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手部边缘性角化弹性组织变性:掌跖角化病的一种独特形式。

Keratoelastoidosis Marginalis of the Hands: A Distinct Form of Palmoplantar Keratoderma.

作者信息

Coutada Andreia, Butina Mirjam R, Luzar Bostjan

机构信息

Pathology, Portuguese Oncology Institute of Porto, Porto Comprehensive Cancer Centre Raquel Seruca, Porto, PRT.

Dermatology, Dermatologija Rogl Fabjan, Ljubljana, SVN.

出版信息

Cureus. 2024 Nov 12;16(11):e73515. doi: 10.7759/cureus.73515. eCollection 2024 Nov.

DOI:10.7759/cureus.73515
PMID:39669842
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11636050/
Abstract

Keratoelastoidosis marginalis of the hands (KEMH) is an acquired form of marginal papular keratoderma, characterized by thickened keratotic plaques predominantly affecting the lateral side of the index finger and the medial side of the thumb. It is often associated with chronic sun exposure and trauma, usually affecting older individuals. Due to clinical similarities with other palmoplantar keratodermas, differential diagnosis is essential for effective treatment management. Although clinical information is often sufficient for differentiation, a skin biopsy can provide valuable diagnostic clues. We report the case of a 63-year-old male patient who presented with rough linear hyperkeratotic lesions on the lateral and medial margins involving the first and second fingers of both hands, which had developed over a period of three years. Skin biopsy revealed orthohyperkeratosis with an underlying epidermis of normal thickness, with no features of actinic keratosis. In the dermis, thickened elastic fibers and degenerated collagen bundles were haphazardly distributed. Based on clinicopathological findings, a diagnosis of KEMH was made. As limited information is available in the literature, we aim to expand the current understanding of KEMH by emphasizing crucial aspects of its pathogenesis, histological characteristics, and main differential diagnoses.

摘要

手部边缘性角化弹性组织病(KEMH)是边缘性丘疹性角化病的一种后天性形式,其特征为角化性斑块增厚,主要累及食指外侧和拇指内侧。它常与长期日晒和创伤有关,通常影响老年人。由于与其他掌跖角化病在临床症状上有相似之处,因此鉴别诊断对于有效治疗管理至关重要。虽然临床信息通常足以进行鉴别,但皮肤活检可提供有价值的诊断线索。我们报告一例63岁男性患者,其双手第一和第二指的外侧及内侧边缘出现粗糙的线状角化过度性病变,这些病变在三年时间里逐渐发展。皮肤活检显示正角化过度,其下方表皮厚度正常,无光化性角化病特征。在真皮层,增厚的弹性纤维和变性的胶原束呈杂乱分布。根据临床病理结果,诊断为KEMH。由于文献中可用信息有限,我们旨在通过强调其发病机制、组织学特征和主要鉴别诊断的关键方面,来扩展目前对KEMH的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/cb4c8b4cb6a0/cureus-0016-00000073515-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/bf248b32db32/cureus-0016-00000073515-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/c0a8d6e814b6/cureus-0016-00000073515-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/2f483dc7a39c/cureus-0016-00000073515-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/07cf2661913f/cureus-0016-00000073515-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/4835810b100e/cureus-0016-00000073515-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/14930671bef9/cureus-0016-00000073515-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/cb4c8b4cb6a0/cureus-0016-00000073515-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/bf248b32db32/cureus-0016-00000073515-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/c0a8d6e814b6/cureus-0016-00000073515-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/2f483dc7a39c/cureus-0016-00000073515-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/07cf2661913f/cureus-0016-00000073515-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/4835810b100e/cureus-0016-00000073515-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/14930671bef9/cureus-0016-00000073515-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/219b/11636050/cb4c8b4cb6a0/cureus-0016-00000073515-i07.jpg

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Acrokeratoelastoidosis as an example of marginal popular acrokeratoderma with prominent elastorrhexis.肢端角化性弹性组织变性作为边缘性丘疹性肢端角化病伴显著弹性组织离解的一个例子。
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Keratoelastoidosis marginalis of the hands: A report in two farmers.手部边缘性角化弹性组织变性:两名农民的病例报告。
Indian Dermatol Online J. 2016 May-Jun;7(3):195-7. doi: 10.4103/2229-5178.182364.
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Degenerative collagenous plaques of the hands and acrokeratoelastoidosis: pathogenesis and relationship with knuckle pads.手部退行性胶原斑块与肢端角化性弹性组织病:发病机制及与指节垫的关系
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Marginal papular acrokeratodermas: a unified nosography for focal acral hyperkeratosis, acrokeratoelastoidosis and related disorders.边缘丘疹性肢端角化病:一种针对局限性肢端角化过度、肢端角化弹性组织变性及相关疾病的统一病症分类法。
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