穆兰线性萎缩性皮肤病。

IF 2.9 3区 医学 Q2 DERMATOLOGY Journal of Dermatology Pub Date : 2024-05-06 DOI:10.1111/1346-8138.17268
Andrea Paradisi, Irina Ciobotariu, Laura Quattrini, Francesco Ricci, Ketty Peris
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引用次数: 0

摘要

莫兰线性萎缩性皮肤病(LAM)是一种罕见的后天获得性皮肤病,它遵循布拉什科(Blaschko)的纹路。1 虽然其确切的发病机制尚不清楚,但其分布被认为反映了基因嵌合。发病前通常没有炎症,发病后也没有硬化,其特征是躯干或四肢出现色素沉着、轻度萎缩的斑块。病变呈单侧分布,但也有双侧受累的报道、4 在最初几个月,LAM 通常以线状萎缩性病变发展,然后病变停止发展,病变持续存在。本病例是一名 16 岁男性,病史 3 年,左侧躯干上部出现进行性、无症状、凹陷的线状病变,并在短期内变得更加清晰。病变出现在躯干后部,表现为色素沉着的萎缩性斑块,从椎旁区域向左肩横向延伸,位于胸椎 T3 水平。2-3 个月后,斑块变得清晰,但无症状,皮肤无炎症或压痕(图 1a-d)。组织病理学检查显示,病变部位表皮的齿状脊消失,基底黑素细胞轻度增生,真皮厚度减少,淋巴细胞浸润稀疏,血管扩张,表层胶原纤维轻微增厚(图 S1)。对于 LAM 是否属于包括帕西尼和皮埃尔尼萎缩性硬皮病以及线性硬皮病在内的一种疾病谱,还存在争议。然而,尽管有一些相似之处,但 LAM 不同的发病年龄、分布、组织学、起源、发展和预后表明它是一种独立的疾病,并强调了认识其不同临床特征的重要性。
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Linear atrophoderma of Moulin

Linear atrophoderma of Moulin (LAM) is a rare, acquired dermatosis that follows Blaschko's lines.1 Although its exact pathogenesis is unclear, its distribution is believed to reflect genetic mosaicism.2

Linear atrophoderma of Moulin onset is typically sudden, most frequently during adolescence or childhood. It is not commonly preceded by inflammation or followed by sclerosis, and is characterized by hyperpigmented, slightly atrophic patches on the trunk or extremities. The lesions show a unilateral distribution, although bilateral involvement has been reported.3, 4 In the first months, LAM usually progresses as linear atrophic lesions, then progression ceases and the lesions persist.

We present the case of a 16-year-old male with a 3-year history of progressive, asymptomatic, depressed linear lesions on the left upper trunk that became more defined over a short period.

The disease arose on the posterior trunk as pigmented atrophic plaques extending laterally from the paravertebral region toward the left shoulder, at the level of T3 of the thoracic spine. After 2–3 months the plaques became well defined, but were asymptomatic with no sign of inflammation or induration of the skin (Figure 1a–d).

There was no underlying disease. None in the patient's family had similar lesions.

On histopathology, the lesion showed epidermal loss of the rete ridges, mild hyperplasia of basal melanocytes, and a reduced dermal thickness with sparse lymphocytic infiltrate, vasodilatation, and slight thickening of the superficial collagen fibers (Figure S1).

There is no effective treatment for LAM, but a partial response to topical calcipotriol has been reported.5 It is debated whether LAM belongs to a spectrum including atrophoderma of Pasini and Pierini and linear scleroderma. However, despite some similarities, the different age at onset, distribution, histology, origin, development, and prognosis of LAM suggest that it is a separate disease and highlights the importance of recognizing its distinct clinical features.

None declared.

Written patient consent was obtained for use of the images.

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来源期刊
Journal of Dermatology
Journal of Dermatology 医学-皮肤病学
CiteScore
4.60
自引率
9.70%
发文量
368
审稿时长
4-8 weeks
期刊介绍: The Journal of Dermatology is the official peer-reviewed publication of the Japanese Dermatological Association and the Asian Dermatological Association. The journal aims to provide a forum for the exchange of information about new and significant research in dermatology and to promote the discipline of dermatology in Japan and throughout the world. Research articles are supplemented by reviews, theoretical articles, special features, commentaries, book reviews and proceedings of workshops and conferences. Preliminary or short reports and letters to the editor of two printed pages or less will be published as soon as possible. Papers in all fields of dermatology will be considered.
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