Department of Dermatology, Queen Elizabeth Hospital, King's Lynn, UK.
Department of Dermatology, Freiburg University Medical Center, Freiburg, Germany.
J Eur Acad Dermatol Venereol. 2015 Nov;29(11):2088-99. doi: 10.1111/jdv.13077. Epub 2015 Mar 6.
The categorization of congenital hypo- or hyperpigmented skin lesions following a segmental pattern has been a long-lasting matter of debate and have been reported under various and often incorrect terms. To reassess published hypomelanotic and hypermelanotic lesions that did not follow Blaschko lines nor a phylloid pattern of mosaicism, we carried out an extensive and critical review of the worldwide literature. Seventy-four retrieved cases consisted of lateralized hypomelanotic lesions arranged in a flag-like pattern or appearing as large patches of grossly oval or angulated shape and sharp, serrated margins. Sometimes lesions harboured maculopapular melanocytic naevi or cooccurred with other segmentally arranged naevi. A probably non-random association with extracutaneous anomalies was also reported on rare occasions. In 70 cases, lateralized hypermelanotic patches were arranged in a flag-like pattern that often appeared as large quadrangular patches. Sometimes lesions harboured Spitz naevi. Ten cases belonged to phacomatosis melanorosea, whereas several others were part of so far uncategorized cases of phacomatosis pigmentovascularis. Flag-like hypomelanosis is a distinct naevus type, for which the term 'flag-like hypomelanotic naevus' is suggested. Its cooccurrence with extracutaneous abnormalities might represent a specific syndrome. Flag-like hypermelanosis is a distinct naevus type, for which the term 'flag-like hypermelanotic naevus' is suggested. Its co-occurrence with naevus roseus defines phacomatosis melanorosea. Flag-like hypermelanotic naevus should be distinguished from the checkerboard-like areas of darker skin as observed in chimaeras.
先天性色素减退或过度色素沉着性皮肤病变的分类一直是一个长期存在的争议问题，并且已经以各种不同且常常不正确的术语进行了报道。为了重新评估未遵循 Blaschko 线或叶状镶嵌模式的发表的色素减退和色素过度沉着性病变，我们对全球文献进行了广泛而严格的回顾。74 例检索到的病例包括呈旗帜样排列的侧化性色素减退病变，或表现为大体上呈椭圆形或有角度的大块斑块，边缘锐利，呈锯齿状。有时病变伴有斑片状黑素细胞痣或与其他节段性排列的痣并存。偶尔也有报道称，病变与皮肤外异常存在可能非随机的关联。在 70 例病例中，侧化性色素过度沉着斑块呈旗帜样排列，通常表现为大的四边形斑块。有时病变伴有 Spitz 痣。10 例属于色素性神经皮肤综合征，而其他几例属于尚未分类的色素血管性神经皮肤综合征。旗帜样色素减退是一种独特的痣类型，建议使用“旗帜样色素减退痣”这一术语。其与皮肤外异常的共存可能代表一种特定的综合征。旗帜样色素过度沉着是一种独特的痣类型，建议使用“旗帜样色素过度沉着痣”这一术语。其与神经皮肤玫瑰疹的共存定义了色素性神经皮肤综合征。旗帜样色素过度沉着痣应与嵌合体中观察到的较深色皮肤的棋盘状区域区分开来。
