摘要患者女,18岁,因外阴和右下肢肿大伴乳白色水疱3年就诊.皮肤科检查:右侧阴唇和右下肢肿胀粗大,右侧大阴唇、两侧小阴唇、阴道口以及右大腿内侧皮肤上群集和散在许多针头至粟粒大乳白色厚壁水疱,有乳白色乳糜液从阴道流出.MRI检查示下腹腔和骨盆右侧的淋巴管显著扩张,右大腿内侧皮肤和皮下组织以及阴道壁和右侧大阴唇内淋巴管扩张扭曲,但子宫未见异常.皮肤组织病理示真皮浅层的淋巴管扩张成囊状,真皮中下部可见纤维组织增生.诊断原发性乳糜反流综合征Ⅰ型.

abstractsAn 18-year-old woman was admitted to the hospital for swelling of the right lower limb and vulva with milky-white vesicles for 3 years.Skin examination revealed swollen and enlarged right lower limb and labium,numerous clustered or scattered pinhead-to millet-sized whitish thick-walled vesicles over the right labium majus,bilateral labium minus,vaginal orifice,and right thigh,with milky-white chylous fluid draining from the vagina.Magnetic resonance imaging revealed obvious lymphangiectasia in the lower abdominal cavity and right side of the pelvis,dilation and distortion of lymphatic vessels in the skin and subcutaneous tissue of the right inner thigh,the vaginal wall and at the medial side of the right labium majus,but no abnormality in the uterus.Histopathological examination of the milky-white vesicles showed cystic dilation of lymphatic vessels in the superficial dermis,and fibrous hyperplasia of the middle and lower dermis.A diagnosis of primary chylous reflux syndrome Ⅰ was made.