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以瘙痒性脐周斑块为表现的罕见穿通性皮肤病

Pruritic Periumbilical Plaque as a Presentation of a Rare Perforating Dermatosis.

作者信息

Bathina Meghana, Hegde Spandana P, Shanavaz Akbar A, Saldanha Prema P

机构信息

Department of Dermatology, Venereology and Leprosy, Yenepoya Medical College, Mangalore, Karnataka, India.

Department of Pathology, Yenepoya Medical College, Mangalore, Karnataka, India.

出版信息

Indian Dermatol Online J. 2020 Jan 13;11(1):68-71. doi: 10.4103/idoj.IDOJ_95_19. eCollection 2020 Jan-Feb.

DOI:10.4103/idoj.IDOJ_95_19
PMID:32055513
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7001405/
Abstract

Perforating dermatoses are characterized by transepidermal elimination of altered dermal components or foreign particles. Owing to their common clinical presentation as umbilicated papules with a keratotic plug, histopathology and special staining play a very crucial role in the diagnosis. Perforating calcific elastosis, (periumbilical perforating pseudoxanthoma elasticum), an uncommon acquired localized cutaneous dermatoses, is characterized by transepidermal elimination of modified elastic fibres. It is usually seen in middle-aged obese multiparous women as well-defined periumbilical hyperpigmented atrophic plaques. We report a case of a 66-year-old female who presented with a mildly pruritic hyperpigmented periumbilical plaque of 2 years duration. Histopathology studies revealed multiple fragmented, thick, short, and curly eosinophilic fibers; along with granular basophilic material in the dermis, which stained positive for calcium and elastin, thus clinching the diagnosis of perforating calcific elastosis. There were no features of hereditary pseudoxanthoma elasticum. We report this case for its rarity.

摘要

穿通性皮肤病的特征是真皮成分改变或异物经表皮排出。由于其常见临床表现为带有角质栓的脐凹性丘疹,组织病理学和特殊染色在诊断中起着至关重要的作用。穿通性钙化弹性组织变性(脐周穿通性类弹力纤维性假黄瘤)是一种罕见的后天性局限性皮肤疾病,其特征是经表皮排出变性的弹性纤维。它通常见于中年肥胖经产妇,表现为边界清晰的脐周色素沉着萎缩性斑块。我们报告一例66岁女性,其脐周出现轻度瘙痒性色素沉着斑块,病程2年。组织病理学研究显示真皮内有多个破碎、粗大、短小且卷曲的嗜酸性纤维;以及颗粒状嗜碱性物质,钙和弹性蛋白染色呈阳性,从而确诊为穿通性钙化弹性组织变性。无遗传性类弹力纤维性假黄瘤的特征。我们报告此病例是因其罕见性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/5d269854bdbb/IDOJ-11-68-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/068fa3f0478d/IDOJ-11-68-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/6392448e37fe/IDOJ-11-68-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/953da7c97425/IDOJ-11-68-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/5d269854bdbb/IDOJ-11-68-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/068fa3f0478d/IDOJ-11-68-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/6392448e37fe/IDOJ-11-68-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/953da7c97425/IDOJ-11-68-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0022/7001405/5d269854bdbb/IDOJ-11-68-g005.jpg

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本文引用的文献

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Transepidermal elimination: Historical evolution, pathogenesis and nosology.经皮排泄:历史演变、发病机制与疾病分类学
Indian J Dermatol Venereol Leprol. 2018 Nov-Dec;84(6):753-757. doi: 10.4103/ijdvl.IJDVL_396_17.
2
Periumbilical perforating pseudoxanthoma elasticum: a rare case report.脐周穿孔性弹性假黄瘤:一例罕见病例报告
Dermatol Pract Concept. 2018 Apr 30;8(2):75-77. doi: 10.5826/dpc.0802a02. eCollection 2018 Apr.
3
Perforating Calcific Elastosis: Revisiting a Rare Entity.穿孔性钙化性弹性组织变性:重新审视一种罕见病症。
Indian J Dermatol. 2018 Mar-Apr;63(2):186-188. doi: 10.4103/ijd.IJD_111_17.
4
Histopathology of pseudoxanthoma elasticum and related disorders: histological hallmarks and diagnostic clues.弹性假黄瘤及相关疾病的组织病理学:组织学特征与诊断线索
Scientifica (Cairo). 2012;2012:598262. doi: 10.6064/2012/598262. Epub 2012 Jul 25.
5
Periumbilical perforating pseudoxanthoma elasticum.脐周穿凿性假黄瘤。
Indian J Dermatol Venereol Leprol. 2009 May-Jun;75(3):329. doi: 10.4103/0378-6323.51246.