Clinical Characteristics Associated with Musculoskeletal Extracutaneous Manifestations in Pediatric and Adult Morphea: A Prospective, Cohort Study.

Morphea is an autoimmune condition of the skin associated with functional sequelae resulting from musculoskeletal involvement. Systematic investigation of risk for musculoskeletal involvement is limited, particularly in adults. This knowledge gap impairs patient care because practitioners are unable to risk stratify patients. To address this gap, we determined the frequency, distribution, and type of musculoskeletal (MSK) extracutaneous manifestations affecting joint and bone with overlying morphea lesions using cross-sectional analysis of 1,058 participants enrolled in two prospective cohort registries (Morphea in Children and Adults Cohort [n = 750] and National Registry for Childhood Onset Scleroderma [n = 308]). Additional analysis included the identification of clinical features associated with MSK extracutaneous manifestations. MSK extracutaneous manifestations occurred in 274 of 1,058 participants (26% overall, 32% pediatric, and 21% adults). Children had a limited range of motion of larger joints (i.e., knees/hips/shoulders), whereas the involvement of smaller joints (i.e., toes/temporomandibular joint) was more common in adults. Multivariable logistic regression showed that deep tissue involvement had the strongest association with musculoskeletal features, with a lack of deep tissue involvement having a negative predictive value of 90% for MSK extracutaneous manifestations. Our results underscore the need to evaluate MSK involvement in adult and pediatric patients and the utility of using depth of involvement in addition to anatomic distribution to risk stratify patients.

An Evaluation of the Performance of Current Morphea Subtype Classifications.

Importance: Numerous classification systems for morphea subtypes exist, but none have been systematically evaluated for their ability to categorize patients with morphea into demographically and clinically coherent groups. Although some subtypes, such as linear morphea, are present across all the classification schemes, others list unique subtypes. This creates confusion among investigators and practitioners and impairs accurate categorization of patients for study and clinical evaluation. Objective: To evaluate how frequently the commonly used morphea classification systems categorize patients with morphea into clinically relevant subtypes using cross-sectional analysis of 2 large patient cohorts. Design, Setting, and Participants: This cross-sectional study comprised 944 adults and children from 2 prospective cohorts-the Morphea in Adults and Children at the University of Texas Southwestern Medical Center (Dallas, Texas), which enrolled participants from July 20, 2007, to September 21, 2018, and the National Registry for Childhood-Onset Scleroderma at the University of Pittsburgh (Pittsburgh, Pennsylvania), which enrolled participants from October 23, 2002, to November 13, 2018. Main Outcomes and Measures: Patient demographic characteristics, morphea subtype, quality-of-life measures, disease activity, and damage as measured by Localized Scleroderma Cutaneous Assessment Tool scores during initial visits. Results: A total of 944 participants (444 patients with adult-onset morphea and 500 patients with pediatric-onset morphea; 741 female participants [78%]; median age at onset, 16 years [interquartile range, 8-44 years]) were included in this study. Most participants were White (723 [77%]) and had the linear (474 [50%]) or generalized subtype of morphea (244 [26%]). With the use of the previously published Padua criteria, most patients were classified to have linear morphea (474 [50%]), followed by generalized morphea (244 [26%]), plaque morphea (141 [15%]), mixed morphea (38 [4%]), and pansclerotic morphea (3 [0.3%]). Overall, the Padua criteria successfully classified 900 patients (95%) in comparison with the Peterson criteria (533 [56%]) and the European Dermatology Forum classification (487 [52%]). Conclusions and Relevance: In this cross-sectional study of morphea subtype classification systems, the Padua criteria performed best in classifying patients into subgroups with cohesive demographic and clinical features, supporting its widespread use. However, they have ambiguities that might lead to misclassification, particularly in terms of generalized and pansclerotic morphea and descriptors such as morphea profunda. Consensus-based approaches are needed to address these ambiguities and develop a unified classification scheme.

Linear Scleroderma of the Head - Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties.

To provide an update on the current management, including evaluation and treatment, and the available diagnostic tools for linear scleroderma of the head, i.e., Parry-Romberg Syndrome and en coup de sabre (PRS/ECDS). A rapid scoping review of the literature was conducted to include manuscripts published in English between 2010 and 2019. Literature searches were performed in PubMed and EMBASE databases. The were analyzed for descriptive statistic reporting. This study reviewed 215 manuscripts reporting these 1430 patients. Surgical reports comprised the majority of the reviewed literature. Most PRS/ECDS did not appear to receive comprehensive multisubspecialty evaluation for extracutaneous manifestations; 21% of cases noted neurological screening, 4% noted dental screening, and 3% noted ophthalmologic screening. Methotrexate and glucocorticoids remain the most frequent choice for immunosuppressive treatment, though fewer than 7% of patients reported receiving systemic medical therapies. Surgical procedures for cosmetic or functional improvement were common (59%) among the reported patients. Autologous fat grafting was the most frequently utilized cosmetic treatment (50% of procedures) followed by free flap transfers (24% of procedures). There is ongoing need for standardized evaluation, monitoring, and treatment to prevent morbidity in PRS/ECDS, especially in children. When these patients are managed by rheumatologists, methotrexate, and steroids remain the first-line treatment, but a review of the published literature reflects that this may be a minority. Most PRS/ECDS patients are not evaluated in a multidisciplinary fashion. We propose comprehensive evaluations across subspecialties at the baseline and follow-up levels to monitor disease activity and record extracutaneous manifestations, treatment algorithms, and surgical intervention considerations.