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慢性苔藓样角化病:一个概念的提出。

Keratosis lichenoides chronica: proposal of a concept.

作者信息

Böer Almut

机构信息

Dermatologikum Hamburg, Germany.

出版信息

Am J Dermatopathol. 2006 Jun;28(3):260-75. doi: 10.1097/00000372-200606000-00052.

Abstract

It has been a subject of controversy whether keratosis lichenoides chronica (KLC) is a distinctive inflammatory disease of the skin or whether it represents a manifestation of another well-known disease, such as lichen planus, lupus erythematosus, or lichen simplex chronicus. In search of clear criteria for diagnosis of KLC the entire literature pertinent to the subject was studied and findings clinical and histopathologic as they were telegraphed in them were compared with a patient of my own experience. Review of the literature reveals more than 60 patients in whom the diagnosis of KLC was made. Three categories emerge based on whether the findings presented in a particular article (1) do not permit any diagnosis to be rendered; (2) do allow a diagnosis specific to be made, such as of lichen simplex, lichen planus, or lupus erythematosus; or (3) do not correspond to any disease well defined, such as lichen simplex, lichen planus, lupus erythematosus, but seem to show attributes morphologic, clinically and histopathologically, that are repeatable. Patients diagnosed as having KLC obviously represent a potpourri of different diseases, the most common of them being lichen simplex chronicus, lichen planus, and lupus erythematosus. Fewer than 25 patients reported on, however, presented themselves with lesions very similar to one another clinically, namely, an eruption that involved the face in a manner reminiscent of seborrheic dermatitis and with tiny papules on the trunk and extremities, which assumed linear and reticulate shapes by way of confluence of lesions. Individual papules were infundibulocentric and acrosyringocentric. Findings histopathologic were those of a lichenoid interface dermatitis affiliated with numerous necrotic keratocytes and covered by parakeratosis housing neutrophils in staggered fashion. These patients seem to have an authentic and distinctive condition that is exceedingly rare. In conclusion, the diagnosis of KLC should be made only for patients who present themselves with features clinical and findings histopathologic that resemble closely those of what is summarized in this article under category 3.

摘要

慢性苔藓样角化病(KLC)究竟是一种独特的皮肤炎症性疾病,还是代表另一种知名疾病(如扁平苔藓、红斑狼疮或慢性单纯性苔藓)的表现,一直是个有争议的话题。为了寻找KLC的明确诊断标准,研究了与该主题相关的全部文献,并将其中报道的临床和组织病理学发现与我自己诊治的一名患者进行了比较。文献回顾显示,有60多名患者被诊断为KLC。根据某一特定文章中呈现的发现可分为三类:(1)无法做出任何诊断;(2)确实能做出特定诊断,如慢性单纯性苔藓、扁平苔藓或红斑狼疮;(3)与任何明确界定的疾病(如慢性单纯性苔藓、扁平苔藓、红斑狼疮)均不相符,但在形态学、临床和组织病理学上似乎具有可重复性的特征。被诊断为KLC的患者显然代表了不同疾病的混杂情况,其中最常见的是慢性单纯性苔藓、扁平苔藓和红斑狼疮。然而,报告的患者中不到25例临床皮损彼此非常相似,即一种皮疹,累及面部,类似脂溢性皮炎,躯干和四肢有微小丘疹,皮损融合后呈线状和网状。单个丘疹以漏斗中心和顶泌汗腺导管中心分布。组织病理学表现为苔藓样界面性皮炎,伴有大量坏死角质形成细胞,表面有角化不全,中性粒细胞呈交错状分布。这些患者似乎患有一种真实且独特的疾病,极为罕见。总之,只有那些临床特征和组织病理学发现与本文第3类总结的情况非常相似的患者,才能诊断为KLC。

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