Rusk Amanda M, Crute Meghan M, Goebel Jens, Schecter Marc G, Gupta Anita, Schwentker Ann R, Sheth Anita P, Lucky Anne W
Department of Dermatology, University of Cincinnati, Cincinnati, Ohio, USA.
Department of Nephrology, Children's Hospital of Colorado, Aurora, Colorado, USA.
Pediatr Dermatol. 2020 Nov;37(6):1023-1029. doi: 10.1111/pde.14323. Epub 2020 Aug 12.
Trichodysplasia spinulosa is a rare disorder caused by the ubiquitous trichodysplasia spinulosa-associated polyomavirus (TSPyV) and characterized clinically by predominately centrofacial, but often generalized, folliculocentric papules with protuberant keratinaceous spines. Although seroprevalence reaches up to 70% in adult populations, TSPyV causes clinical manifestations in a small percentage of patients who are immunosuppressed. Diagnosis can be made using typical clinical and histologic features, SV40T antibody immunostaining, and PCR of various tissues including the keratinaceous spine, skin, serum, urine, and CSF. Various topical and systemic medications have demonstrated variable success. Decreasing or discontinuing immunosuppression has also been shown to improve or alleviate clinical manifestations.
棘状毛发发育异常是一种由普遍存在的棘状毛发发育异常相关多瘤病毒(TSPyV)引起的罕见疾病,临床特征主要为面中部,但常为全身性的、以毛囊为中心的丘疹,伴有突出的角质棘。尽管在成人人群中血清阳性率高达70%,但TSPyV仅在一小部分免疫抑制患者中引起临床表现。可根据典型的临床和组织学特征、SV40T抗体免疫染色以及对包括角质棘、皮肤、血清、尿液和脑脊液在内的各种组织进行PCR来做出诊断。各种局部和全身用药的疗效不一。减少或停用免疫抑制也已证明可改善或减轻临床表现。