Jeon Jiehyun, Kim Joo Ha, Ahn Jae Woo, Song Hae Jun
Department of Dermatology, Korea University Guro Hospital, Seoul, Korea.
Ann Dermatol. 2015 Apr;27(2):197-200. doi: 10.5021/ad.2015.27.2.197. Epub 2015 Mar 24.
Poikiloderma vasculare atrophicans (PVA) is a rare poikilodermatous variant of early-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectasia. In 2001, a 14-year-old male presented with asymptomatic brownish-gray polymorphic macules throughout the body with flexural accentuation. A skin biopsy showed increased melanophages with focal hydropic changes. Ashy dermatosis was considered a possible diagnosis. In 2005, the lesions began to show darkening and lichenification in the lower part of the trunk. In 2011, his skin showed definite poikilodermatous changes, and a biopsy showed band-like inflammatory infiltrations of atypical lymphocytes, epidermal atrophy, and epidermotropism of predominantly CD4(-)CD8(+) atypical T cells. In addition, results of T-cell receptor gene rearrangement analysis were positive. Based on the aforementioned findings, he was diagnosed with PVA. If a patient shows long-standing and progressive hyperpigmentary skin changes, periodic follow-up and repeated skin biopsies are recommended to determine the underlying condition.
血管萎缩性皮肤异色症（PVA）是蕈样肉芽肿早期一种罕见的皮肤异色症变体，其特征为全身性皮肤异色症、萎缩、斑驳状色素沉着异常和毛细血管扩张。2001年，一名14岁男性全身出现无症状的棕灰色多形性斑疹，屈侧部位更为明显。皮肤活检显示噬黑素细胞增多并伴有局灶性水样变性。考虑可能诊断为灰皮病。2005年，躯干下部的皮损开始出现色素沉着加深和苔藓样变。2011年，其皮肤出现明确的皮肤异色症改变，活检显示非典型淋巴细胞呈带状炎性浸润、表皮萎缩以及主要为CD4(-)CD8(+)非典型T细胞的亲表皮现象。此外，T细胞受体基因重排分析结果为阳性。基于上述发现，他被诊断为血管萎缩性皮肤异色症。如果患者出现长期且进行性的色素沉着性皮肤改变，建议定期随访并反复进行皮肤活检以确定潜在病情。
