Momtahen Shabnam, Nuovo Gerard J, Magro Cynthia M
*Department of Pathology and Laboratory Medicine, Weill Cornell Medical College of Cornell University, New York, NY; and †Ohio State University Comprehensive Cancer Center Core Lab, Powell, OH.
Am J Dermatopathol. 2015 Sep;37(9):724-9. doi: 10.1097/DAD.0000000000000242.
Mycosis fungoides (MF), the most common form of cutaneous lymphoma is derived from postthymic T cells that migrate to the skin likely under the influence of chronic antigen stimulation. Less common histomorphologic variants are diagnostically challenging because of their resemblance to reactive conditions. Three men aged 46, 73, and 74 years and one 83-year-old woman were encountered in the files of one of the authors and represented the patients. The patients had a longstanding eruption for several years. In 2 cases, the clinical course was aggressive with extracutaneous lymph node and/or peripheral blood dissemination. One patient had vesicles noted clinically. In all cases, vesiculation was a prominent feature histologically, which lead to an erroneous categorization initially as a reactive process. Basilar colonization by cerebriform lymphocytes along with the mucinous quality of the vesicle was diagnostic clue histologically, whereas the phenotypic and molecular profile was typical for MF. Strong expression of interleukin 5 and interleukin 10 in atypical lymphocytes in comparison with interferon gamma suggested a T-helper type 2-dominant cytokine microenvironment. In reviewing the literature of the 6 previously reported cases, 3 patients died of the disease; all these patients had vesicular lesions both clinically and histologically. We concluded that vesicular MF is a distinct histological and in some instances clinical variant of MF. The correlation of the vesicular eczematous quality of the eruption and a more aggressive clinical course may reflect the skewing toward a T-helper type 2-dominant cytokine milieu typical of advanced disease.
蕈样肉芽肿(MF)是皮肤淋巴瘤最常见的形式,起源于胸腺后T细胞,可能在慢性抗原刺激的影响下迁移至皮肤。不太常见的组织形态学变异型因其与反应性疾病相似,在诊断上具有挑战性。本文作者之一的病例档案中有3名男性(年龄分别为46岁、73岁和74岁)和1名83岁女性,他们代表了这些患者。这些患者有多年的长期皮疹。2例患者的临床病程呈侵袭性,伴有皮肤外淋巴结和/或外周血播散。1例患者临床上可见水疱。在所有病例中,水疱形成在组织学上是一个突出特征,最初导致错误地归类为反应性过程。脑回状淋巴细胞的基底浸润以及水疱的黏液样性质是组织学诊断线索,而表型和分子特征是MF的典型表现。与干扰素γ相比,非典型淋巴细胞中白细胞介素5和白细胞介素10的强表达提示以2型辅助性T细胞为主导的细胞因子微环境。在回顾之前报道的6例病例的文献时,3例患者死于该病;所有这些患者在临床和组织学上均有水疱性病变。我们得出结论,水疱性MF是MF一种独特的组织学类型,在某些情况下也是临床变异型。皮疹的水疱性湿疹样性质与更具侵袭性的临床病程之间的相关性可能反映了向晚期疾病典型的以2型辅助性T细胞为主导的细胞因子环境的转变。