Duangurai K, Piamphongsant T, Himmungnan T
Institute of Dermatology, Bangkok, Thailand.
Int J Dermatol. 1988 May;27(4):248-52. doi: 10.1111/j.1365-4362.1988.tb03219.x.
Sézary cells were present in a number of less than 1000/mm3 in 52 of 78 cases of various benign diffuse dermatoses and mycosis fungoides. The count was higher than 1000/mm3 in two cases of Sézary syndrome in which the histopathology was that of mycosis fungoides and in six cases of exfoliative erythroderma of undetermined etiology with the histopathology of nonspecific chronic dermatitis. One of these six cases developed palmoplantar keratoderma, but abnormal cells were not present in the tissue. The diagnosis of Sézary syndrome in these cases of exfoliative erythroderma could not be established, even though the Sézary cell count was more than 1000/mm3, because the abnormal cells were absent in the skin biopsy specimen. It is concluded that the number of Sézary cells present is not significant for the diagnosis of Sézary syndrome. Our cases with the diagnosis of Sézary syndrome are an erythrodermic variant of mycosis fungoides rather than a progressive conversion from exfoliative erythroderma.
在78例各种良性弥漫性皮肤病和蕈样肉芽肿病例中,52例的 Sézary 细胞数量少于1000/mm³。在2例组织病理学表现为蕈样肉芽肿的 Sézary 综合征病例以及6例病因不明的剥脱性红皮病病例(组织病理学为非特异性慢性皮炎)中,Sézary 细胞计数高于1000/mm³。这6例中的1例出现掌跖角化病,但组织中未发现异常细胞。尽管这些剥脱性红皮病病例的 Sézary 细胞计数超过1000/mm³,但由于皮肤活检标本中不存在异常细胞,因此无法确诊为 Sézary 综合征。结论是,Sézary 细胞的数量对 Sézary 综合征的诊断并不重要。我们诊断为 Sézary 综合征的病例是蕈样肉芽肿的红皮病变体,而非剥脱性红皮病的进行性转化。