Efficacy of mycophenolate mofetil in antimalarial-resistant cutaneous lupus erythematosus.

BACKGROUND: 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). OBJECTIVES: Our objective was to summarize and report our experience with the use of MMF in patients with CLE recalcitrant to antimalarial therapy. METHODS: 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. RESULTS: 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. LIMITATIONS: This is an open-label retrospective review. Severity of disease was assessed by qualitative assessment of the clinician. MMF was not used as monotherapy. CONCLUSIONS: 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.

Leflunomide as adjuvant treatment of dermatomyositis.

We report 3 cases of recalcitrant dermatomyositis that responded to the addition of daily leflunomide, a novel immunomodulatory drug that is currently used to treat rheumatoid arthritis. In our patients, leflunomide proved both safe and effective as adjuvant therapy in treating dermatomyositis.

Methotrexate in dermatology.

Methotrexate is a folic acid analog pioneered for use in inflammatory diseases by dermatologists, and used successfully for over 40 years for a wide variety of cutaneous diseases. In addition to its antiproliferative properties, methotrexate has other more recently recognized anti-inflammatory properties related to its effects on adenosine. Further research concerning its mechanism of action and genetic enzymatic variations suggest future possibilities for maximizing therapy and predicting adverse events. In this review the present authors will explore methotrexate's pharmacokinetics, mode of administration, dosing guidelines, side effect profile, and medication interactions. In addition, the present authors hope to offer practical guidelines for dose initiation and adjustment, and to summarize new research on its mechanism of action and implications for future therapy.

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.

Is Degos' disease a clinical and histological end point rather than a specific disease?

Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near "pathognomonic" appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied. We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions. Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.

Fatal interstitial pulmonary fibrosis in anti-Jo-1-negative amyopathic dermatomyositis.

Amyopathic dermatomyositis (DM) describes a subpopulation with the cutaneous eruption of DM, but without muscle involvement. Interstitial pulmonary fibrosis is a recognized complication of DM, often correlated with antisynthetase enzymes, such as anti-Jo-1. We describe a case of fatal IPF in a patient with anti-Jo-1 antibody-negative amyopathic DM.

Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus.

Neonatal lupus erythematosus is an uncommon autoimmune disease with distinctive cutaneous findings. Descriptions of chronic cutaneous sequelae are rare. We describe a 12-year-old girl with persistent dyspigmentation, scarring, and atrophy as a result of neonatal lupus occurring during infancy.