Background: Prospective, longitudinal studies examining the features of linear morphea are limited. Objective: To utilize the Morphea in Adults and Children cohort to determine clinical characteristics, impact on life quality, and disease course of linear morphea in a prospective, longitudinal manner. Methods: Characteristics of linear morphea versus other subtypes were compared in a cross-sectional manner. Next, linear morphea participants were examined in depth over a 3-year period. Results: Linear morphea was the most common morphea subtype (50.1%, 291/581) in the cohort. Deep involvement was more common in linear (64.3%, 187/291) than other morphea subtypes. Linear morphea participants with deep involvement were more likely to have a limitation in range of motion (28.6%, 55/192) than those without (11.1%, 11/99, P \ .001). Adult-onset disease occurred in 32.6% (95/291) of those with linear morphea. Frequency of deep involvement was similar between pediatric (66.8%, 131/196) and adult-onset linear morphea (58.9%, 56/95, P = .19). Quality of life and disease activity scores improved over time, while damage stabilized with treatment. Limitations: Results of the study are associative, and the University of Texas Southwestern Medical Center is a tertiary referral center. Conclusion: A substantial number of linear morphea patients have adult-onset disease. In all age groups, linear morphea with deep involvement was associated with functional limitations.

We prospectively compared subtalar arthroereisis with lateral column calcaneal lengthening for the treatment of painful flatfeet. Twenty-four feet (mean age of patients 12.8 years) were treated. Kinematic motion analysis, pedobarometry, and radiography were performed, and the Oxford Ankle-Foot Questionnaire for Children was administered for each patient before surgery and at the 1-year follow-up. We found statistically significant improvements in both groups, with no difference in their outcomes. Both groups showed significantly improved hindfoot and midfoot motion and positioning. Hindfoot range of motion was preserved. Radiography and pedobarometry also revealed significant improvements. Subtalar arthroereisis is a valid and potentially less-invasive alternative to lateral column lengthening that merits further investigation.

IntroductionLesional skin of patients with discoid lupus erythematosus (DLE) contains macrophages, whose polarization has yet to be investigated. To test our hypothesis that M1 macrophages would be increased in DLE skin, we examined transcriptome alterations in immune cell gene expression and macrophage features in DLE and normal skin by using gene expression and histochemical approaches.MethodsGene expression of RNA from DLE lesional and normal control skin was compared by microarrays and quantitative real-time polymerase chain reaction (RT-PCR). Both skin groups were analyzed for CD163 expression by immunohistochemistry. Double immunofluorescence studies were performed to characterize protein expression of CD163+ macrophages.ResultsDLE skin had twice as many upregulated genes than downregulated genes compared with normal skin. Gene set enrichment analysis comparing differentially expressed genes in DLE and normal skin with previously published gene sets associated with M1 and M2 macrophages showed strong overlap between upregulated genes in DLE skin and M1 macrophages. Quantitative RT-PCR showed that several M1 macrophage-associated genes—e.g., chemokine (C-X-C motif) ligand 10 (CXCL10), chemokine (C-C motif) ligand 5 (CCL5), and signal transducer and activator of transcription 1 (STAT1)—had amplified mRNA levels in DLE skin. CD163+ macrophages were increased near the epidermal-dermal junction and perivascular areas in DLE skin compared with normal skin. However, double immunofluorescence studies of CD163+ macrophages revealed minor co-expression of M1 (CXCL10, tumor necrosis factor-alpha, and CD127) and M2 (CD209 and transforming growth factor-beta) macrophage-related proteins in DLE skin.ConclusionWhereas a subset of CD163+ macrophages displays mixed polarizations in DLE skin, other immune cells such as T cells can contribute to the expression of these macrophage-related genes.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0839-3) contains supplementary material, which is available to authorized users.

Summary Background Validated scoring measures in morphoea can facilitate clinical trials. Objectives To ascertain the clinical significance of scores on the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and identify the change in scores correlated with clinically meaningful change. Methods A prospective study of 120 participants from the Morphea in Adults and Children (MAC) cohort was undertaken. Physician's subjective assessments of severity and of improvement were completed at each visit. Receiver operating characteristic analysis determined LoSCAT scores corresponding with mild, moderate and severe disease, and absolute and percentage changes in scores corresponding with improved or worsened disease activity or damage. Results Mild, moderate and severe activity corresponded with LoSCAT activity index (LoSAI) scores of 0–4, 5–12 and 13 and over, and with Physician's Global Assessment of activity (PGA‐A) scores of 0–10, 11–30 and 31 and over. Mild, moderate and severe damage corresponded with LoSCAT damage index (LoSDI) scores of 0–10, 11–15 and 16 and over, and with PGA of damage (PGA‐D) scores of 0–18, 19–30 and 31 and over. Improved activity was best indicated by LoSAI decrease of at least 2 points or 27·5%, or PGA‐A decrease of at least 6 points. Improved damage was best indicated by LoSDI score decrease of at least 2 points. Worsening activity was best indicated by LoSAI increase of at least 2 points or 19·5%, or PGA‐A increase of at least 4 points. Worsening damage was best indicated by LoSDI increase of at least 25·5%. Conclusions The LoSCAT can be used to classify patients with morphoea by disease severity, and identify clinically significant improvement in activity. What's already known about this topic? The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical tool that separately quantifies disease activity and damage in morphoea, and prior studies have demonstrated validity and reliability. What does this study add? The LoSCAT can be used to classify patients with morphoea by disease severity into mild, moderate and severe groups, and to identify clinically significant improvement in disease activity in patients with morphoea. The LoSCAT may be limited in its ability to detect clinically significant changes in disease damage.

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