20 520 mg for prednisone alone. Eight of 36 patients (22%) who received prednisone alone withdrew from treatment owing to AEs; one rituximab-plus-prednisone patient withdrew due to pregnancy. Overall, 24 of 36 patients (67%) on prednisone alone experienced a grade 3/4 CS-related AE vs. 13 of 38 patients (34%) on rituximab plus prednisone., Conclusions: In patients with moderate-to-severe PV, rituximab plus short-term prednisone was more effective than prednisone alone. Patients treated with rituximab had less CS exposure and were less likely to experience severe or life-threatening CS-related AEs. What's already known about this topic? Pemphigus vulgaris (PV) is the most common type of pemphigus. Corticosteroids, a standard first-line treatment for PV, have significant side-effects. Although their effects are unproven, adjuvant corticosteroid-sparing agents are routinely used to minimize steroid exposure and corticosteroid-related side-effects. There is evidence that the anti-CD20 antibody rituximab is effective in the treatment of patients with severe recalcitrant pemphigus and in patients with newly diagnosed pemphigus. What does this study add? This study provides a more detailed analysis of patients with PV enrolled in an investigator-initiated trial. Rituximab plus prednisone had a steroid-sparing effect and more patients achieved complete remission off prednisone. Fewer patients experienced grade 3 or grade 4 steroid-related adverse events than those on prednisone alone. This collaboration between academia and industry, utilizing independent post hoc analyses, led to regulatory authority approvals of rituximab in moderate-to-severe PV., (© 2019 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.)

Bier's spots are asymptomatic and permanent white macules, standing on a cyanotic background. These spots are associated with venous stasis and usually appear in physiological conditions. We report the case of a 47-year-old woman with a 2-year history of systemic scleroderma, who developed Bier's white spots associated with a vascular and renal crisis. Interestingly, these spots appeared with the crisis, and disappeared with the treatment and resolution of the crisis. They are probably a result of anatomical and functional damage to the small vessels of the skin. The pathophysiology in the skin is probably the same as that which happens in renal vessels during scleroderma renal crisis.

Background: Drug-induced aphthous ulcers have been the subject of several isolated and heterogeneous case reports for the last three decades., Objectives: To perform a case-control study to evaluate the risks linked to drug exposure in aphthous ulcers., Methods: Eighty patients with typical clinical patterns of aphthous ulcers and 152 control patients who had had consultations for skin tumours were studied. A standardized questionnaire, concerning clinical features, life-style and medications taken during the last month, was completed for each patient., Results: Case patients had a much higher intake of medications than control patients, respectively, 5.1 and 2. 8 medications per patient (P < 0.0001). Multivariate paired analysis showed an association between aphthous ulcers and two classes of drugs: non-steroidal anti-inflammatory drugs (P < 0.001) and beta-blockers (P = 0.002). Smoking could have a protective effect against aphthous ulcers (P < 0.001)., Conclusions: Previous case reports and the results of this study suggest a real link between beta-blockers and aphthous ulcers. Our study did not confirm a role of other drugs but a few interesting case reports with positive reintroduction have to be considered. These results could be beneficial for patients, as healing may occur when the incriminated drug is discontinued.

Cogan's syndrome is a rare clinical entity defined by the association of a nonsyphilitic interstitial keratitis and vestibuloauditory dysfunction, typically Menière's disease-like; the condition has been reported in association with a variety of cutaneous diseases. We now report a case of pyoderma gangrenosum complicating Cogan's syndrome in a 57-year-old woman, which then healed dramatically, as more interestingly did the associated uveitis with minocycline therapy.

We report the case of a 72-year-old man with clinical features resembling those of non-lethal junctional epidermolysis bullosa associated with IgG autoantibodies to the beta chain of laminin-5. The patient presented with a sudden onset of blistering and severe fragility of the skin and mucous membranes resulting in atrophic scars. Electron microscopy showed that the blistering arose in the lamina lucida. Indirect immunofluorescence indicated that the autoantibodies bound to the dermal side of 1 mol/L NaCl-split skin, and both direct and indirect immunoelectron microscopy demonstrated antibody binding to the lamina densa. Postembedding immunogold electron microscopy also revealed labelling in the lamina lucida beneath the hemidesmosomes. On immunoblotting, we found the autoantibodies to comigrate with the beta chain of laminin-5. Following the nomenclature of inherited junctional epidermolysis bullosa with mutations of the laminin-5 gene, we propose the name acquired junctional epidermolysis bullosa for this newly recognized disease.

The prognosis of bullous pemphigoid (BP), a disorder which usually affects elderly patients, is not well established and conflicting data have been reported about the mortality rate of the disease. Our objective in this study was to assess the clinical and immunological factors determining survival in a prospective series of 94 patients with BP. A cohort of 94 consecutive patients with BP (mean age +/- SD: 81 +/- 4 years) was studied over an 8-year period (1987-94) in one department and patients followed up for at least 1 year. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (i.e. linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting. Our analysis (median duration of follow-up: 5 years) showed that 37% of BP patients were dead within a year of starting treatment. The clinical or immunological factors which may influence the prognosis of BP were studied according to the criterion of death or survival by the end of the first year of treatment. None of the following factors was found to be significantly linked to the prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, or the presence of circulating basement membrane zone autoantibodies by indirect IF. An impaired general condition and a history of coronary artery disease indicated a bad prognosis. The presence of circulating autoantibodies against BP180 autoantigen but not autoantibodies against BP230, as detected by immunoblotting on epidermal extracts, was found to be significantly more frequent (60% vs. 25%) in BP patients who died within the first year of treatment (P < 0.01). We conclude that the presence of circulating autoantibodies against BP180 represents the first intrinsic prognostic factor that has been demonstrated in BP. This result supports the growing body of evidence for the pathophysiological importance of the anti-BP180 autoantibodies.