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Background and Objectives: Solar urticaria is a rare photodermatosis, the diagnosis and therapy of which have not yet been standardized. The aim of this research was to use innovative radiation sources for diagnostics with defined and reproducible emission spectra and doses. A uniform therapy step scheme was to be created., Patients and Methods: In a longitudinal study, 27 patients with solar urticaria were examined over 13 years. With a characteristic anamnesis, the diagnosis was confirmed with phototesting (photoprovocation) from various radiation sources (UVB, UVB311nm, UVA, UVA-1, green light, red light) and a therapy step scheme was designed consisting of light protection, antihistamines, rush hardening with UVA-1, and administration of omalizumab., Results: Action spectrum: UVB 44 %, UVA 70 %, UVA-1 89 %, green light 37 % and red light 22 %. Rush hardening with subsequent maintenance therapy was performed on 20 patients, 17 of whom were hereby adequately protected. In three further patients, omalizumab was additionally administered., Conclusions: Phototesting with UVB, UVB311nm, UVA, UVA-1, and visible light with innovative radiation sources is uniformly possible in every major skin clinic. With the help of the therapy step scheme the patients can be adjusted well. Rush hardening with UVA-1 is a safe method to help the patients during the sunny season. Omalizumab as the last therapy option is effective, but currently only possible in off-label use., (© 2020 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.)

Background and Objectives: Solar urticaria is a rare photodermatosis, yet lacking standardization in diagnosis and therapy. The aim of this research was to use innovative radiation sources for diagnostics with defines and reproducible emission spectrum and doses. A uniform therapy step scheme was to be created., Patients and Methods: In a longitudinal study, 27 patients were examined with solar urticaria over 13 years. With a characteristic anamnesis, the diagnosis was confirmed with phototesting from various radiation sources (UVB, UVB311nm, UVA, UVA1, green light, red light) and a therapy step scheme consisting of light protection, antihistamines, rush hardening with UVA-1 and the treatment with omalizumab., Results: Action spectrum: UVB 44 %, UVA 70 %, UVA1 89 %, green light 37 % and red light 22 %. Rush hardening was performed on 20 patients, 17 of whom were adequately protected. In three further patients, omalizumab was additionally treated in off-label use., Conclusions: Photoprovocation with UVB, UVB311nm, UVA, UVA-1 and visible light with innovative radiation sources is uniformly possible in every major skin clinic. With the help of the therapy step scheme the patients can be adjusted well, a Rush Hardening with UVA-1 is a safe method to help the patients during the sunny season. Omalizumab as the last therapy option is effective, but currently only possible in off-label use., (© 2020 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.)

Objective: Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC., Methods: Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC., Results: Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 ± 2.8 and a mean time of followup of 3.6 ± 3.8 years. In total, 70 patients developed SRC (70/2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc-overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC., Conclusion: In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.