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具有细胞毒性标志物表达的儿童CD8+/CD56+蕈样肉芽肿:一种病程惰性的变体

Pediatric CD8+/CD56+ mycosis fungoides with cytotoxic marker expression: A variant with indolent course.

作者信息

Rovaris Marco, Colato Chiara, Girolomoni Giampiero

机构信息

Department of Medicine, Section of Dermatology and Venereology, University of Verona, Verona, Italy.

Department of Pathology and Diagnostics, Section of Pathology, University of Verona, Verona, Italy.

出版信息

J Cutan Pathol. 2018 Oct;45(10):782-785. doi: 10.1111/cup.13317. Epub 2018 Jul 24.

DOI:10.1111/cup.13317
PMID:29968317
Abstract

Mycosis fungoides is predominantly a disease of older patients, but occasionally occurs in children. We report a rare case of CD8+/CD56+ mycosis fungoides with cytotoxic marker (perforin, TIA-1, and granzyme B) expression in a 10-year-old boy. Disease presented with three asymptomatic, slowly progressive erythematous and scaling plaques, surrounded by hypochromic alone in the left tight and lower trunk. UVB narrow band associated with topical corticosteroids resulted in complete remission in about 2 months, and no recurrence at 2-year follow-up. Three similar cases have been retrieved in children through PubMed search, showing similar clinical presentation with erythematous scaling lesions, good response to skin-directed treatments and a favorable prognosis.

摘要

蕈样肉芽肿主要是一种老年患者的疾病,但偶尔也会发生在儿童身上。我们报告了一例罕见的CD8+/CD56+蕈样肉芽肿病例,该病例发生在一名10岁男孩身上,伴有细胞毒性标志物(穿孔素、TIA-1和颗粒酶B)表达。疾病表现为三个无症状、缓慢进展的红斑鳞屑性斑块,仅在左大腿和下腹部周围有色素减退。窄谱中波紫外线联合外用糖皮质激素治疗约2个月后完全缓解,随访2年无复发。通过PubMed检索在儿童中发现了三例类似病例,表现出类似的红斑鳞屑性病变临床表现,对皮肤定向治疗反应良好且预后良好。

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