Search

Serum levels of the IFN-regulated cytokine CXCL13 have been found to correlate with SLEDAI and renal involvement in systemic lupus erythematosus. This study investigates whether CXCL13 can also be a marker of disease activity in patients with subacute cutaneous or chronic cutaneous lupus erythematosus (SCLE, CCLE). We analysed CXCL13 levels in 60 patients' sera (18 SLE, 19 SCLE, 23 CCLE) at five time points within 1 year and correlated these levels with disease activity scores and laboratory markers. Clinical scores with no/mild, moderate or high/severe disease activity were categorized by SLEDAI in SLE, by CLASI in SCLE/CCLE. CXCL13 levels were significantly higher in SLE (median 122.5, IQR 88.0-239.0 pg/ml) than in CCLE patients (median 69.0, IQR 60.0-102.0 pg/ml) (p = 0.006). CXCL13 levels were elevated in 59% (41/70) of SLE patient visits with mild or no disease activity, but in 90% (9/10) with high disease activity. CXCL13 levels correlated with ECLAM, dsDNA-antibodies, and inversely with complement factors C3 and C4 in SLE, and with IgA and ESR in SCLE. In CCLE CXCL13 did not correlate with CLASI or laboratory markers. One SCLE and two CCLE patients with CXCL13 levels > 500 pg/ml had conversion to SLE or an underlying autoimmune disease. CXCL13 seems to be a useful marker of disease activity in SLE, but not in SCLE and CCLE. Conversion from normal to elevated CXCL13 may indicate a flare of SLE. Whether high CXCL13 levels in cutaneous LE indicate the development of SLE should be further investigated.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy)., (© 2017 European Academy of Dermatology and Venereology.)

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum., (© 2017 European Academy of Dermatology and Venereology.)

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP 50 ).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP 50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com, (Copyright ©ERS 2017.)

Background: A variety of therapeutic options have been reported for cutaneous lupus erythematosus (CLE); one of these is dapsone. However, no high priority has been given to this drug., Objective: To determine whether dapsone is an effective and safe treatment alternative in patients with LE., Methods: We reviewed the literature and analyzed retrospectively 34 CLE patients who were treated with dapsone as monotherapy or combined with antimalarials. We assessed the course of the disease under treatment, response, concomitant treatment and side effects., Results: Six patients went into remission, 14 patients improved, and in 6 patients disease remained constant during the observation period. Dapsone was discontinued in 9 patients: in 4 due to reversible side effects and in 5 patients due to poor efficacy., Conclusion: Our data confirm that dapsone with/without antimalarials is effective in more than 50% of patients with CLE and could be used as second-line therapy for CLE., (© 2015 S. Karger AG, Basel.)

Background: The treatment of severe morphea is challenging, and treatment experience concerning the use of immunosuppressive agents for this condition is limited., Objective: The purpose of this study is to analyze the use of cyclosporine, its tolerability, and its effect on skin sclerosis., Materials and Methods: Patients with severe morphea who underwent treatment with cyclosporine were studied retrospectively., Results: Five of 12 patients with morphea showed complete remission and 6 patients had partial remission at the end of therapy (9-46 months, median 14) under a median cyclosporine dose of 2.4 mg/kg. The mean affected body surface area fell from 50% (2-80, median 65) to 17% (0-40, median 18). Side effects were hypertension, elevated transaminases, cholesterol, and weight gain., Conclusion: Cyclosporine can be effective in morphea. The side effects were reversible. However, the duration of treatment with cyclosporine is limited because of its potential permanent side effects. Prospective placebo-controlled studies are needed to establish the superiority of cyclosporine over other immunosuppressive drugs in this setting., (© 2016 S. Karger AG, Basel.)

Purpose: To compare Borrelia-specific intrathecal antibodies by two different ELISAs, an immunoblot (IB) and CXCL13., Methods: Twenty-seven adults and 23 children with clinical symptoms compatible with NB were tested for Borrelia-specific intrathecal antibodies by flagellum ELISA-AI (flELISA), a recombinant ELISA-AI (rELISA) and by IB. Patients were classified according to the European Federation of Neurological Societies (EFNS) criteria as definite NB, possible NB, or non-NB. CSF CXCL13 levels were measured by ELISA., Results: Among 50 patients, definite NB was diagnosed with the rELISA-AI in 29 (58%) patients, confirmed by IB in 19/29 patients, with flELISA-AI in 17 (34%) patients, confirmed by IB in 15/17 patients, and with IB in 20 (40%) patients. CXCL13 was positive in 22 (44%) patients. In 4 of 8 patients with negative AI, IB showed many detectable bands both in the CSF and serum., Conclusions: The diagnosis of NB strongly relies on the used test method. The rELISA-AI test appears to be the most sensitive while the flELISA-AI is the least sensitive. However when the ELISA-AIs were confirmed by IB, different patients were identified as NB, while only 26% were identified by all performed test methods. There is a demand for standardized test methods with well-defined sensitivity and specificity to establish validated diagnostic criteria for NB including the use of the IB assay and CXCL13 as an additional non-Borrelia specific determinant in early NB., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: Systemic sclerosis (SSc) is a rare and potentially life threatening autoimmune disorder. The burden of disease compared to other dermatoses is unknown. The purpose of this study was to assess both the quality of life in patients with SSc and the variables that are associated with poor quality of life. Forty-one patients with systemic sclerosis (29 limited, 2 diffuse, 10 undifferentiated forms) were assessed with respect to their health status and compared to published data for the normal population, SSc patients from other studies, and patients with chronic skin diseases., Results: For the most part, our SSc patients had better outcomes in all 8 dimensions of the SF-36 than SSc patients from other studies, and poorer scores than the healthy population and those with occupational contact dermatitis, ichthyosis, non-melanoma skin cancer, contact dermatitis, atopic eczema, chronic nail disease, vitiligo, health care workers with work-related disease, and those with other chronic skin diseases, but significantly better scores for mental health than those with nail disease, vitiligo, and health-care workers. Patients with atopic dermatitis, psoriasis and pemphigus had significantly poorer mean scores in social function and mental health than SSc patients. Patients with pemphigus were also significantly impaired in their physical and emotional roles. Patients with systemic lupus erythematosus (SLE) had the significantly poorest mean scores for QoL in all 8 domains except bodily pain and emotional role., Conclusion: Besides SLE, SSc is one of the most severe chronic dermatologic diseases in terms of reduced QoL. Since SSc cannot be cured, treatment strategies should include therapeutic interventions such as psychotherapy, social support, physiotherapy, and spiritual care. Their beneficial effects could be studied in future.

Toxocarosis is a parasitic infection caused by Toxocara canis or Toxocara cati. Their definite hosts are the domestic dog and cat, where their adult forms live within the lumen of the small intestine. In humans, infective larvae hatch after ingestion of eggs, but the juvenile stages fail to develop into mature adult worms. Instead, they migrate through the body with the potential to affect virtually every body site. DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) is a serious cutaneous drug reaction, which also involves other organ systems. We report on a 45-year-old man who was admitted with fever up to 40 °C, skin rash and pruritus not responding to amoxicillin/clavulanic acid. Laboratory results showed a slightly elevated white blood cell count, with 62 % eosinophils. History revealed contact to roaming cats. Travel history was unremarkable. Extensive diagnostic workup was performed, and the patient was finally diagnosed with DRESS syndrome, responded to systemic corticosteroid therapy and was discharged. Serology for Toxocara came back positive a few days later. Stool was again sent for microscopy and was found positive for Giardia lamblia, which was treated with a 5-day course of metronidazole. The patient had again developed rash and eosinophilia (62 %). Total immunoglobulin (Ig)E was 1,689 U/l (normal limit: < 100 689 U/l). Follow-up serology showed a marked increase of Toxocara TES ELISA IgG (70 U, normal limit: < 20 U), confirming toxocarosis. Antiparasitic treatment with albendazole 400 mg bid for 5 days was initiated; eosinophilia resolved; and the patient had no further complaints. Although generalized exanthema due to Toxocara has not yet been described in literature, toxocarosis is known to cause a wide spectrum of cutaneous manifestations. Whether our patient had both, a drug reaction with eosinophilia and systemic symptoms and a parasitic infection, or whether a hypersensitivity reaction to Toxocara antigen was mimicking a DRESS syndrome remains unclear.