外阴Paget样网状细胞增生症

Vulvar pagetoid reticulosis:a case report

报告1例外阴Paget样网状细胞增生症。患者女,70岁。外阴红斑、增生、破溃、渗液2年。皮肤科检查见外阴及其周围有一11cm×10cm浸润性暗红斑,边界清楚,边缘略隆起。皮损组织病理检查示表皮下部和真皮上部可见不典型单核细胞浸润,多个异形细胞聚集成巢,甚似Pautrier微脓肿;免疫组化染色结果示CD3、CD43、Ki-67均(+),CD4、CD8、CD20、CD79α、CD30均(-)。诊断:外阴Paget样网状细胞增生症。

A case of vulvar pagetoid reticulosis is reported .A 70-year-old woman presented with erythema,proliferation,ulceration and exudation on the vulva for 2 years ,Clinical examination showed a 11cm × 10cm well-defined infitrated dull red plaque with a slightly elevated edge on the vulva .The histopathologic examination showed numerous atypical mononuclear cells infiltrating the lower epidermis and the upper dermis and the atypical cells gathered in a nested distribution,similar to the Pautrier＇s microabscesses.The heteromorphic cells were positive for CD3,CD43,Ki-94,but negative for CD4,CD8,CD20,CD79α and CD30.A diagnosis of vulvar pagetoid reticulosis was made.