一例舍普夫-舒尔茨-帕萨热综合征病例。
A case of Schöpf-Schulz-Passarge syndrome.
作者信息
Hampton P J, Angus B, Carmichael A J
机构信息
Pathology, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, UK.
出版信息
Clin Exp Dermatol. 2005 Sep;30(5):528-30. doi: 10.1111/j.1365-2230.2005.01855.x.
Schöpf-Schulz-Passarge syndrome (SSPS) is a rare ectodermal dysplasia characterized by hypodontia, hypotrichosis, nail dystrophy, palmoplantar keratoderma, and periocular and eyelid margin apocrine hidrocystomas. Several other skin tumours have been described in association with this syndrome, in particular, multiple palmoplantar eccrine syringofibroadenoma (ESFA). We report a case of SSPS with diffuse palmoplantar hyperkeratosis, which was shown by histology and immunocytochemistry to be due to ESFA.
朔普夫-舒尔茨-帕萨热综合征（SSPS）是一种罕见的外胚层发育异常疾病，其特征为牙齿发育不全、毛发稀少、指甲营养不良、掌跖角化病以及眼周和眼睑边缘大汗腺汗管囊肿。还发现该综合征与其他几种皮肤肿瘤有关，尤其是多发性掌跖小汗腺汗管纤维腺瘤（ESFA）。我们报告一例伴有弥漫性掌跖角化过度的SSPS病例，组织学和免疫细胞化学检查显示其病因是ESFA。
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