Happle R
Department of Dermatology, Freiburg University Medical Center, Freiburg, Germany.
J Eur Acad Dermatol Venereol. 2015 Dec;29(12):2295-305. doi: 10.1111/jdv.13147. Epub 2015 Apr 10.
The name capillary malformation has caused much confusion because it is presently used to designate numerous quite different disorders such as naevus flammeus, the salmon patch, the vascular naevus of the hereditary 'megalencephaly-capillary malformation syndrome' and the skin lesions of non-hereditary traits such as 'capillary malformation-arteriovenous malformation' and 'microcephaly-capillary malformation'. To avoid such bewilderment, the present review describes the distinguishing clinical and genetic criteria of 20 different capillary malformations, and a specific name is given to all of them. The group of capillary naevi includes naevus flammeus, port-wine naevus of the Proteus type, port-wine naevus of the CLOVES type, naevus roseus, rhodoid naevus, cutis marmorata telangiectatica congenita, congenital livedo reticularis, segmental angioma serpiginosum, naevus anaemicus, naevus vascularis mixtus and angiokeratoma circumscriptum. Capillary lesions that perhaps represent naevi are the mesotropic port-wine patch, Carter-Mirzaa macules, unilateral punctate telangiectasia and unilateral naevoid telangiectasia of the patchy type. Capillary malformations that do not represent naevi include X-linked angiokeratoma corporis diffusum (Fabry disease), autosomal dominant angiokeratoma corporis diffusum, hereditary haemorrhagic telangiectasia, hereditary angioma serpiginosusm and the salmon patch. In this way, we are able to discriminate between various non-hereditary capillary naevi such as naevus roseus and the hereditary rhodoid naevus and several hereditary traits that do not represent naevi such as angiokeratoma corporis diffusum and hereditary haemorrhagic telangiectasia; between four different types of port-wine stains, three of them being lateralized and one being mesotropic; between cutis marmorata telangiectatica congenita and congenital livedo reticularis; between telangiectatic naevi and the vasoconstrictive naevus anaemicus; and between two different types of angiokeratoma corporis diffusum. Finally, arguments are presented why the salmon patch ('stork bite', 'naevus simplex') cannot be categorized as a naevus.
毛细血管畸形这一名称引发了诸多混淆，因为目前它被用于指代许多截然不同的病症，如焰色痣、鲑鱼斑、遗传性“巨头畸形 - 毛细血管畸形综合征”的血管痣以及非遗传性特征的皮肤病变，如“毛细血管畸形 - 动静脉畸形”和“小头畸形 - 毛细血管畸形”。为避免此类困惑，本综述描述了20种不同毛细血管畸形的鉴别临床和遗传标准，并为它们都赋予了一个特定名称。毛细血管痣组包括焰色痣、变形杆菌型葡萄酒色痣、CLOVES型葡萄酒色痣、玫瑰痣、类玫瑰痣、先天性毛细血管扩张性大理石样皮肤、先天性网状青斑、匐行性血管瘤、贫血痣、混合性血管痣和局限性血管角皮瘤。可能代表痣的毛细血管病变有中间型葡萄酒色斑、卡特 - 米尔扎斑、单侧点状毛细血管扩张和斑片状单侧痣样毛细血管扩张。不代表痣的毛细血管畸形包括X连锁弥漫性躯体血管角皮瘤（法布里病）、常染色体显性遗传性弥漫性躯体血管角皮瘤、遗传性出血性毛细血管扩张症、遗传性匐行性血管瘤和鲑鱼斑。通过这种方式，我们能够区分各种非遗传性毛细血管痣，如玫瑰痣和遗传性类玫瑰痣，以及几种不代表痣的遗传性特征，如弥漫性躯体血管角皮瘤和遗传性出血性毛细血管扩张症；区分四种不同类型的葡萄酒色斑，其中三种为单侧型，一种为中间型；区分先天性毛细血管扩张性大理石样皮肤和先天性网状青斑；区分毛细血管扩张性痣和血管收缩性贫血痣；以及区分两种不同类型的弥漫性躯体血管角皮瘤。最后，阐述了为何鲑鱼斑（“鹳咬痕”、“单纯痣”）不能归类为痣的理由。
