Background: Stiff skin syndrome is a sclerodermalike disorder that presents in infancy or early childhood with rock-hard skin, limited joint mobility, and mild hypertrichosis in the absence of visceral or muscle involvement, immunologic abnormalities, or vascular hyperreactivity.Observations: We describe 6 children who fit criteria for stiff skin syndrome. A review of the clinical range of this disorder and discussion of the differential diagnosis is presented. The age at onset in our cases ranged from infancy to 6 years of age. Stony-hard skin was noted mostly on the thighs, buttocks, and lower back with shoulder and arm involvement in 2 cases. There was associated hypertrichosis in 3 of 6 cases. Extracutaneous manifestations consisted primarily of joint restriction, and several patients had resulting postural and thoracic wall ir-regularities. Histopathologically, our cases showed areas of fascial sclerosis or showed increased fibroblast cellularity with thickened, sclerotic, horizontally oriented collagen bundles in the deep reticular dermis and/or subcutaneous septa without associated inflammation.Conclusions: Stiff skin syndrome is characterized by an early, insidious onset of stony-hard skin, often with associated contracturelike joint restriction, hypertrichosis, and postural and thoracic wall abnormalities. Supportive histopathologic findings consisting of either fascial sclerosis or increased fibroblast cellularity with sclerotic collagen bundles in the deep reticular dermis and/or subcutaneous septa may help to confirm this diagnosis.

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Sarcopenia is underrecognized in patients with rheumatoid arthritis (RA). Risk factors of sarcopenia and its impact on outcomes in RA patients are relatively unknown. We conducted a systematic review to identify factors and outcomes associated with sarcopenia in RA. We conducted this review according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) 2020 guidelines. We searched PubMed, Embase, CINAHL, and Web of Science databases by combining the following search concepts: (1) RA and (2) sarcopenia. Articles were included if they included RA patients, assessed for sarcopenia using a consensus working group definition, and assessed for clinical outcomes. Meta-analysis was performed using studies that shared the same sarcopenia definition and consistency in reporting patient or disease variables. Our search identified 3602 articles. After removal of duplicates, title and abstract screen, and full-text review, 16 articles were included for final analysis. All studies had observational study designs. The pooled prevalence of sarcopenia ranged from 24% to 30%, depending on the criteria for sarcopenia used. Factors associated with sarcopenia included higher 28-joint Disease Activity Scale scores (+0.39; 95% confidence interval, +0.02 to +0.77) and baseline methotrexate use (odds ratio, 0.70; 95% confidence interval, 0.51-0.97). Baseline glucocorticoid use had a positive correlation with sarcopenia in multiple studies. Several studies found lower bone mineral density and higher incidence of falls and fractures in patients with sarcopenia. Sarcopenia is prevalent in RA, and it may be associated with higher RA disease activity, lower bone mineral density, and increased falls and fractures. Therefore, early screening of sarcopenia in RA patients is important to incorporate into clinical rheumatology practice.