Circumscribed palmar or plantar hypokeratosis:: A distinctive epidermal malformation of the palms or soles

Circumscribed palmar or plantar hypokeratosis:: A distinctive epidermal malformation of the palms or soles
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DOI:
10.1067/mjd.2002.120466
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发表时间:
2002-07-01
影响因子:
13.8
通讯作者:
Requena, L
Requena, L
中科院分区:
医学1区
文献类型:
--
作者:
Pérez, A;Rütten, A;Requena, L

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背景:皮肤表皮畸形包括一组异质发育缺陷,这些缺陷是由子宫内表皮形态发生错误引起的。目的。本研究的目的是报告涉及手掌或脚底皮肤的独特表皮畸形的临床和组织病理学特征。方法:本研究纳入了 10 名患者。所有这些患者都表现出相同的临床特征,即手掌或足底出现孤立的局限性圆形红斑凹陷皮肤区域。经组织病理学检查确诊。结果:所有患者均为中老年。九名患者是女性,一名是男性。病变好发于手掌大鱼际和小鱼际区域或足底内侧的皮肤。组织病理学研究表明表皮凹陷,正常皮肤和受累皮肤之间有陡峭的阶梯。与邻近的非受累皮肤相比,覆盖凹陷的表皮显示出明显更薄的角质层和稍微减少的颗粒细胞层。鳞状细胞层的角质形成细胞、颗粒细胞和角质细胞显示出正常的外观。系列切片未能显示角突片状化。结论:根据这10例患者的临床和组织病理学结果,我们将这种畸形命名为局限性掌或足底角化不足。该病变似乎是以前未曾描述过的独特实体。
Background: Epidermal malformations of the skin include a group of heterogeneous developmental defects that result from errors in morphogenesis of the epidermis during intrauterine life.Objective. The purpose of this study was to report the clinical and histopathologic features of a distinctive epidermal malformation involving the skin of the palms or soles.Methods: Ten patients were included in this study. All of them showed the same clinical features that consisted of a solitary circumscribed and circular area of erythematous depressed skin on the palm or on the sole. Diagnosis was confirmed by histopathologic study.Results: All patients were middle aged or elderly. Nine patients were women and one was a man. The lesions showed predilection for the skin of the thenar and hypothenar regions of the palm or the medial side of the sole. Histopathologic study demonstrated a depression of the epidermis, with a sharp stair between normal and involved skin. The epidermis covering the depression showed markedly thinner horny layer and a slightly diminished granular cell layer when compared with adjacent noninvolved skin. Keratinocytes of the squamous cell layer, granular cells, and corneocytes showed, otherwise, a normal appearance. Serial sections failed to demonstrate cornoid lamellation.Conclusion: On the basis of the clinical and histopathologic findings in these 10 patients, we have named this malformation circumscribed palmar or plantar hypokeratosis. This lesion seems to be a distinctive entity that has not been previously described.