ABSTRACT
Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae. Management of SSc cutaneous disease remains challenging and often is driven by extracutaneous manifestations. Methotrexate is the typical first-line therapy for patients with early progressive cutaneous disease. However, in patients with diffuse progressive skin disease and inflammatory arthritis, methotrexate or rituximab monotherapy should be considered. First-line therapy for patients with concomitant myositis includes methotrexate or intravenous immunoglobulin (IVIG). For patients with both cutaneous findings and interstitial lung disease, studies have suggested the efficacy of mycophenolate mofetil or rituximab. Second-line therapies, including UVA-1 phototherapy, IVIG, or rituximab, can be considered in patients with disease refractory to first-line treatments. Clinical trials investigating the utility of emerging therapies such as abatacept and tocilizumab in the treatment of SSc are under way, and preliminary results are promising. Nonetheless, all patients with SSc benefit from a gentle skin-care regimen to alleviate pruritis, which is a commonly reported symptom. Additional cutaneous manifestations of SSc include telangiectasias, calcinosis cutis, microstomia, and Raynaud's phenomenon. Telangiectasia may be managed with camouflage techniques, pulse dye laser, and intense pulse light. Calcinosis cutis therapy is guided by the size of the calcium deposits, although treatment options are limited. Mouth augmentation and oral stretching exercises are recommended for patients with reduced oral aperture. Raynaud's phenomenon is treated with a combination of lifestyle modification and calcium channel blockers, such as amlodipine. Overall, SSc is a clinically heterogenous disease that affects multiple organ systems. Providers should assess extracutaneous involvement and use evidence-based recommendations to select the most appropriate therapy for patients with SSc.
ABSTRACT
Cutaneous lupus erythematosus (CLE) is a connective tissue disease with varying presentations, and clinical sequelae including itching, dyspigmentation, and scarring. CLE can occur as its own entity or in conjunction with systemic disease, known as systemic lupus erythematosus (SLE). Because CLE is clinically diverse, identification of a biomarker may help not only facilitate early diagnosis and management but also identify individuals at risk for poor prognosis and development of SLE. While potential biomarkers in SLE have been extensively studied, few biomarkers for CLE have been identified and incorporated into clinical practice. Anti-SS-A antibody is a commonly used biomarker for diagnosis of subacute CLE patients. Type I interferon-related proteins such as MxA and guanylate binding protein-1 (GBP-1) and chemokines such as CXCR3, CXCL9, and CXCL10 have been identified as biomarkers that may support diagnosis and track disease activity. First-line oral treatment for CLE currently consists of anti-malarials such as hydroxychloroquine (HCQ), chloroquine (CQ), and quinacrine (QC). Studies have found that an increased myeloid dendritic cell population with higher TNF-α expression may be predictive of poor treatment response to HCQ in CLE patients. Autoantibodies against nuclear antigens (e.g., anti-double-stranded DNA and anti-Smith antibodies) and elevated erythrocyte sedimentation rate have been more commonly found in CLE patients progressing to SLE than those who have not. This review aims to summarize previous and emerging biomarkers for CLE patients.
ABSTRACT
BACKGROUND: Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge. OBJECTIVE: To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort. METHODS: Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018. RESULTS: A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5 years; P < .001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P = .004). LIMITATIONS: Selection bias and limited number of affected subjects. CONCLUSION: Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.
