Brusasco A, Veraldi S, Tadini G, Caputo R
Centre for Inherited Cutaneous Diseases, Institute for Dermatological Sciences, IRCCS, Ospedale Maggiore, University of Milan, Via Pace 9, 20122 Milan, Italy.
Br J Dermatol. 1998 Sep;139(3):492-5. doi: 10.1046/j.1365-2133.1998.02416.x.
We report a young woman in whom the history, clinical features, histopathological and ultrastructural findings led to a diagnosis of peeling skin syndrome (PSS). PSS is a rare and not well classified genodermatosis, mainly characterized by the spontaneous separation of the stratum corneum from the stratum granulosum. The unusual feature in our patient was the strict localization to the palm. PSS has been described as a more generalized disease frequently sparing palms and soles. We propose the diagnosis label of 'localized PSS' for this previously undescribed variant of a rare keratinization defect.
我们报告了一名年轻女性，其病史、临床特征、组织病理学和超微结构检查结果均支持剥脱性皮肤综合征（PSS）的诊断。PSS是一种罕见且分类尚不明确的遗传性皮肤病，主要特征为角质层与颗粒层的自发性分离。我们患者的不寻常之处在于病变严格局限于手掌。PSS通常被描述为一种更广泛的疾病，常不累及手掌和足底。我们建议将这种先前未描述的罕见角化缺陷变体诊断为“局限性PSS”。
