Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Pediatric Dermatology Research Alliance, Portland, Oregon, USA.
J Invest Dermatol. 2023 Oct;143(10):1955-1963.e3. doi: 10.1016/j.jid.2023.04.010. Epub 2023 May 2.
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.
硬斑病是一种与肌肉骨骼受累相关的自身免疫性皮肤病，可导致功能性后遗症。对肌肉骨骼受累风险的系统性研究有限，尤其是在成人中。这一知识空白影响了患者的护理，因为医生无法对患者进行风险分层。为了解决这一差距，我们通过对两个前瞻性队列登记处（儿童和成人群体硬斑病队列[ n = 750]和儿童发病性硬皮病国家登记处[ n = 308]）的 1058 名参与者进行横断面分析，确定了影响有硬斑病皮损的关节和骨骼的肌肉骨骼（MSK）皮肤外表现的频率、分布和类型。进一步的分析包括确定与 MSK 皮肤外表现相关的临床特征。1058 名参与者中有 274 名（总体为 26%，儿童为 32%，成人 21%）出现 MSK 皮肤外表现。儿童的大关节（即膝关节/髋关节/肩关节）活动范围有限，而成年人的小关节（即脚趾/颞下颌关节）受累更为常见。多变量逻辑回归显示，深部组织受累与肌肉骨骼特征的相关性最强，深部组织无受累对 MSK 皮肤外表现具有 90%的阴性预测值。我们的研究结果强调了在成人和儿童患者中评估 MSK 受累情况的必要性，以及除解剖分布外，还使用受累深度对患者进行风险分层的效用。
