Department of Dermatology, The University of Texas McGovern Medical School, Houston, TX, USA.
Transpl Infect Dis. 2020 Dec;22(6):e13434. doi: 10.1111/tid.13434. Epub 2020 Aug 16.
Trichodysplasia Spinulosa (TS) is a rare proliferative skin disease that occurs primarily in immunocompromised patients, specifically organ transplant recipients. TS is characterized by uncontrolled inner root sheath cell proliferation and folliculocentric papular eruption that can progress to disfiguring leonine facies when left untreated. TS presents with distinct histological features including the presence of large eosinophilic, trichohyaline granules within hyperproliferating inner root sheath cells of the hair bulb. The discovery of the Trichodysplasia Spinulosa Polyomavirus (TSPyV) and recent studies highlighting the role of TSPyV tumor antigens in cell proliferation pathways have provided new insight into the mechanisms of TS development. In this review, we discuss the expansion of our understanding of TS, specifically over the past 5 years. We summarize novel cases of TS and recent developments in the mechanisms underlying TSPyV-mediated disease progression. We also evaluate advancements in diagnostic methods and treatment options. As the incidence of TS continues to rise, it is becoming critical for clinicians to understand the clinical features of TS and emerging research regarding pathogenesis and therapeutics for early treatment of this potentially disfiguring disease.
棘层松解性外毛根鞘瘤(TS)是一种罕见的增生性皮肤疾病,主要发生于免疫功能低下的患者,特别是器官移植受者。TS 的特征是不受控制的内根鞘细胞增殖和毛囊中心性丘疹性疹,若不治疗可进展为致畸形的狮面。TS 具有独特的组织学特征,包括毛球内过度增生的内根鞘细胞中存在大的嗜酸性、毛角蛋白颗粒。棘层松解性外毛根鞘瘤多瘤病毒(TSPyV)的发现以及最近的研究强调了 TSPyV 肿瘤抗原在细胞增殖途径中的作用,为 TS 发病机制提供了新的见解。在这篇综述中,我们讨论了过去 5 年来对 TS 的认识的扩展。我们总结了 TS 的新病例以及 TSPyV 介导的疾病进展的机制的最新进展。我们还评估了诊断方法和治疗选择的进展。随着 TS 的发病率不断上升,了解 TS 的临床特征以及有关发病机制和治疗学的新兴研究对于早期治疗这种潜在致畸形疾病对于临床医生来说变得至关重要。