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匐行性穿通性弹力纤维病(持久性豆状角化过度症)的临床病理研究

A clinicopathological study of Flegel's disease (hyperkeratosis lenticularis perstans).

作者信息

Price M L, Jones E W, MacDonald D M

出版信息

Br J Dermatol. 1987 May;116(5):681-91. doi: 10.1111/j.1365-2133.1987.tb05902.x.

DOI:10.1111/j.1365-2133.1987.tb05902.x
PMID:2439113
Abstract

The clinical and histopathological features of 12 female patients with Flegel's disease were studied. The onset of this dermatosis was delayed until adulthood. The eruption consisted of scaly papules 1-5 mm in diameter which developed principally on the lower legs, upper arms and pinnae. Histopathologically there were discrete foci of hyperkeratosis with some parakeratosis over an attenuated and partially spongiotic epidermis. A genetic influence in this disorder was suggested by its occurrence in sisters in two families and in a mother and daughter. Immunohistochemical and biochemical studies suggested that there is a disorder of keratinocyte proliferation in the disease.

摘要

对12例患有剥脱性角质松解症的女性患者的临床和组织病理学特征进行了研究。这种皮肤病的发病延迟至成年期。皮疹由直径1-5毫米的鳞屑性丘疹组成,主要发生在小腿、上臂和耳廓。组织病理学上,在变薄且部分海绵形成的表皮上有离散的角化过度灶,并伴有一些不全角化。该病在两个家族的姐妹以及一对母女中出现,提示有遗传影响。免疫组织化学和生化研究表明,该病存在角质形成细胞增殖紊乱。

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A clinicopathological study of Flegel's disease (hyperkeratosis lenticularis perstans).匐行性穿通性弹力纤维病(持久性豆状角化过度症)的临床病理研究
Br J Dermatol. 1987 May;116(5):681-91. doi: 10.1111/j.1365-2133.1987.tb05902.x.
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Hyperkeratosis lenticularis perstans (Flegel's disease). A light and electron microscopic study of involved and uninvolved epidermis.持久性豆状角化过度症(弗莱格病)。对受累及未受累表皮的光镜和电镜研究。
Dermatologica. 1987;174(2):96-101.
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An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease).持久性豆状角化过度症(弗莱格尔病)的一种罕见的全身性形式。
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Hyperkeratosis lenticularis perstans (Flegel's disease): our experience and review of the literature.持久性层状角皮病(Flegel 病):我们的经验和文献复习。
Int J Dermatol. 2021 Jan;60(1):33-38. doi: 10.1111/ijd.14930. Epub 2020 May 13.
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Hyperkeratosis lenticularis perstans (Flegel's disease)--lack of response to treatment with tacalcitol and calcipotriol.持久性豆状角化过度症(弗莱格病)——对他卡西醇和卡泊三醇治疗无反应。
Dermatology. 2001;202(3):255-8. doi: 10.1159/000051648.
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[40 years to Flegel's disease (hyperkeratosis lenticularis perstans)].[距弗莱格病(持久性豆状角化过度症)发病40年]
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[Hyperkeratosis lenticularis perstans (Flegel's disease) - a complex disorder of epidermal differentiation with good response to a synthetic vitamin D3 derivate].持久性豆状角化过度症(弗莱格病)——一种对合成维生素D3衍生物反应良好的表皮分化复杂疾病
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Case 3: widespread scaly papules affecting a patient's limbs and pinnae giving the skin a rough feeling. Diagnosis: Flegel's disease. Syn. hyperkeratosis lenticularis perstans.病例3:广泛的鳞屑性丘疹累及患者的四肢和耳廓,使皮肤有粗糙感。诊断:弗勒格病。同义词:持久性豆状角化过度。
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Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response to isotretinoin therapy.致编辑的图片信:持久性豆状角化过度症(弗莱格尔病)伴不寻常临床表现。对异维A酸治疗的反应
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Hyperkeratosis lenticularis perstans (Flegel's disease). Case report and literature review.持久性豆状角化过度症(弗莱格病)。病例报告及文献综述。
J Am Acad Dermatol. 1987 Jan;16(1 Pt 2):190-5. doi: 10.1016/s0190-9622(87)80060-9.

引用本文的文献

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Systematic review of Flegel disease: clinical presentations, associations, diagnostic pitfalls, and management challenges.弗勒格尔病的系统评价:临床表现、关联因素、诊断陷阱及管理挑战
Arch Dermatol Res. 2025 May 16;317(1):766. doi: 10.1007/s00403-025-04268-x.
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Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel's Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation.泛发性持久性豆状角化过度症(弗莱格病):一种罕见表现的临床病理及皮肤镜特征
Dermatopathology (Basel). 2023 Jan 13;10(1):46-51. doi: 10.3390/dermatopathology10010006.
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Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response to isotretinoin therapy.
致编辑的图片信:持久性豆状角化过度症(弗莱格尔病)伴不寻常临床表现。对异维A酸治疗的反应
J Dermatol Case Rep. 2012 Sep 28;6(3):93-5. doi: 10.3315/jdcr.2012.1111.