Fumaric acid ester treatment in cutaneous lupus erythematosus (CLE): a prospective, open-label, phase II pilot study.

OBJECTIVE: The aim of the study was to assess the efficacy and safety of fumaric acid esters (FAEs) in patients with cutaneous lupus erythematosus (CLE). METHODS: In this 24-week, prospective, open-label, phase II pilot study, 11 patients with CLE, refractory to topical corticosteroids, were included. The primary endpoint of the study was the evaluation of the efficacy of FAEs after 24 weeks of treatment as assessed by the Revised Cutaneous Lupus Disease Area and Severity Index (RCLASI). RESULTS: Compared to baseline, significant improvement in the mean total RCLASI activity score and the mean RCLASI activity score for skin lesions was observed in week 12 (p = 0.002, p = 0.002, respectively) and in week 24 (p = 0.009, p = 0.009, respectively). Most common adverse events included abdominal cramps and headache. CONCLUSIONS: FAEs could be an alternative and safe treatment in patients with therapy-refractory CLE; however, randomized controlled trials are warranted to evaluate the efficacy and safety of FAEs in this disease.

Cutaneous characteristics and association with antinuclear antibodies in 402 patients with different subtypes of lupus erythematosus.

BACKGROUND: Lupus erythematosus (LE) is an autoimmune disease characterized by a heterogeneous spectrum of skin manifestations and organ affection, and is frequently associated with serum autoantibodies, which mostly remain positive through the course of the disease. The classification of LE is still a controversial topic. OBJECTIVES: To examine the prevalence and long-term course of autoantibodies in patients with cutaneous LE (CLE) and/or systemic LE (SLE) treated in the outpatient clinic for connective tissue diseases of the department of Dermatology in Bochum, Germany. METHODS: Four hundred and two patients with LE were evaluated for antinuclear antibodies at a whole of 1572 time points. The prevalence as well as the long-term positivity of antinuclear antibodies and their correlation with the various subtypes of disease was examined. RESULTS: Antinuclear antibody (ANA) testing and anti-ds-DNA antibodies were not only more prevalent in SLE patients (as expected from the ACR criteria for diagnosis of SLE, P < 0.0001), but also have had a more consistent course in the long-term evaluation (P = 0.0001 and P = 0.0111 respectively). Subacute cutaneous LE (SCLE) was associated with ANA (P = 0.0075), anti-Ro (P < 0.0001) and anti-La (P < 0.0001) antibodies, showing also higher consistency rates for these antibodies than discoid LE (DLE, P = 0.049, P = 0.004, P = 0.0004). Our data from 100 patients with LE tumidus (LET) support its perception as a distinct subtype of LE, not correlating with systemic disease or antinuclear antibodies (P < 0.0001). Anti-U1-ribonucleoprotein antibodies correlated with CLE in SLE patients (P = 0.0237), whereas non-LE-specific antinuclear antibodies were a rare, inconsistent autoimmune epiphenomenon in patients with SLE. CONCLUSION: Long-term analysis of antinuclear antibodies has shown significant differences in various clinical subtypes of LE, confirming the actual classification of the disease. A serial evaluation of antinuclear antibodies may support the classification of disease in LE patients with overlapping clinical features.

Influence of smoking on disease severity and antimalarial therapy in cutaneous lupus erythematosus: analysis of 1002 patients from the EUSCLE database.

BACKGROUND: In recent years it has been controversially discussed in the literature if smoking is associated with the activity of cutaneous lupus erythematosus (CLE) and the efficacy of antimalarial agents. OBJECTIVES: To investigate the influence of smoking on disease severity and antimalarial treatment in patients with CLE using the Core Set Questionnaire of the European Society of Cutaneous Lupus Erythematosus (EUSCLE). METHODS: A total of 1002 patients (768 female, 234 male) with different CLE subtypes were included in this cross-sectional study, which was performed in 14 different countries. Smoking behaviour was assessed by the EUSCLE Core Set Questionnaire in 838 patients and statistically analysed using an SPSS database. The results were correlated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the efficacy of antimalarial treatment. RESULTS: A high percentage (87·2%) of the 499 patients with CLE, who have ever smoked, had already smoked at the date of their first diagnosis. Patients with intermittent CLE have ever smoked significantly more often than patients with subacute CLE (P < 0·05) and chronic CLE (P < 0·05). The total CLASI activity and damage score of patients with CLE was 6·6 ± 7·1 and 2·6 ± 4·3, respectively, and was higher in patients who have ever smoked than in nonsmokers. Antimalarial treatment was successful in 84·3% of cases, with a significantly higher efficacy in nonsmokers than in patients with CLE who have ever smoked (P < 0·05). CONCLUSIONS: This analysis of a multicentre study population of 838 patients with CLE assessed by the EUSCLE Core Set Questionnaire confirms that smoking negatively influences CLE disease severity and the efficacy of antimalarial treatment.

The impact of the EUSCLE Core Set Questionnaire for the assessment of cutaneous lupus erythematosus.

Epidemiological data and standard European guidelines for the diagnosis and treatment of cutaneous lupus erythematosus (CLE) are lacking in the current literature. In order to provide a standardized tool for an extensive consistent data collection, a study group of the European Society of Cutaneous Lupus Erythematosus (EUSCLE) recently developed a Core Set Questionnaire for the assessment of patients with different subtypes of CLE. The EUSCLE Core Set Questionnaire includes six sections on patient data, diagnosis, skin involvement, activity and damage of disease, laboratory analysis, and treatment. An instrument like the EUSCLE Core Set Questionnaire is essential to gain a broad and comparable data collection of patients with CLE from different European centres and to achieve consensus concerning clinical standards for the disease. The data will also be important for further characterization of the different CLE subtypes and the evaluation of therapeutic strategies; moreover, the EUSCLE Core Set Questionnaire might also be useful for the comparison of data in clinical trials. In this review, the impact of the EUSCLE Core Set Questionnaire is discussed in detail with regard to clinical and serological features as well as therapeutic modalities in CLE.

Use of methotrexate in patients with psoriasis.

Psoriasis is considered to be a polygenetically influenced, immune-mediated, organ-specific disease of dysregulated inflammation that is triggered by environmental factors such as infections, medications, and physical and/or emotional stress. It is recognised as one of the most prevalent skin diseases, affecting 2% to 3% of Caucasian populations. Major advances in understanding of disease pathogenesis indicate that patients with psoriasis have an increased risk of comorbidities such as metabolic syndrome and cardiovascular disease. A wide range of systemic drugs have been developed in recent years for treatment of psoriasis and comorbidities. Low-dose methotrexate (MTX) is one of the classical agents and is still one of the most frequently used systemic treatments for psoriasis worldwide. Low-dose MTX is also effective in treatment of psoriatic arthritis. The mechanism of action is not fully understood, but MTX is suggested to act primarily as an anti-inflammatory and immunosuppressant drug. A favourable efficacy and safety profile has been established for MTX in a large number of clinical trials, as well as in common practice. This review summarises the nature of the disease and our present knowledge about MTX in the treatment of psoriasis, including combination therapies.

Primary Cutaneous CD30+ Anaplastic Large T Cell Lymphoma in a Patient Treated with Cyclosporine for Actinic Reticuloid.

Actinic reticuloid (AR)-a subtype of chronic actinic dermatitis-clinically and histopathologically shows lymphoma-like features. We report a male patient initially diagnosed with erythrodermic cutaneous T cell lymphoma (CTCL) who developed severe broadband photosensitivity. Clinical evaluation, histopathology, and phototesting were consistent with AR. The patient was treated with cyclosporine 150-300 mg/d. Under this therapy, he developed several times primary cutaneous anaplastic large cell lymphomas (C-ALCL) which in part tended to regress spontaneously under cyclosporine reduction. The association between cyclosporine treatment and development of C-ALCL and other CD30+ lymphoproliferative disorders has previously been reported in patients with atopic dermatitis, psoriasis, and transplant patients. In conclusion, the present case highlights the difficulties arising in the distinction between AR and CTCL and shows that long-term cyclosporine treatment may cause C-ALCL development in AR as well.

Primary osseous leiomyosarcoma of the spine: a rare entity--case report and review of the literature.

Primary osseous leiomyosarcoma of the spine is an extremely rare entity with only 13 previous cases having been reported in the literature. We present the case of a 57-year-old woman with a tumor of the eleventh thoracic vertebra, causing compression of the spinal cord. Histopathology and immunohistochemical results of the tumor biopsy specimen disclosed a primary leiomyosarcoma of the thoracic spine. We report on the features of this rare entity but also review and summarize the imaging and pathology characteristics that can be extrapolated from the existing literature.