Rheumatic Skin Diseases Research Articles

International exchange on the clinical practice and research of rheumatic skin diseases: A report of the 5th International Conference of Cutaneous Lupus Erythematosus.

The 5th International Conference of Cutaneous Lupus Erythematosus was held in Tokyo, Japan on May 9 and 10, 2023. The latest topics on the pathogenesis, diagnosis, assessment, and treatment of cutaneous lupus erythematosus, dermatomyositis, and scleroderma (systemic sclerosis, morphea) were presented by experts in each field and new developments discussed. In these rheumatic skin diseases, many clinical trials of novel therapies targeting cytokines, signaling molecules, plasmacytoid dendritic cells, B cells, and other molecules are currently underway, and standardization of outcome assessment was discussed. In addition, the selection of the therapeutic agents available for the diversity of each case is becoming more important, together with the ongoing pathophysiological analysis of the diseases. The achievements of this conference will further promote the development of clinical practice and research in rheumatic skin diseases through international exchange among researchers. We hope that by reporting a summary of the conference in this manuscript, we can share its contents with readers.

A case series of hydroxychloroquine exacerbating the dermatomyositis rash.

Hydroxychloroquine (HCQ) is an antimalarial agent that is commonly used in the management of rheumatic skin disease. Few reports exist documenting exacerbation of dermatomyositis (DM) related to HCQ. Herein, we describe three adult patients with worsening DM cutaneous disease after starting HCQ and resolution or improvement with cessation. The time to exacerbation ranged from two weeks to nine months after the initiation of HCQ 400mg/day. Two of the three patients had antibodies to transcription intermediary factor 1γ (TIF1γ) and the other had antibodies to anti-nuclear matrix protein 2 (NXP2). After discontinuation of HCQ, the time to improvement or resolution of cutaneous symptoms ranged from six weeks to six months. Hydroxychloroquine may be associated with worsening cutaneous features in DM. In patients who are not improving despite escalation of immunosuppressive medications, or are worsening, we recommend a trial of discontinuing HCQ.

Decreased risk of rheumatic skin diseases in HIV/AIDS inpatients, potential role of antiretroviral therapy?

Decreased risk of rheumatic skin diseases in HIV/AIDS inpatients, potential role of antiretroviral therapy?

BI06 (P42): Decreased risk of rheumatic skin diseases in HIV/AIDS

BI06 (P42): Decreased risk of rheumatic skin diseases in HIV/AIDS

P42: Decreased risk of rheumatic skin diseases in HIV/AIDS

P42: Decreased risk of rheumatic skin diseases in HIV/AIDS

Antimalarial drug toxicities in patients with cutaneous lupus and dermatomyositis: A retrospective cohort study

Although existing evidence demonstrates the efficacy of antimalarials for rheumatic skin disease, the safety of these medications, and particularly quinacrine, remains debated. We investigated the toxicity risk associated with antimalarials in patients with cutaneous lupus erythematosus and dermatomyositis. A total of 532 patients (mean age, 52.29years; sample composition by sex, 85.15% female vs 14.85% male) were selected from 2 databases on cutaneous lupus erythematosus (69.92%) and dermatomyositis (30.08%). Details regarding treatment and toxicities were extracted and 5 treatment courses were defined (ie, hydroxychloroquine [HCQ], chloroquine [CQ], quinacrine [Q], HCQ-Q combination therapy [HCQ-Q], and CQ-Q combination therapy [CQ-Q]). The hazard ratio for each major toxicity was estimated by using the Cox proportional hazard model to compare the different treatments with HCQ. The most common toxicities included cutaneous eruption, gastrointestinal upset, mucocutaneous dyspigmentation, neurologic toxicity, and retinopathy. The hazards of cutaneous eruption, gastrointestinal upset, and neurologic toxicities were lower with HCQ-Q than with HCQ; however, this may represent selection bias. Although there was increased retinopathy risk with CQ and CQ-Q versus with HCQ, retinopathy was not seen with Q. Retrospective analysis. With the exception of retinopathy, which was not seen with Q, the risks for other toxicities associated with Q monotherapy or combination treatment were not significantly different from those with HCQ.

Skin disease in pregnancy: The approach of the obstetric medicine physician

This review presents the approach of the obstetric medicine physician to skin disease in pregnancy. It elaborates on common skin-related problems during gestation, such as pruritus, with or without eruption, and drug eruptions. An algorithmic approach to the differential diagnosis of pruritus in pregnancy is outlined. Also, the review focuses on how to diagnose promptly endocrinopathies presenting with skin manifestations in pregnancy, such as Addison disease, diabetes, and hyperthyroidism. The prompt diagnosis of endocrine disorders can help to optimize management and improve outcomes. Finally, the authors outline their approach to minimizing maternal and fetal risks associated with skin disease. The risks associated with obstetric cholestasis, pemphigoid gestationis, and impetigo herpetiformis are discussed. Prompt diagnosis helps to minimize the serious risks associated with certain infections. Preconception counseling and a multidisciplinary approach are crucial to preventing risks associated with rheumatic skin disease and genodermatoses. Challenging, real-life obstetric medicine cases are discussed.

Skin and Rheumatic Disease

Many rheumatic diseases may involve any organ system of the body including the skin. Proper understanding of rheumatic skin disease is necessary for making a diagnosis. In addition, there are important relationships existing between the cutaneous and systemic manifestations of rheumatic diseases. In management of these diseases, the interdisciplinary approach could generate better results. Among rheumatic diseases, the cutaneous manifestations of three major rheumatic diseases such as lupus erythematosus (LE), dermatomyositis (DM)/polymyositis and scleroderma/systemic sclerosis (SSc) will all be reviewed.

Efficacy of mycophenolate mofetil in antimalarial-resistant cutaneous lupus erythematosus

Mycophenolate mofetil (MMF) is an immunomodulatory drug shown to be effective in the treatment of systemic lupus erythematosus. Several anecdotal reports have suggested that MMF may be efficacious in the treatment of cutaneous lupus erythematosus (CLE). Our objective was to summarize and report our experience with the use of MMF in patients with CLE recalcitrant to antimalarial therapy. We retrospectively analyzed our open-label observations of 24 patients with CLE refractory to antimalarial therapy. The records of all patients visiting the Rheumatic Skin Disease Clinic at the University of Texas Southwestern Medical Center at Dallas from January 1, 2001, to July 1, 2006, were reviewed. MMF was tolerated well and, in conjunction with other therapies, was highly effective in the treatment of antimalarial-resistant CLE. With the addition of MMF to the existing regimen, a majority of patients achieved full control of disease signs and symptoms. All patients experienced some degree of improvement. This is an open-label retrospective review. Severity of disease was assessed by qualitative assessment of the clinician. MMF was not used as monotherapy. Our results indicate that MMF, used as an additional agent in conjunction with standard therapy, is both well tolerated and efficacious in the treatment of refractory CLE. Despite the obvious limitations of the study, we believe this represents further evidence that MMF should be considered early in the treatment of patients refractory to antimalarial therapy.

Rheumatic manifestations of skin disease

There is an increasing interest in improving the understanding of pathophysiology, outcome measures, and therapies of rheumatic skin disease. Increasingly, studies are using the skin as a primary endpoint for evaluating therapies. This will review the current state of the art for the most common rheumatic skin diseases. A number of medications, including biologics such as tumor necrosis factor alpha and interferon, have been associated with onset of cutaneous lupus. The cutaneous lupus erythematosus area and severity index has been further validated and utilized in a number of studies. Smoking continues to be associated both with presence and refractoriness of cutaneous lupus erythematosus to therapy. There are several tools now available for evaluating the skin disease of dermatomyositis, but there is a need for new effective therapies. Measurement of skin disease in scleroderma continues to be a challenge, and there is a need for more effective therapies. Several studies show efficacy of intravenous iloprost for severe Raynaud's and skin ulcers, and of bosentan for digital ulcers. The present review covers new outcome measures, treatments, and unusual manifestations of cutaneous lupus, dermatomyositis, scleroderma, and rheumatoid arthritis. There have been a number of new studies related to validation of disease activity measures, as well as their use in evaluation of new therapies for these conditions. Validated outcome measures are required to perform meaningful studies, and will facilitate organized epidemiologic, quality of life, and therapeutic studies.

Update on Connective Tissue Diseases in Dermatology

Recent advances in understanding the pathogenesis of autoimmune diseases, including lupus erythematosus, dermatomyositis, and scleroderma, have allowed for reorganization of the classification of these disorders. With these novel stratifications, early identification of rheumatic skin diseases with systemic implications and consistency in designing and executing therapeutic trials will be enhanced. This review will provide a compilation of updates on epidemiology, pathology, evaluation, and classification with a predominant focus on therapeutics, reflecting the growth is this area.

Skin manifestations of systemic autoimmune connective tissue disease: diagnostics and therapeutics.

Skin disease can significantly affect the quality of life of patients suffering from rheumatic diseases. In addition, important relationships exist between the cutaneous and systemic manifestations of rheumatic disease. It is thus important for practicing rheumatologists to have a solid working understanding of the subject of rheumatic skin disease. Unfortunately, it is not possible within the scope of this chapter to provide a comprehensive overview of the recognition and management of all of the cutaneous manifestations of lupus erythematosus (LE), much less those of dermatomyositis (DM)/polymyositis and scleroderma/systemic sclerosis (SSc) as well. As can be seen in in the text, the cutaneous manifestations of polygenic autoimmune disorders such as LE can be as heterogeneous clinically as are its systemic manifestations. This discussion will therefore focus on recent key developments concerning the diagnosis and management of the more common skin changes that the practicing rheumatologist is likely to encountered in the three major rheumatic diseases: LE, DM and SSc.

Immunosuppressive and cytotoxic drugs in the treatment of rheumatic skin disorders.

Cytotoxic and immunosuppressive drugs are regularly used to treat proliferative, immunologically mediated inflammatory disorders and some neoplastic diseases of the skin. Methotrexate, azathioprine, mycophenolate mofetil, cyclosporin cyclophosphamide, chlorambucil, and other related drugs have potential benefits in the treatment of severe and/or recalcitrant rheumatic skin diseases. The therapeutic window for these agents is narrow. The major uses of these drugs are for life-threatening cutaneous disorders or as steroid-sparing agents.