Lichturtikaria: Klinik, Diagnostik, Verlauf und Therapiemanagement bei 27 Patienten.

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.

Solar urticaria: Clinic, diagnostic, course and therapy management in 27 patients.

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.

Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: a multicenter, randomized, double-blind, vehicle-controlled trial.

BACKGROUND: Topical calcineurin inhibitors are licensed for the treatment of atopic dermatitis; however, the efficacy of tacrolimus in cutaneous lupus erythematosus (CLE) has only been shown in single case reports. OBJECTIVE: In a multicenter, randomized, double-blind, vehicle-controlled trial, we sought to evaluate the efficacy of tacrolimus 0.1% ointment for skin lesions in CLE. METHODS: Thirty patients (18 female, 12 male) with different subtypes of CLE were included, and two selected skin lesions in each patient were treated either with tacrolimus 0.1% ointment or vehicle twice daily for 12 weeks. The evaluation included scoring of clinical features, such as erythema, hypertrophy/desquamation, edema, and dysesthesia. RESULTS: Significant improvement (P < .05) was seen in skin lesions of CLE patients treated with tacrolimus 0.1% ointment after 28 and 56 days, but not after 84 days, compared with skin lesions treated with vehicle. Edema responded most rapidly to tacrolimus 0.1% ointment and the effect was significant (P < .001) in comparison to treatment with vehicle after 28 days. Clinical score changes in erythema also showed remarkable improvement (P < .05) after 28 days, but not after 56 and 84 days. Moreover, patients with lupus erythematosus tumidus revealed the highest degree of improvement. None of the patients with CLE demonstrated any major side effects. LIMITATIONS: The study was limited by the small sample size. CONCLUSION: Explorative subgroup analyses revealed that topical application of tacrolimus 0.1% ointment may provide at least temporary benefit, especially in acute, edematous, non-hyperkeratotic lesions of CLE patients, suggesting that calcineurin inhibitors may represent an alternative treatment for the various disease subtypes.

Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication.

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.